PAEDIATRICS Flashcards
1
Q
What is the etiology of anaphylaxis?
A
- IgE mediated type 1 allergic reaction
- Triggers histamine release from mast cells and eosinophils
- Causes capillary leakage, edema, shock and asphyxia
2
Q
What are the initial symptoms of anaphylaxis?
A
- Pruritis (itchy skin)
- erythema (redness)
- urticaria (hives, red raised rash)
- rhinitis
- conjunctivitis
- angiooedema
3
Q
What are the general symptoms of anaphylaxis?
A
- Palpitations
- tachycardia
- nausea and vomiting
- abdominal pain
- collapse
- loss of consciousness
4
Q
What are the airway symptoms of anaphylaxis?
A
- itching of the palate of external auditory meatus
- dyspnoea
- bronchospasm (wheezing) –> edema and acute stridor
- cyanosis
- circulatory collapse (rare)- reduced CRT hypotension, tachycardia
5
Q
What is the initial management for anaphylaxis (before ABCDE)
A
- lay the patient flat
- raise legs with care
6
Q
What is A in anaphylaxis?
A
Look for:
- obstruction
- swelling
- signs of allergen
Manage with:
- call for help
- intubation
- high flow o2, 15L through non-rebreathe mask
7
Q
What is B in anaphylaxis?
A
Look for signs of respiratory distress:
- tracheal tug
- nasal flaring
- intercostal recession
- headbobbing
8
Q
What is C in anaphylaxis?
A
Look for:
- Colour
- Pulse
- BP
- CRT
Manage with:
- IV fluid challenge- 20mL/kg of 0.9% NaCl in 5mins
- Maintenance fluid- 100ml/kg for 1st 10kg, then 20ml/kg
9
Q
What is D in anaphylaxis?
A
Assess consciousness level
10
Q
What is E in anaphylaxis?
A
Assess glucose level and do system review
11
Q
What is the medical management for anaphylaxis?
A
ADRENALINE IM --> 500mcg if >12 --> 300mcg if >6 --> 150mcg if <6 repeated after 5min if no effect
CHLORPHENAMINE
- -> 10mg >12
- -> 5mg >6
- -> 2.5mg <6
CORTICOSTEROIDS (hydrocortisone IV)
- -> 200mg >12
- -> 100mg >6
- -> 50mg <6
SALBUTAMOL
IPRATROPIUM BROMIDE
AMINOPHYLLINE
12
Q
What are the values in monitoring after anaphylaxis?
A
A- intubation with bag and mask ventilation
B- Maintain saturations at 94-98%
C- BP- 0-1 mth (50-60mmHg)
- <1 yr (>70mmHg)
- >1-10 yrs 70 + (age x2) mmHg
- > 10 yrs minimum 90mmHg
13
Q
What to do if there is no improvement in anaphylaxis symptoms following initial treatment and monitoring?
A
- repeat fluid challenge
- measure serum mast cell tryptase
- admit patient under paeds team
- educate parents on epi pens and BLS on discharge
14
Q
What are the causes of cardio-respiratory arrest in children?
A
- SEVERE RESPIRATORY DISEASE
- UPPER AIRWAY OBSTRUCTION
- CARDIAC DISEASE (arrhythmias, cardiac failure, myocarditis)
- NEUROLOGICAL DISORDER (birth asphyxia, cerebral oedema, coning (brain/brainstem squeezed through the foramen magnum))
- DRUG/ TOXIN
- SEVERE HYPOXIC-ISCHAEMIC INSULT (suffocation, drowning)
- ANAPHYLAXIS
15
Q
What is the management for cardio-respiratory arrest in children?
A
- Danger?
- Are you okay/ can you hear me? SHOUT FOR HELP
AIRWAY
- head tilt chin lift or jaw thrust. look for obstruction
BREATHING
- look a chest, listen and feel for breathing for 10 seconds
- 5 rescue breaths and if the chest does not rise, airway is not clear
CIRCULATION
- feel for pulse (BRACHIAL IN INFANT, CAROTID IN OLDER CHILD) for 10 secs
- 15 chest compression in lower half of the sternum.
- 1 finger breadth above the xiphersternum,
- 1 hand for toddler
- two fingers for baby
15: 2 at 100-120 bpm
CALL FOR HELP
16
Q
What is the airway recognition in a collapsed child?
A
Assess latency
- looking for chest/abdominal movement
- listening for breath sounds
- feeling for expired air
Vocalisation (e.g. crying/talking) indicates patency
Look for Signs of airway obstruction- foreign body visible? fully obstructed airway will be silent
17
Q
What is the airway response in a collapsed child?
A
Call for help if signs of airway obstruction Basic airway manoeuvres - older child : head tilt chin lift - infant: neutral position - jaw thrust
Airway adjuncts
- Oropharyngeal airway
- Nasopharyngeal airway
Suction secretions
Give oxygen O2
Call an anesthetist for definitive airway management
18
Q
What is the breathing recognition in a collapsed child?
A
Check the effort of breathing
- respiratory rate
- recessions
- accessory muscle use
- flaring of nostrils
- inspiratory and expiratory noises: wheeze, stridor, and crepitations?
- Grunting
- Posture/ position
EFFICACY OF BREATHING
- equal air entry?
- percussion note?
- trachea central?
- gasping?
- SpO2
- Chest movement
EFFECT ON BODY
- heart rate
- capillary refill
- conscious level
19
Q
What is the breathing response in a collapsed child?
A
If not breathing, ventilate with bag and mask
- Give O2 15L/min via a reservoir bag
- Aim O2 saturations 94-98%
- Blood gas (ABG)- usually performed venously or capillary in infants and small children
- Chest X ray
20
Q
What is the circulatory recognition in a collapsed child?
A
VITAL SIGNS
- heart rate
- pulse volume
- blood pressure
SKIN AND MUCOUS MEMBRANE PERFUSION
- capillary refill time (central and peripheral)
- temperature
- colour
ORGAN PERFUSION
- effects on breathing
- mental status
- urine output
21
Q
What is the circulatory response in a collapsed child?
A
Intravenous access (intraosseus needle if cannulation not rapidly established)
- BLOODS (lactate, FBC, U+Es, LFTs, CRP, blood culture, cross match and coagulation studies)
- 12 LEAD ECG
- FLUID BOLUS (20mls/kg) and assess response
22
Q
What is the disability recognition in a collapsed child?
A
- AVPU
- Pupils size and reaction to light
- posture
- blood glucose
- evidence of seizure activity?
23
Q
What is the disability response in a collapsed child?
A
- Protect the airway
- Endotracheal tube if GCS <8
- Recovery position if airway not protected
- Give glucose if hypoglycemia
- Treat seizure activity with benzodiazepines
24
Q
What is the exposure recognition in a collapsed child?
A
- Expose the patient (maintain dignity and minimize heat loss) to assess for injuries, signs of infection, bleeding etc.
- Check temperature and review physiological markers
- Full history and examination
- Drug and fluid chart review
- Investigation results
25
What is the exposure response in a collapsed child?
- Senior/ expert medical advice?
- Management plan
- Documentation
- Communication (SBARR)
- Organise transfer to HDU/ICU
26
What can be classed as choking in a child?
Acute upper airway obstruction can be intrinsic (EPIGLOTTITIS) or extrinsic (FOREIGN BODY) but is most likely aspiration of FB in children
- Large objects are likely to obstruct at the level of carina, which is immediate and dramatic
27
What are the clinical features in a choking child?
- Possibility of foreign body aspiration
- Progressive stridor and malaise (croup/epiglottitis)
- Cyanosis
- Collapse
- Stridor
- Marked subcostal and intercostal recession
- If epiglottis is suspected, DO NOT examine the throat)
28
What is the management for choking in an infant <1
In a SEATED position, support the infant head downwards, prone position to let gravity aid the removal of the foreign body
- Support the head by placing the thumb of one hand at the angle of the lower jaw and 1/2 fingers from the same hand at the same point on the other side of the jaw
- Deliver 5 sharp blows with the heel of your hand to the middle of the back between the shoulder blades.
- After each blow assess to see if the foreign body has been dislodged, if not- repeat up to five times.
After 5 unsuccessful back blows, use chest thrusts:
- turn the infant into a supine position by placing your free arm along the infants back, encircling the occiput with a hand
- identify the landmark for chest compression and deliver five chest thrusts.
29
What is the management for choking in a child >1
- Place small child across lap
- Deliver 5 sharp blows with the heel of your hand to the middle of the back between the shoulder blades.
- After 5 unsuccessful back blows, abdominal thrusts may be used in children over 1 year old
- Stand/kneel behind the child with arms around the torso
- Place clenched fist between the umbilicus and the xiphisternum (with no pressure)
- grasp this hand with the other hand and pull sharply inwards and upwards, repeating up to 5 times
30
What are the possible complications associated with the choking child?
```
Inhaled foreign body:
- BRONCHIECTASIS
- LUNG ABSCESS
- Persistent cough
- Difficulty swallowing
- Sensation of an object present
(do a CXR/ bronchosopy)
```
Iatrogenic:
- abdominal thrusts can cause gastric and splenic rupture, (so abdominal exam)
Hypoxic brain injury and death
31
What are the four main areas for developmental assessment?
1. GROSS MOTOR
2. FINE MOTOR
3. SPEECH AND LANGUAGE
4. SOCIAL
32
How is gross motor development assessed?
- Informal observation and parents
- Obseve lying down and then pull baby to sitting position to observe head control- is the baby able to sit?
- Assess mobility
33
How is fine motor development assessed?
- Assess dexterity and cognition (important to differentiate between the two)
- Observe use of hands (open or closed grasp)
34
How is speech and language development assessed?
- Assess understanding, expression and play.
- Number of words/ level of babbling
- Do they respond to questions?
- Can they carry out simple tasks?
- Always question hearing if there is delay
35
How is social development assessed?
Interaction and everyday skills
36
What are the normal developmental milestones at birth?
GM- when held prone, knees under abdomen
FM- startles to sudden noises
SH- Quiets to voice
S- N/A
37
What are the normal developmental milestones at 6-8 wks?
GM- Raises head to 45o when held prone, sits w/o support
FM- fixes and follows through 90o
SH- Vocalises
S- Social smile
38
What are the normal developmental milestones at 3 months?
GM- Holds head up, loss of palmar grip
FM- reaches with palmar grasp, holds rattle, follows through 180o
SH- Turns to sound at ear level
S- laughs
39
What are the normal developmental milestones at 4-5 months?
GM- Reaches out for toys
FM- plays. hands together
SH- squeals
S- excited by food
40
What are the normal developmental milestones at 6-9 months?
GM- lifts head spontaneously from supine, sits w/o support, crawls
FM- transfers object hand to hand
SH- Babbles, turns to voice
S- Stranger awareness from 9 months, permanence of objects
41
What are the normal developmental milestones at 10-12 months?
GM- Cruising furniture
FM- immature pincer
SH- Double syllable babble
S- Waves bye bye, plays peek-a-boo, empties cupboards
42
What are the normal developmental milestones at 12 months?
GM- Walks unsteadily
FM- Casting, neat pincer grip
SH- single words
S- understands no, gives up a toy
43
What are the normal developmental milestones at 15 months?
GM- Walks steadily
FM- to and fro scribble
SH- N/A
S- Domestic mimicry, indicates wants
44
What are the normal developmental milestones at 18 months?
GM- Run
FM- 2 block tower
SH- points to 1 body part
S- drinks from a cup, can ask for food and drink
45
What are the normal developmental milestones at 2 years?
GM- Jumps, throws overarm
FM- circular scribble, towers 6-7 bricks
SH- 2 word sentences, 50 words, points to 2 body parts
S- has difficulty sharing, feeds self with spoon
46
What are the normal developmental milestones at 3 years?
GM- rides a tricycle, up and down stairs in adult fashion, hops
FM- towers 9-10 bricks, makes a train of bricks, copies circle
SH- name, sex, age on request, 1000 words. 'whats that?' counts to 10, 5 body parts
S- mostly dry by day, dresses and undresses self, understands turn taking
47
What are the normal developmental milestones at 4 years?
GM- Stands on 1 leg for 4 seconds, climbing frame
FM- copies a square and a cross, 10 block tower
SH- tells stories, uses past tense, understands adverbs, counts 1-20
S- Dresses with help, parallel play with other children
48
What are the normal developmental milestones at 5 years?
GM- N/A
FM- copies a triangle
SH- Name, age, address
S- knows what to do if lost, cold, hungry, comforts in distress
49
What are developmental warning signs at any age?
1. MATERNAL CONCERN
2. DISCORDANCE IN DIFFERENT DEVELOPMENTAL AREAS
3. REGRESSION
4. PERSISTING PRIMITIVE REFLEXES
50
What are developmental warning signs at 10 weeks?
No smile
51
What are developmental warning signs at 6 months?
1. Persistent primitive reflexes and squint
2. Hand preference
3. Little interest in people, toys
52
What are developmental warning signs at 10-12 months?
1. No sitting
2. No double syllable babble
3. No pincer grip
53
What are developmental warning signs at 18 months?
1. Not walking
2. <6 words
3. Mouthing and drooling
54
What are developmental warning signs at 2.5 years?
No 2-3 word sentences
55
What are developmental warning signs at 4 years?
Unintelligible speech
56
What is colostrum?
The thin watery milk produced in first few days (high IgG)
57
What is the Let-Down reflex in breastfeeding?
1. Babies suckling stimulates nerve endings in areola
2. Neural reflex (message) is passed to pituitary gland via the hypothalamus
3. Oxytocin contracts the muscle wall of alveoli to release milk during feeding (posterior pituitary)
4. Prolactin stimulates the alveoli to produce breastmilk for future feedings (anterior pituitary)
58
What is the physiology of lactation?
- During pregnancy nipple size, alveoli and ducts increase in response to hormonal changes
- In the third trimester, prolactin sensitizes the glandular tissue which then produces colostrum
- The let down reflex determines the flow of milk and is stimulated by suckling and crying
- Suckling activates afferent nerves and releases oxytocin which acts on smooth muscle
59
What are the advantages of breastfeeding?
- Perfect constituents
- Little risk of contamination
- Anti-infective
- Lowers risk of atopics
- Brain growth and development
- Convenient and no expense
- Bonding
- Exposed to variety of flavours
- Health benefits for mother (CV and DM)
- IQ increase (?)
60
When is breastfeeding contraindicated?
1. HIV positive
2. TB
3. TETRACYCLINES (teeth stain)
4. ANTIMETABOLITES
5. OPIATES
61
What is important to know about formula feeding?
- Based on cow's milk but adapted to meet nutritional requirements
- Casein predominant milks
- Given as supplements if still hungry
- Differences between this and breast milk lie with protein and fat content
- Can be contaminated by bad water
- Expensive and it may increase the risk of allergy
62
What are the recommendations for weaning etc.
0-6 months: Breast or formula
6 months: Introduce solids- puree
7-9 months: More soft foods and encourage finger feeding. Fruit juices in a cup
9-12 months: Mash food. 3 meals a day. 1 with family
1 year: undiluted cow's milk in a cup
63
What is important to know about weaning?
- Healthy infants do not require weaning until 6 months
- cereals and rusks are first solid foods and all of these are gluten free
- can also puree vegetables, fish or meat
- will eventually be able to use a spoon but initially will use fingers
- at 9 months, able to eat at least 1 meal with the family
- give multivitamin preparation at 6 months
- no honey until 1 year old (botulinum toxin)
64
What three elements are assessed on centile charts?
1. Weight
2. Height
3. Head circumference
65
How is head circumference measured?
- using flexible non-stretchable tape
| - obtain three successive measurements and take the largest to be the occipito-frontal circumference (OFC)
66
What is the definition of a neonate?
(TERM)- BIRTH - 28 DAYS
| (PRETERM)- BIRTH- 44 POST MENSTRUAL WEEKS
67
What is neonatal jaundice?
Most commonly due to physiological causes and reflects slow conjugation
68
What are some risk factors for neonatal jaundice?
- PRETERM
- LOW WEIGHT
- FAMILY HISTORY
- MATERNAL DM
- MALE
- EAST ASIAN
69
What are the causes of physiological neonatal jaundice?
- Immature liver function
- Increased erythrocyte breakdown
- baby is generally well
- Bilirubin levels are <200umol/L
70
What are the causes of EARLY neonatal jaundice (<24hrs)?
THIS IS ALWAYS PATHOLOGICAL
- SEPSIS infection: TORCH (toxoplasmosis, other, rubella, cytomegalovirus, herpes)
- HAEMOLYTIC: ABO incompatibility, hemolytic disease of the newborn (rhesus), red cell defects e.g. G6PDD
- HAEMATOMA
- MATERNAL AUTOIMMUNE HAEMOLYTIC ANAEMIA (SLE)
- Gilbert's syndrome
- Crigler-Naijar or Dublin Johnson
71
What are the causes of PROLONGED neonatal jaundice (>14 days in term, >21 days in preterm)?
- BREAST MILK JAUNDICE (starts day 4, usually resolves by 6-24wks.
- -> inhibition of liver conjugation enzymes (protein in breast milk stops infant breaking down bilirubin)
- -> split the bilirubin to rule out conjugated hyperbilirubinaemia
- -> distinguish from hypernatraemic dehydration due to inadequate lactation.
- -> keep breastfeeding
- BREAST FEEDING JAUNDICE= newborns that struggle with breast feeding don't get enough breast milk
- GALACTOSAEMIA= deficiency in an enzyme responsible for adequate galactose degradation.
- INFECTION
- HYPOTHYROIDISM
- HYPOPITUITARISM
- GI: Biliary atresia
72
What are the causes of CONJUGATED neonatal jaundice?
- Neonatal hepatitis
| - Bile duct obstruction
73
What are the clinical features of neonatal jaundice?
- Jaundice first visible in the face, then moves peripherally
- Neurological signs e.g. change in tone, seizures, altered crying (kernicterus)
- hepatosplenomegaly
- petechiae
- microcephaly
- conjugated hyperbilirubinaemia (PALE STOOLS, DARK URINE)
74
What investigations are done in suspected neonatal jaundice?
- Transcutaneous bilirubinometer (TCB) in babies >35 weeks and >24 hours age
- Serum bilirubin
- ultrasound
- hepatobiliary scan (HIDA)
- liver biopsy
- laparotomy
- BLOODS- U+Es, LFTs, TFTs, blood group, infective screen
- haemolytic tests
75
What particular investigations should be done in early neonatal jaundice?
- SEPSIS, LP, septic screen
- Rhesus status
- Direct Coombs test
- Blood group
- U+Es
76
What is the management for neonatal jaundice?
- Monitor the bilirubin level- if present within 24h, URGENT REFERRAL
- Increase fluid intake
- PHOTOTHERAPY- start if rapidly increasing bilirubin or <24 hours. Side effects include dehydration and loose stools
- EXCHANGE TRANSFUSION- umbilical vessel is catheterized and a small volume of blood from the newborn is replaced with double the amount of donor blood and is repeated.
77
What is kernicterus?
Bilirubin encephalopathy
It is where unconjugated bilirubin enters the brain and causes neuronal damage in basal ganglia.
It can lead to irritability, high pitch cry, coma, death or handicap
78
What are the risk factors for kernicterus?
- high serum bilirubin
- preterm birth
- acidosis
- hypoxia
79
How is kernicterus treated?
PHOTOTHERAPY
| EXCHANGE TRANSFUSION
80
What is biliary atresia?
- Conjugated hyperbilirubinaemia
- Absence of intra/extrahepatic bile ducts
- Develops over a period of weeks
- Stools become CLAY COLOURED
81
What are clay colored stools a sign of?
Biliary atresia
82
How is biliary atresia treated?
if detected within the first 6 wks:
| - KASAI PROCEDURE (hepatoporto-enterostomy)
83
What are the causes of jaundice in older children?
- hepatitis/ chronic liver disease
- G6PD
- autoimmune chronic hepatitis
- Reye's syndrome (induced by aspirin)
- paracetamol overdose
- Wilsons disease
- Crigler-Najjar disease
- Gilbert's syndrome
84
What is birth asphyxia/ hypoxic ischemic encephalopathy?
A consequence of intrapartum foetal hypoxia
85
How is birth asphyxia diagnosed?
- pH <7.05 (acidotic)
- depression of APGAR scores (0-5 at 10mins)
- Delay in establishing spontaneous respiration (>10mins)
86
How is mild hypoxic ischemic encephalopathy classified?
- irritable
- high pitch cry
- poor feeding
- normal prognosis
87
How is moderate hypoxic ischemic encephalopathy classified?
- Lethargic
- hypotonic
- poor feeding
- fits
- 40% risk of cerebral palsy
88
How is severe hypoxic ischemic encephalopathy classified?
- diminished consciousness
- no movement
- multiple seizures
- organ failure
- 90% risk of cerebral palsy
- 30% mortality
89
What is the management for birth asphyxia/ HIE?
- rapid resuscitation
- avoid cerebral oedema
- treat convulsions
90
What is the prognosis for birth asphyxia/HIE?
- death and severe handicap (25%)
- controlled therapeutic cooling can improve outcomes
- HIE requires admission to ICU
- 20% of cerebral palsy due to HIE
91
TO KNOW: Pigmented naevi
- usually appear ay 2 years of age
- flat or slightly elevated
- very low malignancy risk unless large congenital naevi
92
TO KNOW: Cafe au lait spots
- uniformly pigmented
- sharply demarcated, macular lesions
- vary greatly in size
- often harmless but associated with neurofibromatosis
93
TO KNOW: Strawberry naevi (superficial hemangioma)
- bright red
- more common in females
- protuberant, compressible, sharply demarcated lesions
- resolve spontaneously
- rapidly increase in size for the first 6 months before eventually shrinking and disappearing around age 7
- don't intervene unless impacting function
94
TO KNOW: naevus flammeus nuchae/simplex (salmon patch/stork marks)
- more common in caucasian
- most common type of vascular birthmark and occur in around 50% of babies.
- small, pink, flat lesion- more noticeable when crying
- nape of neck most common
- usually fade and disappear within 18 months
- forehead marks (up to 4 years)
- back of the neck (do not go away)
95
TO KNOW: mongolian spots
- blue/slate grey lesions in sacral area
- 80% black/asian babies
- fade during the first few years
- usually gone by 4
- harmless
- DOCUMENT as this could be confused with bruising and could raise safeguarding concerns.
96
TO KNOW: Port wine stain (naves flammeus)
- present at birth
- mature dilated dermal capillaries
- lesions are macular, sharply circumscribed
- pink/purple and vary in size
- if located in the TRIGEMINAL AREA- consider (STURGE-WEBER SYNDROME- where there is an underlying meningeal hemangioma and intracranial calcification
97
What is a cephalhaematoma?
- Subperiosteal bleeding
| - swelling and amount of blood loss is limited by periosteum attached to margins of skull.
98
What is a possible complication of cephalhaematoma?
It can make jaundice worse as blood is reabsorbed.
99
What are risk factors for cephalhaematoma?
- forceps delivery
- large baby
- first pregnancy
- difficult and prolonged labours
100
What is the treatment for cephalhaematoma?
- Most resolve in 3 months, but if infection occurs it can be drained.
- BLOOD TRANSFUSION if low red cell count
- PHOTOTHERAPY for any resultant jaundice
101
What is haemolytic disease of the newborn?
It is a disease usually due to rhesus but can be ABO.
| The disease does not occur until the second pregnancy, after the mother has been sensitized with the first.
102
What is the etiology of hemolytic disease of the newborn?
- Maternal IgG crosses the placenta and reacts with antigen of fetal RBCs
- the fetus is RHESUS POSITIVE and the mother is RHESUS NEGATIVE, so in the 1st pregnancy the mother will make anti-Rh antibodies once exposed to foetal blood
- In the 2nd pregnancy these antibodies cross the placenta and destroy RBCs.
103
How does hemolytic disease of the newborn present?
- jaundice
- pallor
- hepatosplenomegaly
- hydrops fetalis (accumulation of fluid in 2 or more compartments)
- polyhydramnios
104
What are the investigations for hemolytic disease of the newborn?
- INDIRECT COOMB'S - at 1st antenatal visit
- ULTRASOUND- hydrops fetalis
- FETAL BLOOD SAMPLING- if Doppler scan finds anaemia then can take blood from HV or cord insertion.
- FBC- anaemia, reticulocytes and if DIC then schistocytes and burr cells, neutropenia and thrombocytopenia.
- BIOCHEM- hypoglycemia and hyperinsulinaemia
105
What is the management in utero for hemolytic disease?
TRANSFUSION WITH GROUP O NEG PACKED CELLS CROSS MATCHED AT 18 WEEKS
- this should be done by umbilical vein rather than intraperitoneal route.
- Deliver at 37-38 weeks but 32 weeks if necessary.
106
What is the management for hemolytic disease after delivery?
- Monitor late onset anaemia at 6-8 weeks
- Moderate disease may require TRANSFUSION
- PHOTOTHERAPY in significant hyperbilirubinaemia to avoid kernicterus
- RESUSCITATION
- INTENSIVE SUPPORT
- TRANSFUSION
- CORRECTION OF ACIDOSIS
107
What are the possible complications of hemolytic disease?
Kernicterus
108
What is the prevention for hemolytic disease?
Anti- D immunoglobulin should be given to ALL rhesus negative women who have NOT been sensitized.
109
What is the definition of prematurity?
<37 weeks from the day of last menstruation.
110
What are the complications in prematurity for the eye?
- Retinopathy of prematurity due to abnormal vascularization of the developing retina
- requires laser treatment to prevent retinal detachment and blindness
111
What are the complications in prematurity for the brain?
- intraventricular haemorrhage
- post hemorrhagic hydrocephalus
- periventricular leucomalacia
- increased risk of cerebral palsy
112
What are the complications in prematurity for the respiratory system?
- RDS
- surfactant deficiency
- apnoea and bradycardia
- pneumothorax
- chronic lung disease
113
What are the complications in prematurity for the cardiovascular system?
- hypotension
| - patent ductus arteriosus
114
What are the complications in prematurity with temperature control?
- increased surface area to volume ratio leads to loss of heat
- immature skin cannot retain heat and fluid efficiently
- reduced subcutaneous fat reduces insulation
115
What are the metabolic complications in prematurity?
- hypoglycemia
- hypocalcaemia
- electrolyte imbalance
- osteopenia of prematurity
116
What are the complications in prematurity for the blood?
- anaemia of prematurity
| - neonatal jaundice
117
What are the complications in prematurity with infections?
- increased risk of sepsis (especially group B strep and coliform)
- pneumonia is common
- infection is a common complication of central venous lines
118
What are the GI complications in prematurity?
- Necrotizing enterocolitis
- GOR
- inguinal hernias (with high risk of strangulation)
119
What are the complications in prematurity for the nutrition?
- May require parenteral nutrition
- nasogastric feeds until sucking reflex develops at 32-34 weeks
- difficult to achieve in-utero growth rates
120
What are the risk factors for prematurity?
- young maternal age
- multiple pregnancy
- infection
- maternal illness
- cervical incompetence
- antepartum haemorrhage
- smoking
- alcohol
- infection
121
What are the potential indications for fetal resuscitation?
- prematurity
- fetal distress
- thick meconium staining of the liquor
- emergency caesarean section
- instrumental delivery
- known congenital abnormalities
- multiple births
122
APGAR SCORE AT 1 MINUTE:
7- 10 : NORMAL
4-6: MODERATELY ILL BABY
0-3: SEVERELY COMPROMISED in need of urgent resuscitation
- Requires intubation and may require cardiac massage
123
What can be used to establish cardiac output?
- IV ADRENALINE
| - BICARBONATE
124
What constitutes as evidence for severe asphyxia?
- Cord blood pH <7
- APGAR score of <5 at 10mins
- Delay in spontaneous respiration beyond 10 mins
- Development of HIE with abnormal neurological signs including convulsions
- Death or severe handicap in >75%
125
What is the value of therapeutic hypothermia?
cool to 33oC for 72 hours
may prevent secondary neuronal damage following moderate/severe asphyxia
126
What is IUGR?
A condition whereby the baby's growth slows or stops in the uterus.
127
What are the major risk factors for IUGR?
- Maternal age >40 years
- >11 cigarettes per day
- cocaine use
- daily vigorous exercise
- previous SGA/stillbirth
- Maternal SGA
- HTN, DM, CVD, renal impairment
- antiphospholipid syndrome
- heavy bleeding similar to menstrual periods
- fetal echogenic bowl
- pre-eclampsia, severe pregnancy induced hypertension, unexplained antepartum haemorrhage
128
What are the differentials associated with IUGR?
- PLACENTAL INSUFFICIENCY: head sparing
| - CHROMOSOMAL ABNORMALITY: uniform restriction
129
How is IUGR diagnosed?
Fetal abdominal circumference or estimated fetal weight <10th centile
If <10th centile or reduced growth velocity, offer serial assessment of fetal size and umbilical artery Doppler scan.
130
What are the investigations/assessments for IUGR?
- Umbilical Artery Doppler Scan- reduces perinatal morbidity and mortality and is the primary surveillance tool.
- Abnormal UADS at 20-24 weeks= serial ultrasound measurement of fetal size and assessment of well-being with UADS commencing at 26-28 weeks
- Normal UADS do NOT require serial measurement unless they develop specific pregnancy complications; antepartum haemorrhage or hypertension
131
What are the UADS criteria?
- Elevated ratio of femoral length to abdo circumference
- Elevated ratio of head circumference to AC
- Oligohydramnios (low amniotic fluid)
132
What is the antenatal management for IUGR?
In a SGA baby between 26+0 and 35+6 where delivery is being considered, they should receive a single course of ANTENATAL CORTICOSTEROIDS.
- 2 doses, 12 hours apart of dexamethasone if <34 weeks gestation.
133
What is the postnatal management for IUGR?
- Paediatrician at birth
- Delay cord clamping for 1 miunte if not compromised
- Keep warm
- Provide respiratory support as required
- NIV (5:20)
- ET tube and surfactant if 27 weeks
- Keep SCBU and measure weight and temperature carefully
- Encourage breast feeding but can use NG/IV
- Benzylpenicillin and gentamycin if septic
- Minimal handling of child but also support parents
134
What is Respiratory Distress Syndrome (RDS)?
Hyaline membrane disease
- Caused by surfactant deficiency. Surfactant is the phospholipid which reduces surface tension in alveoli.
The stress of premature birth and RDS causes corticosteroid release from the adrenals and this will in fact stimulate surfactant production.
THEREFORE this condition is self limiting as endogenous surfactant will resolve the condition in 7 days
135
What can prevent RDS?
ANTENATAL ADMINISTRATION OF CORTICOSTEROID FOR 48 HRS
136
What are the clinical features of respiratory distress syndrome?
- tachypnoea
- intercostal, subcostal and sternal recession
- cyanosis
- expiratory grunting
137
What are the investigations for RDS?
- CXR will show:
- AIR BRONCHIOGRAM
- GROUND GLASS APPEARANCE OF LUNG FIELDS
- BELL SHAPED THORAX
138
What is the management for RDS?
- Titration of O2 against sats
- CPAP (if spontaneously breathing, maintain positive pressure and prevents alveolar collapse
- IPPV INTUBATION- if unable to breather
- EXOGENOUS SURFACTANT- via endotracheal tube.
139
What are the complications associated with RDS?
- pneumothorax
- pneumonia
- intracranial haemorrhage
- hydrocephalus
- patent ductal arteriosus
- necrotising enterocolitis
- retinopathy of prematurity
- chronic lung disease
- cerebral palsy
140
What is the prognosis associated with RDS?
- 40% develop chronic lung disease- additional O2 needed after 28 days. Usually self limiting as O2 requirement falls with age.
141
What is talipes (club foot)?
- Common at birth due to fetal foot position in utero and Achilles tendon is tight. These need to be treated to prevent gait problems.
142
Which neuromuscular problems are associated with (talipes) club foot?
- cerebral palsy
- spina bifida
- arthrogryposis
143
What is the treatment for club foot (talipes)?
- PONSETI technique
- Stretching and manipulation of the joint before setting in plaster cast.
- 10 weeks
- Then wear special boots to prevent recurrence
144
When are immunizations contraindicated?
if a child:
- YOUNGER THAN INDICATED IN THE SCHEDULE
- ACUTELY UNWELL WITH FEVER
- HAS HAD AN ANAPHYLACTIC REACTION TO A PREVIOUS DOSE
145
What are the vaccinations given at 8 weeks?
- Diptheria, Tetanus, Pertussis (DTaP)
- Polio (IPV)
- Haemophilus influenzae type B (Hib)
- Hepatitis B (HepB)
- Pneumococcal
- Meningococcal group B (MenB)
- Rotavirus
146
What vaccinations are given at 12 weeks?
- Diptheria, Tetanus, Pertussis (DTaP)
- Polio (IPV)
- Haemophilus influenzae type B (Hib)
- Hepatitis B (HepB)
- Rotavirus
147
What vaccinations are given at 16 weeks?
- Diptheria, Tetanus, Pertussis (DTaP)
- Polio (IPV)
- Haemophilus influenzae type B (Hib)
- Hepatitis B (HepB)
- Pneumococcal
- MenB
148
What vaccinations are given at one year old?
- Hib and MenC
- Pneumococcal
- MMR (measles, mumps and rubella)
- MenB
149
What vaccinations are given at eligible pediatric groups?
Influenza (both nostrils)
150
What vaccinations are given at 3yr4mths or soon after?
- Diphtheria, Tetanus, Pertussis (DTaP)
- Polio (IPV)
- MMR
151
What vaccination is given to girls aged 12/13 years?
- HPV (2 doses 6-24 months apart)
152
What vaccination is given to 14 year olds?
- Tetanus, diphtheria (Td)
- Polio (IPV)
- Meningococcal groups (MenACWY)
153
TO KNOW: MMR
- Should not be given to children who are severely immunosuppressed
- Should not be given to pregnant girls
- SE: common to have rash and fever 5-10 years later
- mild mumps 2 weeks later
154
TO KNOW: BCG
- Protects against TB
- Given intradermally
- papule forms and often ulcerates
- heals over 6-8 weeks with a scar
155
TO KNOW: Tetanus
- In the case of a dirty wound, give tetanus immunoglobulin with booster if last vaccination was >10 years ago
156
TO KNOW: DTaP/IPV/Hib
- swelling and redness at site
- fever
- diarrhea and/or vomiting
- papule at injection site lasting a few weeks
- irritability for 48 hours
- rarely high fever, febrile convulsions, anaphylaxis
157
Diphtheria
- very rare in developed countries
- caused by CORYNEBACTERIUM DIPHTHERIAE
- throat, forming a pharyngeal exudate, which leads to membrane formation and obstruction of the upper airways
- An exotoxin may cause myocarditis, neutrils and paralysis
158
Tetanus
- Clostridium tetani
- Found in soil, which enters the body through open wounds
- Progressive painful muscle spasms are caused by a neurotoxin produced by the organism
- Respiratory muscle affected results in asphyxia and death
159
Pertussis (whooping cough)
- Bordetella pertussis
- 6-8 weeks
- 3 stages: CATARRHAL, PAROXYSMAL, CONVALESCENT
- Paroxysms of coughing are followed by a whoop ( a sudden inspiratory effort against narrowed glottis) with vomiting, dyspnoea and sometimes seizures
- Complications of pertussis include: bronchopneumonia, convulsions, apnoea and bronchiectasis
- Diagnosis is clinical and can be confirmed by NASOPHARYNGEAL CULTURE
160
Polio
- poliomyelitis virus
- mild febrile illness, progressing to meningitis in some children
- anterior horn cell damage leads to paralysis, pain and tenderness
- respiratory failure and bulbar paralysis
- residual paralysis is common in those who survive
161
Haemophilus influenzae B
- Main cause of meningitis before the vaccine was introduced
| - Led to severe neurological sequelae e.g. deafness, cerebral palsy and epilepsy.
162
Pneumococcal disease
- Streptococcus pneumoniae which can produce septicemia, meningitis and pneumonia
163
Meningococcal C
- purulent meningitis in young children with a purpuric rash and septicemia shock
- complications include: hearing loss, seizures, brain damage, organ failure and tissue necrosis
164
Measles
- MACULOPAPULAR RASH, fever, coryza, cough and conjunctivitis
- complications include ENCEPHALITIS leading to neurological damage and high mortality rate
- usually improves in 7-10 days
- Child must stay off school for 4 days
- avoid contact with pregnant women and immunosuppressed
- paracetamol and water (symptomatic relief)
- Safety net for confusion, coughing blood and chest pain
165
Mumps
- FEVER
- swollen parotid glands
- complications include aseptic meningitis, sensorineural deafness, orchitis and infertility in men.
- resolves in 1-2 weeks
- bed rest, paracetamol, water and soft food should be given
- avoid pregnant women
- Child should stay off school 5 days after first symptoms have developed.
- Safety net for testicular pain, pancreatitis, meningitis
166
Rubella
- Mild illness causing rash and fever
- Harmful to foetus in pregnancy
- Can cause congenital defects such as cataracts, deafness and congenital heart disease
167
Tuberculosis
- Affects lungs, meninges, bones and joints
- cough, tiredness, weight loss, night sweats, hemoptysis and lymphadenopathy
- positive reaction on Mantoux skin testing
- BCG is recommended at birth for babies born where there is high prevalence
168
Hepatitis B
- Cause of acute and chronic liver disease
- Perinatally from mothers, blood transfusion, needlestick injuries and biting insects
- children may be asymptomatic
- Babies born to HBsAg +ve mothers receive vaccination at birth
169
Human papilloma virus (HPV)
- Infection with human papilloma virus is common with over 50% of sexually active women affected
- Two strains of HPV (16 and 18) are the cause of cervical cancer in over 70% of cases.
170
What is the background for meningitis?
- Infection of the meninges covering brain and cord
- 75% of all meningitis is <15y.o.
- Bacterial (1/3) or viral (2/3)
171
What are the risk factors for meningitis?
- CSF shunts
- Spinal procedures
- Diabetes
- Crowding
172
What are the viral causes for meningitis?
1. COXSACKIE (most common virus)
2. ADENOVIRUS
3. EBV
173
What are the main bacterial causes for meningitis?
1. NEISSERIA MENINGITIDES
2. STREPTOCOCCUS PNEUMONIA
3. HAEMOPHILUS INFUENZA
4. MYCOBACTERIUM TUBERCULOSIS
174
Which bacterial causes most commonly affect neonates?
- Group B strep (50%)
- E. coli (20%)
- Listeria (5-10%)
175
Which bacterial causes most commonly affect infants and children?
- Neisseria meningitidis type B (most common gram -ve diplococci)
- Strep pneumoniae (gram +ve elongated cocci)
- Haemophilus influenzae (gram -ve coccibacillary)
176
Which bacterial causes most commonly affect adolescents and adults?
- Neisseria meningitidis
| - Strep pneumoniae
177
What are the other causes of meningitis?
- Disease that is partially treated
- Fungal
- Parasites
- Kawasaki
- Mollaret's (benign recurrent lymphocytic meningitis- chronic inflammation)
178
What are the common features of meningitis- no matter the cause?
- An acute squint
- Petechial haemorrhage
- Kernig's sign in older children (flex hip and extend knee)
- A bulging fontanelle
179
What are the clinical features of viral meningitis?
1. often preceded by pharyngitis or GI upset
2. Infection of mucous membrane to start with
3. lymph node involvement
4. initial viral illness symptoms
5. fever, headache and neck stiffness
6. head retraction is a late feature
7. often indistinguishable from bacterial but may be milder
8. enterovirus, parechiovirus, herpes
180
What are the clinical features of bacterial meningitis?
1. Invasion of nasopharyngeal epithelium
2. invades blood stream then meninges
3. leaky vessels cerebral edema
4. decreased blood flow
5. drowsiness and vacant expression
6. photophobia
7. coma
8. reduction in level of consciousness is unique to bacteria
9. meningeal cry (high pitched)
10. convulsions due to Invasion of nasopharyngeal epithelium
181
What are the clinical features of meningitis seen in neonates?
- fever no focus
- irritability
- seizures
- poor feeding
- respiratory distress
- coma
182
What are the clinical features of meningitis seen in infants?
- fever no focus
- nausea and vomiting
- cold peripheries
- lethargy
- unsettled
- refusing food
183
What are the clinical features of meningitis seen in adolescents?
- unwell
- headache with photophobia
- myalgia
- neck stiffness
- nausea and vomiting
184
What are clinical signs of raised ICP?
- papilloedema
- altered consciousness
- increased BP
- decreased pulse
- bulging fontanelle
- neck retraction
185
What is coning?
- herniation of the brain through the foramen magnum
- can follow an LP
- The release of CSF results in a pressure differential between intracranial and intraspinal compartments
- Causes very acute and severe brainstem signs with paralysis and respiratory inhibition
186
What are some differential diagnoses for meningitis?
- Septicaemia
- Raised ICP
- Menigismus- neck stiffness due to tonsillitis, otitis media, pneumonia, pyelonephritis
187
What are the appropriate investigations for meningitis?
Distinction between bacterial and viral meningitis can't be made clinically, so if suspected:
- LUMBAR PUNCTURE
- PCR
188
When is lumbar puncture contraindicated in the investigation of meningitis?
- If there is suspicion of raised ICP, due to the increased risk of coning.
- shock
- extensive purpura
- after convulsions
- abnormal clotting
- infection at LP site
- respiratory problems
189
What investigations are done if LP is contraindicated (and in addition to LP)?
- FBC, U+E
- CRP
- coagulation
- blood cultures
- glucose
- ABGs
- Urine for MCS
- Nasal throat swab
- CXR
- blood PCR
190
What can you expect to see in a NORMAL LP?
```
Appearance= CLEAR
Cells (x10^6/L)= 0-4
Type= LYMPHOCYTES
Protein (g/L)= 0.2-0.4
Glucose (mmol/L)= 3-6
```
191
What can you expect to see in a VIRAL LP?
```
Appearance= CLEAR/HAZY
Cells (x10^6/L)= 20-1000
Type= LYMPHOCYTES
Protein (g/L)= +
Glucose (mmol/L)= normal
```
192
What can you expect to see in a BACTERIAL LP?
```
Appearance= CLOUDY/ TURBID PURULENT
Cells (x10^6/L)= 500-5000
Type= NEUTROPHILS
Protein (g/L)= ++
Glucose (mmol/L)= decreased
```
193
Features of bacterial meningitis?
- PMNs outnumber monocytes
- papilloedema occurs in late disease
- acute onset
- high lactate
- low glucose in CSF
194
Features of tubercular meningitis?
- Insidious onset
- slight changes in CSF chemistry
- positive tuberculin
- low chloride
195
Features of fungal meningitis?
- insidious onset
- history of lung infection
- yeast cells in CSF
- slight changes in CSF chemistry
196
Features of syphilitic meningitis?
- insidious onset
- slight change in CSF chemistry
- positive RPR test
197
Features of parasitic meningitis?
- acute onset
- slight change in CSF chemistry
- presence of IgM in CSF (Trypanosoma cruz infection= Chagas' disease, sleeping sickness)
198
Features of viral meningitis?
- acute onset
- slight change in CSF chemistry
- monocytes outnumber PMNs
199
Features of subarachnoid hemorrhage?
- red blood cells in CSF
200
Distinguishing meningitis from meningioma?
- X-ray for tumour presence
201
Distinguishing meningitis from meningismus?
- there would be a history of non-CNS viral disease (a non-infective state resembling meningitis)
202
Features of a brain abscess to distinguish from meningitis?
- PMNs may outnumber monocytes
- papilloedema occurs early in disease
- acute or insidious onset
- sterile CSF
203
What is the management for viral meningitis?
- self limiting, symptomatic treatment
| - for HERPES SIMPLEX MENINGOENCEPHALITIS- ACICLOVIR
204
What is the management for bacterial meningitis in children >3 months?
- IV CEFTRIAXONE for 10-14 DAYS
| - DEXAMETHASONE to reduce inflammation
205
What is the management for bacterial meningitis in infants <3 months?
- IV CEFOTAXIME
| - AMOXICILLIN/AMPICILLIN
206
Who should receive prophylaxis in meningitis?
As N. meningitidis in the nasopharynx has a high carrier rate:
- 'KISSING CONTACTS' and children should be given prophylactic RIFAMPICIN
207
What is the dose of corticosteroid given in bacterial meningitis?
Give:
- DEXAMTHASONE (0.15mg/kg (max dose 10mg), four times daily for four days)
IF LP FINDS:
1. Frankly purulent CSF
2. CSF white blood cell count greater than 1000/microlitre
3. raised CSF white blood cell count with protein concentration greater than 1g/L
4. bacteria on Gram stain
208
When should dexamethasone be omitted in meningitis?
- in TB
- MORE THAN 12 HOURS AFTER STARTING ANTIBIOTICS
- CHILDREN UNDER 3 MONTHS
209
What are the immediate complications of meningitis?
- septic shock
- DIC
- raised ICP
- seizures
- pericardial effusion
- cerebral oedema
- hemolytic anaemia
- subdural effusion
210
What are some late complications of meningitis?
- Hydrocephalus
- Acute adrenal failure
- Deafness (hearing test 6 weeks after discharge)
- Major deficit (CP or learning difficulties)
- Focal paralysis
- Further seizures
- 5-10% mortality
211
What is meningococcal septicemia?
Caused by N. meningitidis, a gram -ve diplococcus found in the nasopharynx. It is the leading cause of infectious death in children, which most commonly presents as bacterial meningitis and septicemia.
212
How is meningococcal disease transmitted?
- via droplets (which can also spread to cause pneumonia or otitis media)
- Occurs within 1-14 days of acquisition
- A,B,C,Y,W cause most
213
What are the typical clinical features of meningococcal septicemia?
- fever, headache
- stiff neck, back rigidity, bulging fontanelle, photophobia
- altered mental state, unconsciousness, toxic/ moribund state
- Kernig's sign (pain and resistance on passive knee extension with hips fully flexed)
- Brudzinski's sign (hips flex on head bending forward)
- paresis, coal neurological deficits (cranial nerves/abnormal pupils)
NON BLANCHING RASH
- Scanty petechial rash (red/purple non blanching macule <2mm in diameter)
- purpuric hemorrhagic rash (spots >2mm in diameter)- this may be absent in early phase of the illness and may initially be blanching or macular in nature.
214
What are the clinical features of shock due to meningococcal septicemia?
- toxic/moribund state; altered mental state/decreased conscious level
- unusual skin colour, cap refill >2 seconds; cold hands/feet
- tachycardia and/hypotension; respiratory symptoms or breathing difficulty
- leg pain
- poor urine output
215
What are the early clinical signs of meningococcal septicemia?
- leg pain
- cold peripheries
- mottling
- dyspnoea
216
What are the late clinical signs of meningococcal septicemia?
- confusion
- seizures
- coma
217
What investigations are done in meningococcal septicemia?
- BLOOD CULTURES
- FBC
- WCC/ CRP
- U&Es
- renal function tests
- LFTs
- PCR for N. meningitidis
- DIC- prothrombin time and aPTT is raised, platelet count and fibrinogen is low
- Pharyngeal swab
- Lumbar puncture (following CT scan to rule out raised ICP)- CSF for microscopy, culture, glucose and PCR.
- Aspirates from other sterile sites that are suspected of infections (e.g. joints)
218
What is the management out of hospital for suspected meningococcal disease?
- DIAL 999
if a non-blanching rash is present:
Give parenteral antibiotics whilst waiting for hospital admission
--> benzylpenicllin (600mg/300mg if under 12months)
--> cefotaxime can be used in case of penicillin allergy
without a non-blanching rash DO NOT GIVE ANTIBIOTICS
- -> lumbar puncture can be done before antibiotic administration to help distinguish between bacterial meningitis and other illnesses
- -> in bacterial meningitis, corticosteroids must be given before/with antibiotics, therefore it is more beneficial to wait.
219
In our of hospital management of meningococcal disease, where should the antibiotics be administered
- IM in children (quads)
220
What are the procedures regarding chemoprophylaxis for meningococcal disease in close family contacts?
Give CIPROFLOXACIN 500mg stat.
(half dose if <12, 30mg/kg if <5)
- prolonged close contact with the case (including conjunctivitis) during the seven days before the onset of illness- (MACKIN)
- those who have had a transient close contact with a case- ONLY if they have been directly exposed to large particle droplets/secretions from the respiratory tract around the time of hospital admission.
- Single prophylactic dose of ciprofloxacin can be used for the prevention of a secondary case in pregnancy.
221
Is meningococcal disease notifiable?
YES
| Inform Public Health England
222
What is the management for meningococcal disease in hospital?
For a person with a fever and petechial rash and any of the following:
- petechiae start to spread
- the rash has become purpuric
- signs of bacterial meningitis/ meningococcal septicemia
- the child or young person is ill
GIVE IV CEFTRIAXONE IMMEDIATELY
- aggressive management of raised ICP
- haemofiltration to reduce oedema
- anticoagulants for DIC.
- corticosteroids to reduce hearing loss and neurological sequelae (NOT HIGH DOSE i.e. dexamethasone 0.6mg/kg/day)
- review by a paediatrician
223
What are the antibiotics that should be given in management of meningococcal disease in hospital?
In those < 3 months:
- IV CEFOTAXIME and AMOXICILLIN or AMPICILLIN
- CEFTRIAXONE can also be used as an alternative but not when contraindicated
In those > 3 months:
- IV CEFTRIAXONE
- VANCOMYCIN in those who have travelled outside of the UK or have had prolonged/multiple exposure to antibiotics
224
When is ceftriaxone contraindicated in the treatment of meningococcal infection?
- premature babies
- babies with jaundice
- hypoalbuminaemia
- acidosis
- if calcium containing infusions are being administered
225
When should prophylaxis NOT be offered in meningococcal disease (meningitis and septicaemia)?
- Staff and children attending same nursery or creche
- Students/pupils in same school/class/tutor group
- Work or school colleagues
- Friends
- Residents of nursing/residential homes
- Kissing on the cheek or mouth
- Food or drink sharing (or similar low level of salivary contact)
- Attending the same social function
- Travelling in the next seat on the same plane, train, bus, car etc.)
226
Why is prophylaxis not offered to certain groups in meningococcal disease?
- harm may arise from drug side effects
- development of antibiotic resistance
- eradication of naturally immunizing strains from the nasopharynx
227
What are the early complications associated with meningococcal disease?
- DIC
- AKI
- adrenal haemorrhage
- circulatory collapse
- seizures
- raised ICP
- hydrocephalus
- cerebral venous thrombosis
228
What are the late complications associated with meningococcal disease?
- deafness
- skin scarring
- renal failure
- limb amputations
- gait problems
- cognitive deficit
- incontinence
- developmental delay
229
What is the prognosis/ follow up?
1. 15% have long term neurological complications including deafness and developmental impairments.
2. Offer a formal audiological assessment ASAP
3. inform the GP, health visitor and school nurse about meningitis/meningococcal septicaemia to monitor to late onset complications.
4. Children with recurrent episodes should be assessed by a specialist in infectious disease or immunology
230
What is 'purpura'?
Purplish discoloration of skin due to small superficial vessel bleeding. Can also occur in mucous membranes. Not a disease, but an indication of underlying cause of bleeding.
```
<1cm= petechia
>3cm= bruising/ecchymoses
```
They can be thrombocytopaenic or not- but generally, purport indicates problem with PLATELETS RATHER THAN CLOTTING FACTORS
231
What are the clinical features of purpura on examination?
- Characteristic rash that does not blanch on pressure
- Note nature of lesions: size, confluence, blisters, infection, location
- Always check mucus membranes
- If tender it suggests an inflammatory process
232
What are the clinical features of purpura to elicit in the history?
- AGE of patient: HSP in children, senile purpura in elderly
- Leukaemia and myeloproliferative disorders can occur at any age
- OVERALL HEALTH- fever? breathlessness? pain?
- Purpuric rash in child that does not seem unwell COULD STILL BE MENINGOCOCCAL
- EASILY BRUISED? (Von Willebrand disease, thrombocytopenia, ITP (antibodies breakdown platelets triggered by recent viral infection), HSP, hemophilia, leukemia, vitamin K deficiency.
- RECENT TRAVEL
233
Which conditions in the acutely unwell patient can present with purpura?
1. Acute bacterial sepsis including invasive meningococcal disease (RAPID ONSET)
2. Acute leukemia (ACUTE-SUBACUTE ONSET)
234
Which conditions in all other child patients can present with purpura?
1. Non-accidental injury (VARIABLE ONSET)
2. Idiopathic thrombocytopaenic purpura (ACUTE ONSET)
3. Drugs (co-trimoxazole, quinine, carbamazepine, valproate) (VARIABLE ONSET)
4. Congenital bleeding disorders (hemophilia and VW disease) (CHRONIC HISTORY)
5. Acquired hemophilia (ACUTE-SUBACUTE ONSET)
6. Non-leukaemic bone marrow failure (SUBACUTE)
7. Vitamin deficiency. (SUBACUTE)
8. Raised SVC pressure (ACUTE)
9. Vasculitis (e.g. SLE, HSP, viral infection) (SUBACUTE-CHRONIC)
235
What are the vascular causes of purpura?
CONGENITAL CAUSES
- Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
- Connective tissue diseases (Ehlers-Danlos syndrome and pseudoxanthoma elasticum)
- Congenital cytomegalovirus (CMV) and congenital rubella
ACQUIRED CAUSES
- severe infections (septicaemia, meningococcal infections, measles)
ALLERGIC CAUSES
- HSP
- SLE
DRUG INDUCED CAUSES
- steroids
- sulfonamides
236
What are the thrombocytopaenic causes of purpura?
Impaired platelet production such as:
- Generalised bone marrow failure (leukemia etc)
- Selective reduction in megakaryocytes
Excessive platelet destruction such as:
- immune problems (immune thrombocytopenia)
- coagulation problems (DIC, hemolytic uraemic syndrome)
Sequestration of the platelets as seen in SPLENOMEGALY
Dilutional loss as might be seen following massive transfusion of stored blood.
237
What investigations should be carried out for a presentation of purpura?
- FBC (leukemia)
- ESR
- PLATELETS
- LFTs
- Coagulation screen
- Plasma electrophoresis
- Autoantibody screen for CT disorders
238
What key flags in new purpura can indicate certain causes?
1. A short acute illness = suspicion of sepsis
2. Recent viral illness or immunization in a well child= ITP, HSP, general vasculitic viral rash
3. Rash is often on the lower limbs and in crying/vomiting children around the head and neck = thrombocytopaenia
4. Bruising on the trunk, ears, face which cannot be adequately explained = non accidental injury/ severe congenital bleeding disorder
5. New drug has recently been started = drug related purpura
6. History of recent blood transfusion = post-transfusion purpura
239
What is HSP?
Henoch Schonlein Purpura- inflammation of blood vessel walls
- often preceded by URTI due to Group B strep infection.
- wounds typically affect fronts of legs and buttocks
- may also present with arthritis, GI pain, nephritis with proteinuria
- rash may continue over several weeks
- Complications include CNS bleeding, intussusception or AKI
- Self limiting but may respond to steroids
240
What is DIC?
Disseminated Intravascular Coagulation
Massive ecchymosis with sharp irregular borders of deep purple colour and an erythematous halo.
Can evolve to hemorrhagic bullae and blu-black gangrene
Often symmetrical involving distal extremities, area of pressure, lips, ears, nose and trunk.
241
What is the effect of strong steroids on purpura?
Long term use of strong steroids can cause widespread purpura and bruising on EXTENSOR SURFACES OF THE HANDS, ARMS AND THIGHS.
This is caused by the atrophy of the collagen fibers supporting blood vessels in the skin.
242
What is amyloid purpura?
Typical "pinch purpura" because of its appearance on the cheeks
243
What is factitial purpura?
Where there are episodes of inexplicable bleeding/bruising.
May represent severe emotional or psychiatric disturbance
May also be a sign of abuse.
244
What is the management for purpura?
- Assess all patients for features of serious illness
- If meningococcal disease= parenteral antibiotics immediately and hospital admission
- In all other patients, exclude severe thrombocytopenia
DO NOT GIVE AN IM INJECTION as hematoma will develop
AVOID ASPIRIN and NSAID
245
What is chicken pox?
Varicella Zoster virus infection with outbreaks during the winter/spring, mostly found in under 5s
It is usually self limiting
246
In whom can chicken pox cause complications?
- Immunocompromised (last for weeks, large bleeding vesicles, pneumonia, DIC)
- OLDER AGER
- MALIGNANCY
- DANGEROUS TO FETUS and can cause LRTI in MOTHER
247
How is chicken pox transmitted/incubated?
- Virus enters through respiratory tract and virus in blood occurs 4-6 days later
- Incubation period between exposure and rash ranges from 10-21 days
248
What are the clinical features of chicken pox?
- Pyrexia
- Abdo pain
- Malaise
- Headache
- Vesicles appear over 3-5 days on head, neck and trunk
- Itchy in older children
- Redness may suggest bacterial infection due to scratching
- Vulval lesions may occur in females
249
What is the pattern of rash development in chicken pox?
1. PAPULES
2. VESICLES
3. PUSTULES (fever)
4. SCABS
250
How is diagnosis of chicken pox made?
CLINICAL DIAGNOSIS
| - but PCR can be used.
251
What is the management for chicken pox?
1. Encourage fluid intake
2. Paracetamol
3. Antihistamines and emollients
4. High dose steroids in the immunocompromised, but it new lesions after 8 days, give IV ACYCLOVIR
5. In >12y.o., pregnancy or close contacts, give:
- -> 5-7 days oral acyclovir, 800mg 5 daily
252
What are the complications of chicken pox?
- Viral pneumonia
- Encephalitis- ataxia 1 week post rash
- Secondary skin infection especially group A strep can cause necrotising fasciitis or toxic shock
- Conjunctival lesions
- Fetal varicella syndrome
253
Chicken pox infection in pregnancy
- Can cause prematurity and a 30% risk of death from severe pneumonia or fulminant hepatitis
- If the rash appears within a week before, or two days after delivery, there is risk of neonatal chicken pox
- Virus is transmitted, but the antibody is not
Treat with IgG and acyclovir
254
What can be done for prevention of chicken pox?
- Avoid contact with pregnant women, neonates and anyone who may be immunocompromised, from the appearance of the spots until they are crusted over.
- Children should be kept away from school and air travel for five days
255
Is there a vaccine program for chicken pox?
- Vaccine programme delayed as fear it would increase herpes zoster incidence but given to health workers, children at high risk- or in contact with someone high risk.
- Two doses are given 4 weeks apart
256
What is infective conjunctivitis?
Inflammation of the conjunctivitis, causing dilation of conjunctival blood vessels so the eye appears red.
Can either be limited to the conjunctiva or may occur secondary to other parts of the eye.
In children can be broadly classified into three categories.
257
What are the three categories of conjunctivitis?
1. Neonatal conjunctivitis
2. Infective conjunctivitis
3. Allergic conjunctivitis
258
What is neonatal conjunctivitis?
- Any conjunctivitis in the first 28 days of life
| - Contamination from maternal GU tract.
259
What are the causes of neonatal conjunctivitis?
- Chlamydia (most common)- usually appears by the end of the first week of life.
- Gonococcal- presents with purulent discharge with swelling within the first 48 hours.
260
How is neonatal conjunctivitis treated?
CHLAMYDIA- 2 week course of ORAL ERYTHROMYCIN or DOXYCYCLINE and TOPICAL TETRACYCLINE
GONOCOCCAL- IV CEFOTAXIME (oral if older)
VIRAL- ACYCLOVIR
261
What is infective conjunctivitis?
Conjunctivitis that can be either bacterial or viral
Bacterial conjunctivitis is usually self limiting but can occasionally cause ocular problems.
Viral is more prolonged and can have lasting issues, especially HSV which can lead to keratitis and uveitis.
262
What are the causes of infective conjunctivitis?
1. Staphylococcus. aureus
2. Staphylococcus. epidermidis
3. Strep. pneumoniae
4. Gonococcal
5. Chlamydial
6. Adenovirus
7. HSV
8. HZ
263
How is infective conjunctivitis treated?
- Stop contact lens wearing
- Chloramphenicol
- Fusidic acid
264
What is allergic conjunctivitis?
A recurrent non-infective conjunctivitis.
- the eyes are red, gritty, itchy, burning and tearful
- can cause rapid onset lid swelling and chemosis
- Most commonly type 1 reaction
265
How is allergic conjunctivitis treated?
- Topical antihistamines
| - Topical mast cell stabilizers (e.g. sodium cromoglicate)
266
How does conjunctivitis present?
1. Discomfort
2. Thick discharge
3. Mild photophobia
4. 'Red eye'- uniforms engorgement of all the conjunctival blood vessels
5. Yellow-white mucopurulent discharge and bilateral if bacterial
6. Eyes difficult to open in the morning- glued together by discharge
7. Pain (minimal)
8. Vision is usually normal- smearing on waking is common
9. Visual acuity- mild temporary blur secondary to discharge which can be wiped away
267
What should be considered in a history of conjunctivitis?
- Ask about contact lens use
| - Time course- onset, duration (in chronic cases consider STDs)
268
What should be seen on examination in conjunctivitis?
- Look for evidence of generalized malaise
- Check visual acuity
- EXTERNAL EYE: assess for evidence of orbital cellulitis, blepharitis, herpetic rash, nasolacrimal blockage
- CONJUNCTIVA: look at the pattern of congestion, discharge and for the presence of follicles/papillae
- CORNEA: is there evidence of corneal involvement? Staining is an essential part of the examination.
- Fundoscopy
269
What are the differentials associated with conjunctivitis?
- Blepharitis
- Uveitis
- Acute glaucoma
- Keratitis
- Scleritis
- Episcleritis
- Orbital cellulitis
- Ocular HSV
270
What are the complications associated with conjunctivitis?
- corneal ulceration
- otitis media
- pneumonia
271
What is orbital cellulitis?
An EXTREMELY SERIOUS (potentially life threatening) but uncommon opthalmic emergency characterized by infection of the soft tissue behind the orbital septum.
272
What are the causes of orbital cellulitis?
1. extension of infection from periorbital structures, and from face, lacrimal sac and dental infection
2. Extension of preseptal cellulitis, particularly in young children
3. Direct inoculation of the orbit from trauma
4. Haematogenous spread from distant bacteraemia
273
What organisms are most responsible for orbital cellulitis?
1. Staphylococcus. aureus
2. Strep. pneumoniae
3. Strep. pyogenes
4. Haemophilus influenzae
274
How does orbital cellulitis present?
- Sudden onset of unilateral swelling of the conjunctiva and lids
- Proptosis (bulging of the eye)
- Pain with movement of the eye, restriction of eye movements
- Blurred vision, reduced visual acuity, diplopia
- Pupil reactions may be abnormal- relative afferent pupillary defect (RAPD)
- Fever, severe malaise
275
What investigations are done in orbital cellulitis?
- Routine bloods and blood cultures
- Any discharge from skin breaks should be swabbed and sent to microbiology. Throat swabs and samples of nasal secretions may also help diagnosis.
- CT of the sinuses as well as the orbit and the brain
IF CNS SIGNS
- lumbar puncture
(although contraindicated until a CT scan has rules out raised ICP).
276
What is the management for orbital cellulitis?
- Hospital admission under the joint care of the ophthalmologists and the ENT surgeons
- IV cefotaxime and flucloxacillin in addition to metronidazole
- Penicillin allergy: clindamycin plus ciprofloxacin
- Optic nerve function is monitored every four hours
- Surgery if orbital collection, no response to abx, visual acuity decreases or atyipical.
277
What are the complications of orbital cellulitis?
- OCULAR: exposure keratopathy, raised intraocular pressure, central retinal artery or vein occlusion, endopthalmitis, optic neuropathy
- ORBITAL ABSCESS- leading to blindness
- SUBPERIOSTEAL ABSCESS
- INTRACRANIAL: meningitis, brain abscess, cavernous sinus thrombosis
278
What is preseptal cellulitis?
Infection anterior to the orbital septum
279
What are the causes of preseptal cellulitis?
1. A result of local skin trauma
2. Due to spread from local infection (e.g. sinuses)
3. Spread from distant infections such as from the upper respiratory tract
280
What organisms are most responsible for preseptal cellulitis?
1. Staphylococcus. aureus
2. Staphylococcus epidermidis
3. Streptococci
4. Anaerobes
281
How does preseptal cellulitis present?
- Acute onset of swelling, redness, warmth and tenderness of the eyelid
- Fever, malaise, irritability in children
- Ptosis
282
What are the investigations for preseptal cellulitis?
- Routine bloods and blood cultures
- Any discharge from skin breaks should be swabbed and sent to microbiology. Throat swabs and samples of nasal secretions may also help diagnosis.
- CT of the sinuses as well as the orbit and the brain
IF CNS SIGNS
- lumbar puncture
(although contraindicated until a CT scan has rules out raised ICP).
283
What is the management for preseptal cellulitis?
- Admit for at least 24 hours and rule our orbital cellulitis
- Oral CO-AMOXICLAV
- IV CEFTRIAXONE
- ENT involvement if sinusitis found
284
What are the possible complications of preseptal cellulitis?
- Progression of infection into orbital cellulitis
285
What are the main management steps for any kind of periorbital cellulitis?
EMERGENCY REFERRAL TO SECONDARY CARE is required for:
- All children
- Any patient with any indication of possible orbital cellulitis
- All patients who are systemically unwell
- Occasions where there is doubt over the diagnosis
- A patient not responding to treatment
- When drainage of a lid abscess is required
286
How are food allergies classified?
1. IMMUNOLOGICAL REACTIONS- both IgE (acute, often rapid onset) and non IgE mediated (delayed and non-acute reactions).
2. NON-IMMUNOLOGICAL REACTIONS
287
What is the epidemiology of food allergy?
Though to affect 5 in 100 children
| 2 in 100 develop cow's milk protein allergy
288
What questions should be asked in a history?
- Why is food allergy suspected?
- What foods do they feel are implicated
- What are the symptoms that occur after eating the foods?
- At what age did the symptoms start?
- How much food is needed to cause the symptoms?
- Do symptoms occur every time?
- How long does it take for symptoms to occur?
- What is the worst reaction that the person has had?
- Is there a personal or family history of allergy?
- Feeding history, age of weaning, formula or breast-fed?
- Previous treatments- have any exclusion diets been tried?
- Is their diet nutritionally adequate?
289
What are the presentations of an IgE mediated response to a food allergy?
- acute urticarial
- acute angio-oedema
- oral itching, nausea, vomiting
- colicky abdominal pain
- nasal itching, sneezing, rhinorrhoea
- cough, SoB, wheezing and bronchospasm
- anaphylaxis
290
What are the presentations of a non IgE mediated response to a food allergy?
- Atopic eczema
- gastro-oesophageal reflux
- infantile colic
- stools: loose, blood or mucus
- constipation
- perianal redness
- pallor and tiredness
- faltering growth
- food aversion or avoidance
291
What are the presentations common to both type of response to food allergy?
- Pruritis
- Erythema
- diarrhoea
- abdominal pain
292
Which are the food most commonly involved in food allergies?
- milk
- eggs
- fish and seafood
- peanuts
- sesame
- tree nuts
- soy beans
- wheat
- kiwi fruit
293
What investigations should be done in food allergy?
- Food diary
- Skin prick testing and serum IgE testing (ELIZA and FEIA)
If non IgE then trial elimination diet (normally between 2-6 weeks)
- Food protein- induced enterocolitis (projectile vomiting, diarrhea, and failure to thrive in the first few months of life. Cow's milk and soy protein formulas)
- Eosinophilic oesophagitis and gastroenteritis (nausea, abdominal pain, reflux and failure to thrive)
- Coeliacs disease
294
When should referral to secondary care for food allergy be made?
- Child is faltering growth with GI symptoms
- They have not responded to a single allergen elimination diet
- They had one or more acute systemic reactions or severe delayed reactions
- They have IgE mediated food allergy and concurrent asthma
- There is significant atopic eczema
- There is clinical suspicion of multiple food allergies
- There is ongoing diagnostic uncertainty
295
What is the management for food allergy?
- Food avoidance
- Dietician referral
- Antihistamines
- Medical emergency identification bracelet if at risk of anaphylaxis
- Inform school and family
296
What is the prognosis for children with food allergies?
- Most children grow out of their allergy to eggs, milk, wheat and soya
- Sensitivity to peanuts, seafood, fish and tree nuts is rarely lost
297
What is infectious mononucleosis?
ALSO KNOWN AS GLANDULAR FEVER
298
What is infectious mononucleosis caused by?
Epstein Barr Virus
299
If tonsillitis is present with infective mononucleosis, what are some differentials for this disease?
- Streptococcal infection
- Diptheria
- Leukaemia
- Lymphoma
- Toxoplasmosis and CMV
- Hepatitis
300
What are the clinical features of infective mononucleosis?
- Marked cervical lymphadenopathy
- fever
- sore throat
- enlarged purulent tonsils
- splenomegaly
- macular rash in 15% especially if amoxicillin is prescribed
301
What investigations should be done in infective mononucleosis?
- Supported by presence of atypical lymphocytes (10-25% of WCC)
- Heterophile antibodies positive in 60% of cases in first week of illness
- EBV IgM present in early stages
- LFTs
302
What is the management for infective mononucleosis?
- Self limiting
- Maintain hydration
- Symptomatic treatment
- Steroids if very severe
- NO CONTACT SPORTS due to splenomegaly
- Often recover without prolonged fatigue.
303
What is Kawasaki disease?
An idiopathic and self limiting disease
| Autoimmune mediated systemic vasculitis that affects small and medium sized arteries.
304
What are the diagnostic criteria for Kawasaki disease?
- Fever lasting >5 days
- Marked irritability of the child
- Erythema, swelling and desquamation affecting the skin of extremities
- Bilateral conjunctivitis
- Widespread non-vesicular rash
- Inflammation of the lips, moth and or tongue (STRAWBERRY TONGUE)
- Cervical lymphadenopathy (15mm anterior cervical chain)
305
What are the prominent features of the ACUTE phase of Kawasaki disease?
1-2 WEEKS FROM FEVER ONSET
- highly febrile
- very irritable
- toxic appearing
- Oral changes rapidly following
- Oedema and erythema of feet
- Rash especially common in the perineal area
306
What are the prominent features of the SUBACUTE phase of Kawasaki disease?
2-8 WEEKS FROM FEVER ONSET
- gradual improvement
- fever settles
- desquamation of the perineum, palms, soles
- arthritis, arthralgia
- thrombocytosis
- coronary artery aneurysms
- myocardial infarction
307
What are the prominent features of the CONVALESCENT phase of Kawasaki disease?
MONTHS TO YEARS FROM FEVER ONSET
- resolution of remaining symptoms
- laboratory values return to normal
- aneurysms may resolve or persist
- Beau lines
- Cardiac dysfunction and myocardial infarction may still occur
308
What are some other signs of Kawasaki disease?
1. CARDIOVASCULAR- pancarditis, aortic or mortal competence, tachycardia
2. GI- hydrops of the gallbladder, jaundice, hepatomegaly, diarrhoea
3. BLOOD- mild anaemia
4. RENAL- sterile pyuria, mild proteinuria
5. CNS- aseptic meningitis
6. MSK- arthritis, arthralgia
7. Others- anterior uveitis, BCG site, inflammation
309
What investigations are done in Kawasaki disease?
- No diagnostic test
- Urinalysis -sterile pyuria, mild proteinuria
- Leukocytosis and neutrophils
- ESR, CRP
- Thrombocythaemia
- Elevation of transaminases and bilirubin
- Gallbladder distension on ultrasound
- ECG and ECHO
310
What is the management for Kawasaki disease?
- Inpatient due to cardiac manifestations
- Aspirin
- IV immunoglobulin (gamma globulin) reduces the incidence of coronary artery aneurysm
- Corticosteroids if severe
- PCI or CABG of coronary artery problems
- Anti-TNF and immunosuppressive treatments
- US of heart follow up
311
What are the complications associated with Kawasaki disease?
- Coronary artery aneurysms
- Sudden cardiac death
- Pericarditis/myocarditis
- Valvular disease
- Cardiac dysrhythmia
- Heart failure
- Acute arthritis
312
What are the measles?
Infection from a single stranded RNA Morbillivirus from paramyxovirus family.
- EXTREMELY CONTAGIOUS via respiratory transmission
- Incubation period of 10-12 days, infective 4 days before and after rash
313
What are the clinical features of measles?
- Morbilloform RASH for >3 days, on FOREHEAD, NECK and spreads to TRUNK and limbs.
This fades after 3-4 days. Brownish discoloration and desquamation.
- FEVER for at least one day and at least one:
- -> cough
- -> coryza
- -> conjunctivitis
- -> catarrh (excessive mucus)
- KOPLIK'S SPOTS: buccal mucosa- opposite the second molar teeth, small red spots with a bluish white speck
- SWELLING AROUND EYES and photophobia
- DIARRHOEA
314
What are the investigations for measles?
- Salivary swab or serum sample for measles-specific immunoglobulin (IgM) taken within 6 weeks of onset
- RNA detection in salivary swabs or other samples
315
What is the management for measles?
- SELF- LIMITING - symptomatic treatment with paracetamol and fluids
- NOTIFIABLE DISEASE- vulnerable contacts should be identified for post-exposure prophylaxis
- Stays off school for 4 DAYS from when rash appeared
- Avoid pregnant and immunocompromised people
316
What are the complications for measles?
1. Bronchopneumonia
2. Giant cell pneumonitis
3. Cervical adenitis
4. Otitis media
5. Encephalitis
6. Vitamin A deficiency and blindless
7. Lymphopenia
8. Miscarriage, low birth weight, preterm birth
317
What are the available prevention options for measles?
- Vaccine and vitamin A are effective interventions
- MMR vaccination within 72 hours of exposure
- Ig within 5 days exposure for immunocompromised children and adults
- Pregnant women may be considered for IM Ig if likely susceptible
318
What is rubella?
A viral infection seen mainly in spring and summer. It is the RNA virus RUBIVIRUS TOGAVIRIDAE
- It is transmitted as airborne droplets between close contacts
- Incubation period is 14-21 days with patients being infections for 7 days before and 4 days after symptoms
- Maternal infection leads to CONGENITAL RUBELLA SYNDROME
319
What are the clinical features of rubella?
- Lack of energy
- Low grade fever
- headache
- mild conjunctivitis
- anorexia with rhinorrhoea
- pink, discrete macules--> starting behind the eyes and on the face, spreading to the trunk, and then the extremities
- cervical, subocciptal and post auricular lymphadenopathy
- petechiae on the soft palate (FORCHHEIMER'S SIGN) but not diagnostic
- rash develops 14-17 days after exposure to the virus
320
What are the investigations for rubella?
- Serological and PCR testing
| - FBC- low WBC, increased lymphocytes and thrombocytopenia
321
What is the management for rubella?
- off school for FIVE DAYS after the rash appears
- Paracetamol or ibuprofen
- fluids
- AVOID aspirin in children
- AVOID contact with pregnant women
322
What are the complications in rubella?
- Rubella encephalopathy may occur 6 days after the rash
- Arthritis and arthralgia
- Thrombocytopenia
- Guillain- Barré syndrome/neuritis
- Panencephalitis
323
What are the preventative measures for rubella?
- Excluded from school for 5 days after onset of the rash
- Vaccination via MMR in the second year of life plus a preschool booster, with antenatal screening for rubella susceptibility
- Immunisation after delivery offers protection for future pregnancies
324
What are the main characteristics of rubella in the mother (for rubella in pregnancy)?
- Incubation period of 14-21 days and self limiting
- Macular rash, prodrome and posterior auricular lymphadenopathy
- Arthralgia in wrist and hands
- Risk of first trimester miscarriage
325
What are the TRANSIENT clinical features of rubella in the fetus (for rubella in pregnancy)?
- Intrauterine growth restriction
- Thrombocytopenia purpura (blueberry skin)
- Hemolytic anaemia
- Hepatoslenomegaly
- Jaundice
- Radiolucent bone disease
- Meningoencephalitis +/- neurological sequelae
326
What are the DEVELOPMENTAL clinical features of rubella in the fetus (for rubella in pregnancy)?
- Sensorineural deafness- rubella= most common cause of congenital deafness
- General learning disability
- Insulin dependent diabetes
- Late onset disease at 3-12 months with rash, diarrhea, pneumonitis and high mortality
327
What are the PERMANENT clinical features of rubella in the fetus (for rubella in pregnancy)?
- Congenital heart disease (patent ductus arterioles or peripheral pulmonary artery stenosis)
- Eye defects: cataracts, congenital glaucoma, pigmentary retinopathy, severe myopia, microphthalmia
- Microcephaly
328
What are the common clinical features that also occur from other viruses in the TORCH group (other than rubella)?
- Pre-term delivery
- Low birth weight
- Anaemia
- Thrombocytopenia
- Hepatitis with jaundice and hepatosplenomegaly
- Microcephaly, mental handicap, seizures and failure to thrive
329
What are the investigations for rubella in pregnancy?
- Detection of specific IgM in saliva samples
- Serological and PCR
- Criteria for postnatal diagnosis in the baby
- --> IgM antibodies do not cross the placenta and indicate a recent infection acquired after birth
- --> Unexpected persistence of rubella IgG
330
What is the management for rubella in pregnancy?
- Termination of pregnancy is usually offered if there is positive IgM in the first 16 weeks of pregnancy
- Cochlear implants and cardiac surgery
331
What prevention is available for rubella in pregnancy?
- Prevention of Congenital Rubella Syndrome through immunization of adolescents and women of childbearing age
- Checking rubella antibody status is part of antenatal care in the UK (for next pregnancy)
- Immunoglobulin is not recommended for the protection of pregnancy women exposed to rubella
332
What are the normal values for haemoglobin in children?
Hb < 11g/dL: 6 months - <5 years
Hb < 11.5g/dL: 5-11 years
Hb < 12g/dL: 12-14 years
333
What is the main cause of childhood anaemia?
- Iron deficiency (due to rapid growth and diet)
334
Where is sickle cell disease more likely found?
Central African origin
335
Where is beta thalassemia more likely found?
- Mediterranean
- Middle Eastern
- Southeast Asian
336
What are the three main ways in which anaemia can be classified?
1. Reduced red blood cell/ haemoglobin production
2. Increased red blood cell destruction (haemolysis)
3. Blood loss e.g. GI blood loss and heavy menstruation in girls
337
What constitutes reduced red blood cell. haemoglobin production?
- Bone marrow aplasia- Fanconi's anaemia, Diamond- Blackfan anaemia
- Bone marrow replacement by tumor cells- leukaemias, secondary metastases
- Bone marrow replacement by fibrous tissue or granulomas
- DEFICIENCY OF IRON
- Deficiency of folic acid (coeliac disease, inflammatory bowel disease and anticonvulsants)
- Deficiency of Vitamin B12- breastfed by vegetarian mother/ pernicious anaemia
- Thalassaemias (mutations in the alpha or beta globin chains)
338
What constitutes increased red blood cell destruction?
GENETIC
- red cell membrane defects- including hereditary spherocytes
- red cell enzyme abnormalities- G6PD
- Haemoglobinopathies- including sickle cell disease, thalassemia.
ACQUIRED
- Isoimmune hemolysis (hemolytic disease of the newborn, blood transfusion reactions)
- Infections (malaria), drugs and toxins
- Disseminated intravascular coagulation
339
What are the clinical features of childhood anaemia?
- Fatigue
- Shortness of breath
- Failure to thrive
- Irritability
- Cardiovascular system- look for exertion tachycardia
- Plot height, weight and head circumference for decreased growth
- Pallor- conjunctivae, nail beds, palmar creases
- Dysmorphic features of Fanconi's anaemia, small stature, small head, frontal bossing, absent thumbs, hyper pigmented skin
- Jaundice
340
What is important to pick up in history taking of childhood anaemia?
- Ethnic origin
- Evidence of blood loss- haempptysis, melaena, hematuria, menorrhagia
- Diet
- Chronic infections
- Drug history
- Familia's history for inherited anaemias
- Recent travel
341
What investigations should be done in chlidhood anaemia?
- FBC including: Hb, haematocrit, mean corpuscular volume, mean corpuscular Hb, mean corpuscular Hb concentration.
- Reticulocyte count (immature blood cells)
- Blood film
- Haemoglobin electrophoresis
- Red cell enzyme studies
- Coombs test
- Bilirubin and lactate dehydrogenase levels
- Folate, vitamin B12 levels
- TFTs
- Iron, ferritin and total iron binding capacity levels
- Bone marrow biopsy
342
What do the results from FBC indicate?
- --> Low MCV= iron deficiency, thalassemia or lead poisoning
- --> High MCV= B12 or folate deficiency or reactive reticulocytes which may be secondary to haemolysis
- --> Normal MCV= marrow failure, anaemia of chronic disease, haemolysis or mixed anaemia
343
What is the management for childhood anaemia?
```
Iron supplements (SODIUM FEREFETATE) for 3 months if cause.
Transfusion is only required if the child is compromised and on the verge of high output cardiac failure
```
344
What is the prescription of sodium ferrate in treating childhood anaemia?
5-11 years old:
- SODIUM FEREDETATE 190mg/5ml THREE TIMES DAILY
345
Which foods are rich in iron?
- Meat
- Beans and lentils
- Eggs
- Fish
- Apricots, prunes and raisins
- Leafy green vegetables
- Oatmeal
- Tuna
- Fortified formula and/or cereal
346
How long does it take for iron levels to return to normal?
- A month or two.
347
Brain tumors:
- 2nd commonest site for tumours in childhood
- 75% are infratentorial or midline
- The most common are ASTROCYTOMAS
- Most are idiopathic
348
What are the five main classifications of brain tumours in children?
1. Gliomas
2. PNETs (primitive neuroectodermal tumours)
3. Congenital
4. Pineal tumours
5. Benign tumours
349
What are examples of gliomas found in children?
- Astrocytic tumour
- Oligodendroglioma
- Ependyoma
- Mixed glioma
- Ganglioma
- Choroid plexus tumour
350
What are examples of PNETs found in children?
- Medullo blastoma
| - Pineoblastoma
351
What are examples of congenital tuners found in children?
- Teratoma
| - Craniopharyngioma
352
What are examples of pineal tumours found in children?
- Germinoma
- Embryonal cell carcinoma
- Choriocarcinoma
- Pineocytoma
- Pineoblasotma
353
What are examples of benign tumours found in children but more common in adults?
- Meningioma
- Acoustic neuroma
- Pituitary tumour
354
What are the clinical features of brain tumours in children?
- HEADACHE, early morning, getting worse, worse when walking
- NAUSEA AND VOMITING
- ABNORMAL GAIT AND COORDINATION
- PAPILLOEDEMA
- MACROCEPHALY
- HYPERREFLEXIA
- VISUA DEFECTS
- SEIZURES
- FAILURE TO THRIVE
- WEIGHT LOSS
355
What investigations should be done in suspected brain tumours in children?
- Urgent 48 hour referral
- MRI is the preferred modality, but CT can also be used
- Contrast to detect damage to BBB
- Excision biopsy
- CSF analysis for pineal tumours (AFP and hCG)
- MRI scans carried out ever 6 months
356
What is the surgical management for brain tumours in children?
- Total resection is best in glioma
- Biopsy if possible
- Phenytoin should be given pre-op to prevent seizures
- Drain or shunt to prevent hydrocephalus
- Resection in <2 years as not eligible for radiotherapy
357
What is the radiotherapy management for brain tumours in children?
- Low doses to localized areas
| - Gamma knife and interstitial seeds
358
What is the chemotherapy management for brain tumours in children?
- Various combinations, but normally VINCRISTINE
| - ETOPOSIDE, CYCLOPHOSPHAMIDE and 5-FLUOROURACIL are also used
359
What are the complications of brain tumours in children?
- Intellectual decline
- Growth hormone deficiency
- Secondary brain tumour if irradiated
- Cavernomas
360
What is the prognosis for brain tumours in children?
- Surgical mortality for craniotomy is 1%
| - 1/3 of all cancer deaths
361
What is hemophilia A?
A bleeding disorder caused by deficiency of FACTOR VIII.
| Type A is 5x more common than type B
362
What is hemophilia B?
A bleeding disorder caused by deficiency of FACTOR IX. It is LESS SEVERE and rarer than type A.
363
What is the etiology of hemophilia?
- The spectrum of severity impacts the presentation age
| - Inheritance is X-LINKED RECESSIVE, affecting MALES born to CARRIER MOTHERS.
364
What is the presentation of hemophilia A in severe disease?
- Neonatal bleeding
- Neonatal intracranial haemorrhage
- History of spontaneous bleeding into joints
- Spontaneous haemarthroses
- Haematuria
365
What is the presentation of hemophilia A in untreated cases of severe disease?
- Arthropathy and joint deformity
- Soft tissue haemorrhage- compartment syndrome and neurological damage
- Extensive retroperitoneal bleeds
- Haematomas
366
What is the presentation of hemophilia A in moderate disease?
- Bleeding following venepuncture
367
What is the presentation of hemophilia A in mild disease?
- Only bleed after major trauma/ surgery
368
What is the presentation of hemophilia B in severe disease?
- Spontaneous hemorrhages and haemarthrosis.
| - Ventouse delivery may produce an enormous haematoma.
369
What is the presentation of hemophilia B in moderate disease?
- Suffer hemorrhage from minor trauma or surgery
| - some times spontaneous haemarthrosis.
370
What is the presentation of hemophilia B in mild disease?
- Unexpected haemorrhage after trauma/surgery
- Bleeding following venesection or circumcision
- Excessive bruising
- Joint problems
- Headache, stiff neck, vomiting, lethargy, irritability and spinal cord syndromes
- hematemesis, melaena
- Hematuria
- Frequent epistaxis
371
What are the physical signs of hemophilia B?
- Painful and swollen joints
- Bleeding into CNS --> neuro signs
- pallor
- dyspnoea
- tachycardia
372
What investigations are done in haemophilia?
- Hb and hematocrit will be reduced if bled
- PT, fibrinogen, vWF will be normal
- APTT= PROLONGED
- Check WCC
- Try imaging in acute situations (e.g. CT head to assess brain bleed)
373
What is the management for haemophilia?
SEVERE: prophylactic recombinant factor weekly infusions
- Bracelet
- Hepatitis immunisations
- Pain relief for joints (but NOT NSAIDS)
- Avoid contact sports
374
What is the management for acute episodes of bleeding in haemophilia?
1. ABCDE
2. Fresh Frozen Plasma (FFP), containing the deficient factor should be transfused
3. Desmopressin
4. Tranexamic acid (which binds to plasminogen- reduces the conversion of plasminogen to plasmin which breaks down fibrin).
375
What are the main complications of haemophilia?
- JOINT DISEASE
| - Haemorrhage and associated complications (dependent on site)
376
How does platelet deficiency (vWF deficiency present)?
- Petechial haemorrhage
| - Ecchymoses
377
How does clotting factor deficiency present?
- haematomas
| - haemarthroses
378
What is Henoch Schönlein Purpura (HSP)?
An IgA mediated autoimmune hypersensitivity vasculitis, most common in
- 4-6 year olds
- females
379
What are the 4 organs affected in HSP?
1. Skin
2. Joints
3. Gut
4. Kidneys
380
What are the risk factors for HSP?
INFECTIONS
- Group A strep
- Myoplasma
- EBV
VACCINATIONS
ENVIRONMENTAL
- Allergens
- Pesticides
- Cold
- Insect bites
381
What are the clinical features of HSP?
- Usually seen during the winter months
- Previous URTI
- Low-grade fever
- Symmetrical, erythematous macular rash on the back of the legs, buttocks and ulnar side of the arms
- Evolves into raised purpuric lesions, which may coalesce and resemble bruises
- Abdominal pain, vomitng and bloody diarrhoea
- Swollen tender knees and ankles
- Renal injury due to IgA deposition
- Scrotal involvement may mimic testicular torsion
- Headaches
- Intussusception in 2-3%
382
What are the investigations done for HSP?
- Primarily clinical diagnosis
- URINALYSIS will show haematuria and proteinuria
- FBC: raised WCC with eosiniphilia
- ESR: raised
- SERUM CREATININE: elevated
- SERUM IgA: increased
- perform an autoantibody screen
- abdominal US and barium enema for diagnosis of intestinal obstruction
- renal biopsy
383
What are the differentials for HSP?
- SLE
- Thrombocytopenia
- Glomerulonephritis
- Acute hemorrhagic edema of infancy (presents with fever, oedema, and targetoid shaped purport on face and limbs)
384
What is the management for HSP?
- It is self limiting
- NSAIDs for joint pain (not in renal insufficiency)
- Steroids, azathioprine, cyclophosphamide and plasmapheresis for renal disease
- Corticosteroids for arthralgia and GI dysfunction
- Plasma exchange sometimes with vasculitis and idiopathic rapidly progressive nephritis
385
What are the complications of HSP?
- Renal involvement is serious in 10%
- End stage kidney disease
- MI
- pulmonary haemorrhage
- pleural effusion
- intussusception
- GI bleeding
- bowel infarction
- seizure
- mononeuropathies
386
What is Hodgkin's lymphoma?
Malignant proliferation of lymphocytes
| - Affects males more than females
387
How is Hodgkin's lymphoma classified?
FOUR SUBTYPES
1. Nodular sclerosing HL (most common)
2. Lymphocyte- rich HL
3. Mixed cellularity HL
4. Lymphocyte- depleted HL
388
What are the hallmark indicators of Hodgkin's lymphoma?
- Presence of clonal Reed-Sternberg cells
- CD30 and CD15 are also expressed
- Increased tires of Epstein Barr antibodies, showing that EBV has a role in pathogenesis.
389
What are the core signs and symptoms of Hodgkin's lymphoma?
- Cervical lymph node enlargement
- painless, rubbery, contagious pattern of spread
- lymph nodes may increase/ decrease in size spontaneously
- Hepato/splenomegaly
- cachexia
- anaemia
SYSTEMIC B SYMPTOMS
- fever
- drenching night sweats
- weight loss of >10% body weight
390
What are the investigations for Hodgkin's lymphoma?
- Lymph node biopsy
- FBC
- U&Es (increased ESR, increased LDH)
- LFTS (usually deranged)
- CXR- mediastinal widening
- CT scan- intrathoracic nodes in 70%
391
What is the Ann-Arbor staging for Hodgkin's lymphoma?
i: confined to a single lymph node
ii: involvement of the nodes on the same side of the diaphragm
iii: involvement of nodes on both sides of the diaphragm
iv: spread beyond the nodes to liver or bone or marrow
divided into A or B
A= absence of symptoms
B= symptoms present
392
What is the management for Hodgkin's lymphoma?
RADIOTHERAPY : IA and IIA
| CHEMOTHERAPY: for more advanced stages
393
What is the standard regime for chemotherapy treatment in Hodgkin's lymphoma?
ABVD
- Adriamycin
- Bleomycin
- Vinblastine
- Dacarbazine
394
What are the complications of radiotherapy use in Hodgkin's lymphoma?
- May increase solid tumor risk
- Lung fibrosis
- Hypothyroidism
- IHD
395
What are the complications chemotherapy use in Hodgkin's lymphoma?
- Myelosuppression
- non-Hodgkin's
- alopecia
- AML
- nausea
- infection
396
What is Non-Hodgkin's lymphoma?
Malignant tumour of lymphoid cells- 70% are B cell origin, 30% are T cell origin.
It is more common that Hodgkin's
397
What is the classification of Non-Hodgkin's lymphoma?
Stage 1: limited to one group of lymph nodes, either above or below the diaphragm
Stage 2: 2 or more lymph node groups are affected- either above or below one side of the diaphragm
Stage 3: Spread to lymph node groups on both sides of the diaphragm
Stage 4: Spread beyond the lymphatic system and is no present in both lymph nodes, and organs/bone marrow
divided into A or B
A= absence of symptoms
B= symptoms present
398
What is the pathogenesis of Non-Hodgkin's lymphoma
- Malignant clonal expansion of lymphocytes which occurs at different stages of lymphocyte development
399
What are the core signs and symptoms of Non-Hodgkin's lymphoma?
- painless and superficial peripheral lymphadenopathy (75%)
- extra nodal presentation is more common in this lymphoma and often involves GI, brain, lung, thyroid and skin.
- SYSTEMIC B SYMPTOMS
- fever
- drenching night sweats
- weight loss of >10% body weight
- pancytopenia
400
What are the investigations for Non-Hodgkin's lymphoma?
- Lymph node biopsy
- FBC
- U&Es (increased ESR, increased LDH)
- LFTS (usually deranged)
- CXR- mediastinal widening
- CT scan- intrathoracic nodes in 70%
401
What are the two main grades of Non-Hodgkin's lymphoma?
LOW GRADE/ INDOLENT: cancer grows slowly, may no experience symptoms for years
HIGH GRADE/ AGGRESSIVE: cancer grows quickly and aggressively
402
What is the management of Non-Hodgkin's lymphoma?
```
Low grade: if symptomless, no management may be needed
High grade: CHOP regime
- Cyclophosphamide
- Hydroxydanorubicin
- Oncovin (Vincristine)
- Prednisiolone
```
as well as
- Rituximab
- AntiCD20 antibody
403
What is childhood leukemia?
It is the most common cancer in children and reaches it's peak for boys aged 3, and girls aged 2.
404
What are the four different types of leukemia?
1. Acute Lymphoblastic Leukaema- ALL (78%)
2. Acute Myeloid Leukaemia- AML (15%)
3. Chronic Myeloid Leukemia- CML
4. Chronic Lymphocytic Leukaemia- CLL
405
What are the risk factors for leukaemia?
- Caucasian
- Boys
- Influenza exposure
- Downs
- Blooms
- Ataxia telangiectasia
- Fanconi's
- Radiation
406
What is the general presentation of leukaemia?
- Anaemia
- Thrombocytopaenia
- hepatosplenomegaly
- lymphadenopathy
- frequent illness
407
What are more specific symptoms associated with leukaemia?
- General malaise
- Prolonged/ recurrent episodes of fever
- irritability
- growth restriction and FTT
- cough, sob, reduced exercise tolerance
- dizziness and palpitations
- bleeding- epistaxis, bleeding gums, easy bruising
- muscular/ bony pain
- constipation
- headaches
- nausea and vomiting
- repeated/ sever common childhood
408
What are the signs of leukaemia?
- pallor (anaemia)
- petechiae, purpura, bruising
- signs related to severe infection
- lymphadenopathy
- hepatosplenomegaly
- expiratory wheeze
- cranial nerve lesions or other focal CNS pathology
- testicular enlargement
409
What are the investigations for leukemia?
- FBC and Blood film: may show pancytopenia, elevated
WCC
- BONE MARROW ASPIRATION AND BIOPSY: leukemia lymphoblasts
- IMAGING
- iMMUNOPHENOTYPING
- LUMBAR PUNCTURE
410
What is the general management for leukemia?
- Individual and family support groups
| - No routine immunisations during therapy and for 6 months afterwards.
411
What is the management for ALL?
- High intensity chemotherapy (via Hickman line)
- Allogenic bone marrow transplant used to eliminate residual leukemia cells
- Myeloablation (cyclophosphamide), followed by transplantation of allogenic haematopoetic stem cells.
412
What is the management for AML?
- Intensive chemotherapy
| - Intense marrow suppression until haematopoietic recovery occurs
413
What is the management for CML?
- Myeloablative haematopoietic stem cell transplantation from fully matched related and unrelated donors
414
What are the early complications of leukemia?
- Neutropenia- overwhelming sepsis
- Thrombocytopaenia (bleeding, pulmonary, GI haemorrhage, stroke)
- Electrolyte imbalance- hyperkalaemia and hyperphosphataemia
- AKI (secondary to hyperuricaemia)
- Acute airway obstruction (secondary to mediastinal thyme mass)
- Leukostasis- stroke, acute pulmonary oedema, heart failure
- CNS involvement- stroke, seizures
415
What are the complications of leukaemia during treatment?
- Tumour lysis syndrome
- Renal or hepatic impairment
- Profoud immunosuppression leading to sepsis (febrile neutropenia)
- Thromboembolism
- Alopecia
- Mucositis causing severe mouth pain
- Hyperemesis
- GI erosion/bleeding
416
What are the late complications of leukaemia?
- Growth hormone deficiency and short stature (cranial irradiation)
- Neurological problems including headache, motor, coordination and cognitive impairment, seizures
- Perioheral neuropathy
- Obesity
- CCF
- Cognitive impairment
- Infertility
- Psychosocial impairment
- Second malignancy
417
What is the prognosis for ALL?
- Overall cure rate of 80%
| - Best for children aged 1-10 years
418
What is sickle cell anaemia?
- Homozygosity for the mutation the causes sickle cell disease.
- Autosomal recessive inherited condition due to a mutation of the beta globin gene.
- Sickle cells have a reduced deformability and are easily destroyed, causing occlusion of the microcirculation and a chronic hemolytic anaemia.
419
What is the classification of sickle cell anaemia?
1. HbSS- SICKLE CELL ANAEMIA: homozygote for the beta S globin with usually a severe or moderately severe phenotype
2. HbS- BETA THALASSAEMIA: severe double heterozygote for HbS and beta thalassemia, and almost clinically indistinguishable from sickle cell anaemia
3. HbSC DISEASE: double heterozygote for HbS and HbC with intermediate clinical severity.
4. HbS- BETA+ THALASSAEMIA: mild to moderate severity, but variable in different ethnic groups
5. HbS- HEREDITARY PERSISTENCE OF FETAL Hb: symptom free
420
What is the sickle cell trait?
- The sickle cell trait protects against malaria
- Highest prevalence of approximately 30-40% in sub-Saharan Africa
- Generally asymptomatic and have no abnormal physical findings.
OCCASSIONALLY
- haematuria
- decreased ability to concentrate urine
- renal papillary necrosis
- splenic infarction
- exertion rhabdomyolysis
- sudden death: induced by hypoxia, dehydration, exercise
- renal medullary cancer
421
How is sickle cell trait investigated?
The only abnormality in laboratory tests is presence of haemoglobin AS on electrophoresis.
422
How should a person with sickle cell trait be managed?
- adequate hydration
| - avoidance of of fluid loss and severe heat to prevent complications.
423
How does sickle cell disease present?
At 3-6 months old:
- anaemia
- jaundice
- pallor
- lethargy
- growth restriction
- general weakness
- increased susceptibility to infections
- splenomegaly
- delayed puberty
424
Why would you urgently refer to hospital in sickle cell disease?
- Severe pain
- Dehydration
- Severe sepsis
- Acute chest syndrome: tachypnoea, signs of lung consolidation
- New neurological symptoms or signs
- Symptoms of signs of acute fall in haemoglobin
- Acute enlargement of spleen
- Marked increase in jaundice
- Haematuria
- Fulminant priapism lasting more than two hours, or worsening or recurrent episodes
425
What are the different forms of sickle cell crises?
1. VASO-OCCLUSIVE CRISES
2. APLASTIC CRISES
3. SEQUESTRATION CRISES
4. ACUTE CHEST SYNDROME
426
What is a vaso-occlusive crisis and how does it present?
OBSTRUCTION OF MICROCIRCULATION BY SICKLED rRBCs, CAUSING ISCHAEMIA
- Precipitated by cold, infection, dehydration, ischemia
- Pain
- Swollen painful joints, tachypnoea, neurological signs, acute abdominal pain, loin pain, retinal occlusion
- Stroke (fits and focal neurological signs, cerebral infarction)
427
What is aplastic crisis and how does it present?
CESSATION OF ERYTHROPOIESIS --> SEVERE ANAEMIA
- Precipitated by infection with parvovirus B19
- Drop in Hb over one week which may need transfusion
- Might be present congestive heart failure
428
What is sequestration crisis and how does it present?
ACUTE INCREASE IN SPLEEN SIZE
- Acute fall of Hb and elevated reticulocytes, together with an acute increase in spleen size
- Causes significant mortality
- Recurrent splenic sequestration is an indication for splenectomy
429
What is acute syndrome in relation to sickle cell disease?
VASO-OCCLUSIVE CRISES AFFECTING THE LUNGS
- New pulmonary infiltrate on CXR combined with fever, cough, sputum production, tachypnoea, dyspnoea
- Lung infections tend to predominate in children
430
What are the investigations done in sickle cell disease?
- FBC: Hb is 60-80g/dL with a high reticulocyte count of 10-20%
- The blood film may show sickled erythrocytes and features of hyposplenism
- Sickle solubility test: turbid solution rather than clear
- Haemoglobin electrophoresis
- Diagnosis is a POSITIVE SICKLING TEST WITH HAEMOGLOBIN S ON ELECTROPHORESIS
- Baseline investigation and monitoring. Imaging if complications.
431
What is the screening process for sickle cell disease?
- Heel prick blood spots are usually collected 3-10 days after birth
- Preconceptual testing for haemoglobinopathies is recommended in high risk groups
- Prenatal diagnosis can be done: amniocentesis, CVS, FBS
432
How can painful crises be managed at home?
- Simple analgesia
- Community support
- Avoid exposure to cold (DACTYLITIS- swollen finger)- common early manifestation
- Increased fluid intake, warmth and rest
- Always look for a cause
433
How is sickle cell disease managed?
- Haemoglobinopathy cards
- Monitored regularly for growth and development
- Parental and patient education: avoid precipitating factors.
- Palpate the spleen
- Provide folate, zinc, and vitamin D supplements
434
How is infection in sickle cell disease managed?
- Oral penicillin prophylaxis should be started at diagnosis
| - Routine childhood vaccination including pneumococcal.
435
How are blood transfusions used in management of sickle cell disease?
- They decrease morbidity and mortality
- Partial exchange transfusion to reduce percentage of Hb S quickly in acute life- threatening complications
- Iron overload is a possible complication of regular transfusions therefore it is important to start iron chelation
436
How is hydroxycarbamide (hydroxyurea) used in management of sickle cell disease?
- It can reduce the frequency of crises
- The episodes of acute chest syndrome
- The need for blood transfusions
- It increases Hb and decreases platelet and white cell counts
437
How is potential stroke managed in sickle cell disease?
Transcranial Doppler ultrasonography should be performed annually in children aged 2-16 years with sickle cell
Exchange transfusion should be performed when a stroke occurs.
438
How is acute chest syndrome treated in sickle cell crises?
- CPAP and exchange transfusion
| - Antibiotics- macrolide with intravenous cephalosporin
439
How is priapism in sickle cell crises, treated?
- Hydration and analgesia
- Adrenaline
- Etilefrine
440
What are the complications associated with sickle cell disease?
- Chronic pain
- Nocturnal enuresis
- Infection
- Stroke
- Priapisms
- Cor pulmonale
- Gallstones
- Retinopathy
- Chronic leg ulcers
- Avascular necrosis of femoral and humeral head
- CKD
- Learning difficulties
441
What is neuroblastoma?
- A SOLID TUMOUR THAT ARISES FROM THE DEVELOPING SYMPATHETIC NERVOUS SYTEM.
- It is an embryonal neoplasm that occurs in the first 2 years.
- They are most commonly adrenal and paraspinal in origin
442
What is the pathogenesis for neuroblastoma?
- Chromosomal and molecular abnormalities
443
What is the oncogene associated with neuroblastoma?
MYCN
444
What are the clinical features of neuroblastoma?
- Loss of appetite
- Watery diarrhea due to vast-active intestinal polypeptide (VIP secretion)
- Vomiting
- Weight loss
- Fatigue
- Bruising due to pancytopenia as a result of marrow infiltration
- Periorbital bruising
- Weakness, limping, paralysis and bladder and bowel dysfunction due to spinal cord compression from paraspinal sympathetic tumors.
- Bone pain
- Mild fever
- Abdominal dissension due to enlarger liver
- Hypertension- due to pressure on the renal artery
- Horner's syndrome due to thoracic lesion
- 'BLUEBERRY MUFFIN BABY'- purpura due to extrameduallry haematopoiesis.
- Dancing eye syndrome
445
What investigations are done in neuroblastoma?
- FBC- anaemia
- ESR raised
- Coagulation tests (PTT)
- Urinalysis: catecholamine by products, homovanillic acid and vanillymandelic acid, neuron-specific enolase
- CT scan
- MRI for paraspinal tumours
- Bone scan to assess secondaries
- METAIODOBENZYLGUANIDE (MIBG) can help detect metastasis
- Biopsy
446
What is the management for neuroblastoma?
LOW RISK
- observed or undergo local resection
HIGH RISK
- surgery (laparotomy)
- chemotherapy (carboplatin, cyclophosphamide, doxorubicin and etoposide)
- radiotherapy (MIBG)
447
What are the complications of neuroblastoma at presentation?
- Cord compression
- Severe HTN
- Renal insufficiency
448
What are complications of neuroblastoma due to chemotherapy and surgery?
- Myelosuppression and immunosuppression
- Hearing loss
- Tumour lysis syndrome- hyperkalaemia, hyperuricaemia, hyperphosphataemia
- Intussusception
- Haemmorhage.
449
What is A-thalassaemia?
Mutation of the alpha globing gene, (4 different genes)
450
What is B- thalassemia?
Beta globin gene defects (2 different genes)
451
What is the aetiology of thalassemia?
- recessively autosomal inherited decreased or absence of synthesis of one of the two polypeptide chains that form haemoglobin molecule. this therefore results in anaemia.
452
What are the signs and symptoms of thalassemia?
- Bony deformities (frontal bossing, prominent facial bones and dental malocclusion
- Marked pallor and slight to moderate jaundice
- Exercise intolerance
- Cardiac flow murmur
- HF secondary to anaemia
- FTT and growth restriction
453
What are the investigations for thalassemia?
- FBC: microcytic hypochronic anaemia
- Serum iron and ferritin raised
- Haemoglobin electrophoresis
- Skeletal survey
- CXR: HF
- ECG
- BM and liver biopsy
454
What is the management for thalassemia?
- Blood transfusion (target is Hb>95)
- Iron chelation: desferrioxamine
- Regular reviews
- Haemoglobinopathy cards
- Family support
455
What are the complications associated with thalassemia?
- Iron overload --> endocrine dysfunction
- Transfusion risks
- Osteoporosis
- Gall stone and gout
- Hepatocellular carcinoma
456
What is Wilms tumour?
The most common intra-abdominal tumour. It is an undifferentiated mesodermal tumour of the intermediate cell mass (primitive renal tubules and mesenchymal cells)
- They usually develop in otherwise healthy children but can be associated with:
- -> Overgrowth syndromes
- -> Trisomy 18, Bloom's syndrome
- -> Wilms with Aniridia Gonadoblastoma and Retardation (WAGR)
457
What is familial Wilms tumour?
```
Hereditary Wilms tumour- is uncommon
All are transmitted in an autosomal dominant manner, caused by mutations in one of at least three genes:
- WT1 (chr11)
- WT2 (chr11)
- WT3 (chr16)
```
458
What are the clinical features of Wilms tumour?
- 95% are unilateral
- Most common presentation is asymptomatic abdominal mass
- Abdominal pain
- UTI
- Haematuria
- HTN
- Respiratory issues due to mets
459
What investigations are associated with Wilms tumour?
- FBC
- renal function
- electrolytes
- urinalysis
- genetic studies
- USS and intravenous pyelogram may show distortion of the renal pelvis
- CT and MRI to assess invasion
460
What is the management for Wilms tumour?
- Nephrectomy followed by chemotherapy
| - Radiotherapy to the flank in stage III
461
How many stages of Wilms tumour are there?
Five
462
What is Stage I Wilms tumor?
- Tumour limited to the kidney and completely excised
- Renal capsule is in tact
- Tumour is not ruptured before or during removal
- The vessels of the renal signs are not involved
- There is no residual tumour apparent beyond the margins of excision
463
What is Stage II Wilms tumour?
- Tumour extends beyond the kidney but is completely excised
- No residual tumour is apparent at or beyond the margins of excision
- Regional extension of the tumour (i.e. penetration through the outer surface of the renal capsule)
464
What is Stage III Wilms tumour?
- Residual tumour confined to the abdomen
- May be tumour positive intraabdominal lymph nodes
- Diffuse peritoneal contamination by the tumour
- Tumour is not completely resectable because of local infiltration into vital structures
465
What is Stage IV Wilms tumour?
- Haematogenous metastases- beyond stage III i.e. to the lung, liver, bone, or brain
466
What is Stage V Wilms tumour?
- Bilateral renal involvement at initial diagnosis
An attempt should be made to stage each side according to the Stage I-IV criteria.
467
What is a venous hum?
- Blowing continuous murmur in systole and diastole
- Heard below the clavicles
- Disappears on lying down
468
What is a pulmonary flow murmur?
- Brief high pitched murmur at SECOND LEFT INTERCOSTAL SPACE
| - Best heard when child is lying down
469
What is a systolic ejection murmur?
- Short systolic murmur at left sternal edge or apex
- Musical sound
- Changes with child's position
- Intensified by fever, exercise and emotion
470
How does aortic stenosis present as a murmur?
- SOFT EJECTION SYSTOLIC MURMUR AT RIGHT UPPER STERNAL BORDER
- Radiates to NECK and down LEFT STERNAL BORDER
- Causes dizziness and loss of consciousness in older children
471
How does atrial septal defect present as a murmur?
- SOFT SYSTOLIC MURMUR AT SECOND LEFT INTERCOSTAL SPACE
- Wide fixed splitting of the second sound
- May not be detected until later childhood
472
How does pulmonary stenosis present as a murmur?
- SHORT EJECTION SYSTOLIC MURMUR AT LOWER LEFT STERNAL BORDER
- Radiates all over chest
- Signs of heart failure may be present
473
How does coarctation of the aorta present as a murmur?
- SYSTOLIC MURMUR ON THE LEFT SIDE OF THE CHEST
- Radiates to the back
- Absent or delayed femoral pulses
- Hypertension
474
Which pathologies present at the UPPER LEFT STERNAL BORDER?
1. Pulmonary stenosis
2. Atrial septal defect
3. Innocent pulmonary flow murmur
4. Tetralogy of Fallot
5. Coarctation of the aorta
6. Aortic stenosis
7. Patent ductus arteriosus with pulmonary HTN
475
Which pathologies present at the UPPER RIGHT STERNAL BORDER?
1. Aortic stenosis
2. Supraventricular aortic stenosis
3. Subaortic stenosis
476
Which pathologies present at the LOWER LEFT STERNAL BORDER?
1. Ventricular septal defect
2. Still's murmur
3. Hypertrophic obstructive cardiomyopathy
4. Tricuspid regurgitation
477
Which pathologies present at the APICAL AREA?
1. Mitral regurgitation
2. Mitral valve prolapse
3. Hypertrophic obstructive cardiomyopathy
4. Vibratory innocent murmur
478
Which pathologies produce SYSTOLIC MURMURS?
1. VSD
2. TR
3. MR
4. PS
5. AS
6. ASD
7. PDA
479
Which pathologies produce DIASTOLIC MURMURS?
1. AR
2. PR
3. MS
480
Which pathologies produce CONTINOUS MURMURS?
1. PDA
2. Venous hum
3. Arteriovenous malformations
481
How should heart murmurs be analyzed?
- INTENSITY (Grade 1-6)
- TIMING (Systolic or diastolic)
- LOCATION
- TRANSMISSION
- QUALITY (musical, vibratory, blowing, harsh etc)
482
What are the 6 grades of heart murmur intensity?
```
Grade 1: barely audible
Grade 2: soft but easily heard
Grade 3: loud but no thrill
Grade 4: thrill
Grade 5 and 6: very loud murmurs which may be audible with stethoscope partly or completely off chest
```
483
What is an 'innocent murmur'?
- They are very common
- Occur due to rapid flow and turbulence of blood through the great vessels and across normal heart valves
- No underlying abnormality
- Most common ones are FLOW MURMUR and VENOUS HUM
484
What are the clinical features of an innocent murmur?
- Sensitive (i.e. change with child's position or with respiration)
- Short duration (not pan systolic)
- Single (no associated clicks or gallops)
- Small (murmur limited to a small area and not radiating)
- Soft (low amplitude)
- Sweet (not harsh sounding)
- Systolic (occurs and is limited to systole)
485
What are the 6 signs to indicate that a systolic murmur is likely to be pathological?
1. Pansystolic murmur
2. Harsh murmur
3. Abnormal heart sounds
4. Early/mid-diastolic click
5. >Grade 3 murmur
6. Heard over upper left sternal border
486
What are examples of innocent murmurs?
- STILL'S MURMUR
- PULMONARY FLOW MURMUR
- VENOUS HUM
- CAROTID BRUIT
- PERIPHERAL PULMONARY STENOSIS
487
What are the features of Still's murmur?
- Mid left sternal border
- mid systolic
- grade 2-3
- twanging string
- musical
- vibratory sound
Differential: VSD
488
What are the features of pulmonary flow murmur?
- Upper left sternal border
- Mid-systolic
- Grade 1-3
- Grating
Differential: PS, ASD
489
What are the features of venous hum?
- Right and/or left infraclavicular
- continuous
- only heard in upright position
- diastolic component
- louder than systolic
Differential: PDA
490
What are the features of carotid bruit?
- Supraclavicular area
- ejection systolic
- grade 2-3
Differential: AS
491
What are the features of peripheral pulmonary stenosis?
- Upper left sternal border
- Grade 1-2
- Radiates to axillae and neck
- Usually disappears by 6 months
Differential: PS
492
What are the congenital heart diseases that present with a murmur?
- Pulmonary valve stenosis
| - Atrial septal defect
493
What are the congenital heart diseases that present with cyanosis?
- Tetralogy of fallot
| - Transposition of the great arteries
494
What are the congenital heart diseases that present with heart failure?
- Ventricular septal defect
- Atrioventricular septal defect
- Patent ductus arteriosus
495
What are the congenital heart diseases that present with shock?
- Coarctation of aorta
| - Aortic valve stenosis
496
What is tetralogy of fallot?
The most common CYANOTIC congenital heart disease
| Commonly seen in Downs syndrome and 22q11 microdeletion (Digeorge's)
497
What are the four anatomical abnormalities associated with tetralogy of fallot?
1. VSD
2. OVERRIDING AORTA
3. PULMONARY STENOSIS
4. RIGHT VENTRICULAR HYPERTROPHY
498
How does the cyanosis occur in tetralogy of fallot?
- Occurs as blood is shunted across to the aorta via the VSD due to outflow obstruction
499
What are the clinical features of tetralogy of fallot?
- Low birth weight
- delayed development
- clubbing
- cyanosis (not present at birth)
- paroxysmal hyper cyanotic spells (restlessness and tachypnoea)
- systolic thrill at lower left sternal edge
- aortic ejection click
- long, loud systolic murmur with a thrill along right ventricular tract
- BOOT shaped heart on CXR
500
What is the benefit of knee-to-chest/ squatting in tetralogy of fallot?
- Calms the infant
- Reduces systemic venous return
- Increases systemic vascular resistance
501
What are the best investigations for Tetralogy of fallot?
ECHOCARDIOGRAM is most comprehensive
| ECG and CXR are also helpful
502
What is the management for Tetralogy of Fallot?
- Full surgical repair within the first year of life
| - Prostaglandin E infusion in early neonatal period if severe cyanosis to keep ductus arteriosus open
503
What are the complications associated with tetralogy of fallot?
- Cerebral thrombosis
- Brain abscess
- Bacterial endocarditis
- CCF
- Sustained VT and aortic root dilation
- Sudden death
504
What is atrial septal defect?
Defect that allows a leg to right shunt of blood
Third most common CHD
Incidence higher in females
Most are sporadic with no cause
They are commonly found in congenital conditions such as Downs, FAS, DM
505
What are the 5 types of atrial septal defect?
1. Patent foramen ovale
2. Secundum ASD
3. Primum ASD
4. Sinus venosus defect
5. Coronary sinus defect
506
What is a patent foramen ovale?
This is a normal communication in fetal life and is space between an appropriately developed septum primum and normal septum secundum
507
What is secundum ASD?
Most common- accounting for 50% to 70% of ASDs
| -Secundum is a defect within the fossa ovalis due to defects in the septum primum
508
What is primum ASD?
Also known as partial AVSD and is associated with the presence of a common atrioventricular orifice.
509
What is sinus venosus defect?
A defect of the tissue that separates the right pulmonary veins from the superior vena cava and adjacent free atrial wall.
510
What is coronary sinus defect?
Tissue separating coronary sinus from the left atrium, allowing a shunt through the defect and coronary sinus orifice
511
What are the clinical features of ASD?
- Usually asymptomatic
- can present with tachypnoea
- poor weight gain
- recurrent pneumonia
- shortness of breath and palpitations if found later in life
- Widely split second heart sound without respiratory variation
- soft systolic ejection in pulmonary area at upper sternal border
- diastolic rumble over lower left sternal edge
512
What are the investigations in ASD?
ECG will show tall p waves and right axis deviation
| Echocardiography
513
What is the management for ASD?
Current practice is to repair defects at 4-5 years.
| This can be done surgically or with transcatheter closure if the defect is small
514
How is VSD classified?
Based on location of the lesion in the septum?
515
What are the types of VSD?
- Perimembranous: inlet, trabecular and infundibular
- Muscular
- Acquired
- Inlet/AV canal
- Subarterial infundibular
516
What are the clinical features of VSD?
- Asymptomatic with small defects
- Reduced feeding, tachypnoea and increased respiratory effort
- Weight gain slower
- Recurrent respiratory infection
- Large defect- cyanosis, pulmonary hypertension
- Harsh pan systolic murmur at the lower left sternal edge, Can also be a left parasternal heave
517
What are the investigations for VSD?
ECG will show biventricular and left atrial hypertrophy
| Echocardiography
518
What is the management of VSD?
FIRST LINE: diuretics for heart failure and high energy feeds to increase calorie intake
- ACE inhibitors to reduce afterload
- Digoxin can be used for it's inotropic effect
Surgical repair if symptomatic
519
What are some of the associated complications of VSD?
- Perimembranous VSD can cause aortic valve prolapse
- Some VSDs can cause outflow obstruction
- Reversal of shunt in Eisenmengers syndrome.
- Bacterial endocarditis
520
What is persistent ductus arteriosus?
The PDA connects the pulmonary artery to the proximal descending aorta and is normal in fetal life. It acts as a shunt to miss the lungs.
After birth the PDA usually closes in 12-18 hours.
It may remain open for >3 months in preterm babies
If it is open for >1 year at full term, this is PDA
521
What are the risk factors associated with PDA?
- Prematurity
- asphyxia
- rubella infection
- genetic syndrome
- vampiric acid during pregnancy
- high altitude births
522
What are the clinical features of PDA?
- Low diastolic pressure
- LRTI
- Poor feeding and growth with FTT due to heart failure
- Tachycardia, tachypnoea and wide pulse pressure
- Systolic thrill may be palpable at upper left sternal border
- CONTINUOUS MACHINERY MURMUR at left infraclavicular area or upper left sternum.
- Bounding peripheral pulses and a diastolic mitral rumble (train through tunnel)
523
What investigations are done in PDA?
- Echocardiogram
524
What is the management for PDA?
- regular echocardiogram
- in well patients, the duct is closed at 1 year old by cardiac catheterization
- surgical ligation urgently if in heart failure or pulmonary hypertension
- Treat HF (diuretics if necessary)
- In preterm or <1 month, ibuprofen, indomethacin
525
What is coarctation of the aorta?
- Localised constriction of the aorta usually at the origin of the ductus arteriosus
- Arterial blood bypasses the obstruction via collateral vessels which enlarge and leads to LVH
- May lead to heart failure
- In severe cases, may lead to collapse in the 1st week when the ductus arteriosus closes
- It is commonly seen in Turner's syndrome
526
What are the clinical features of coarctation of the aorta?
- Poor feeding
- Lethargy
- tachypnoea
- CCF/ shock
- become ill quickly as the DA closes
- pulses are commonly reduced
- radio-femoral delay
- differential cyanosis- upper body pink, but legs are blue
- SYSTOLIC MURMUR in the LEFT INFRACLAVICULAR area, but a range of murmurs can occur due to collateral circulation
- Ejection click
- Associated berry aneurysms
527
What investigations should be done in coarctation of the aorta?
- Antenatal US
- CXR: CCF, indentation of aortic shadow
- Echocardiogram
- U&Es- renal impairment
- BP: HTN in upper limbs, hypotension in lower limbs
- ECG: LVH
- Cardiac catheterization when not clear at the ultrasound and can use stent
528
What is the management for coarctation of the aorta?
- Prostaglandin E1 to open DA
- Treat CCF with diuretics and inotropes.
- Anti-hypertensives for HTN
- Treat post correction HTN with ACE inhibitors
- Balloon angioplasty or surgery
529
What are the complications associated with coarctation of the aorta?
- Recoarctation after repair
- CCF
- Aortic dissection, thoracic aortic artery aneurym and cerebral aneurysm rupture
530
What is transposition of the great arteries (TGA)?
The aorta and pulmonary artery are transposed, so that the aorta arises from the right ventricle and vice versa
Most common cause of cyanotic CHD presenting in the neonate
Most common in males
531
What are the maternal risk factors for TGA?
- rubella infection
- FAS
- aged >40 years old
- DM
532
What are the three subtypes of TGA?
1. TGA with intact ventricular septum
2. TGA with VSD
3. TGA with VSD and pulmonary stenosis
533
What are the clinical features of TGA?
- Cyanosis
- respiratory distress
- if large VSD, commonly diagnosed later due to mixing of blood
- If PS is severe, similar presentation to ToF
534
What investigations are done in TGA?
- Antenatal US
- Pulse oximetry shows low O2 saturations
- CXR: egg on string appearance, cardiomegaly
- ECG: RVH and RAH
- Echocardiogram
535
What is the management for TGA?
- Prostaglandin E1 infusion to open DA
- Transfer to cardiac centre
- Balloon atrial septostomy if intact septum to improve mixing
- Arterial switch surgery in the first week of life
536
What are the complications associated with TGA?
- Neopulmonary stenosis
- Neoarotic regurgitation
- Neoaortic root dilatation
- Coronary artery disease
- Sudden cardiac death
- Neurodevelopmental delay: Low gestation age and lactate are most important predictors of developmental outcome.
537
How is asthma in children described?
Chronic inflammatory disease of airway leading to obstruction and:
- Wheeze
- Cough
- Dyspnoea
- Chest tightness
538
What is the presentation of asthma in children?
WHEEZE AND DYSPNOEA
- Frequent
- Worst at night and early morning
- Have specific triggers
ATOPIC DISEASE
NOCTURNAL COUGH WHICH IS ALSO WORSE WITH EXERCISE
539
What are the physical signs of asthma in childhood?
- Widespread wheeze
- Increased work of breathing and signs of respiratory distress
- Response to bronchodilators
- Barrel chest
540
What are the features that increase the probability of childhood asthma?
More than one of:
- wheeze
- cough
- difficulty breathing
- chest tightness
With
- frequent and recurrent symptoms
- worse at night and early morning
- occurring in response to exercise/trigger
- occurring separately from other cold symptoms
- Personal history of atopy
- Family history of atopy or asthma
- Widespread wheeze on auscultation
- History of symptom of lung function improvement after therapy
541
What features decrease the probability of childhood asthma?
- Symptoms only occurring in conjunction with colds; no interval symptoms
- Isolated cough without wheeze or breathing difficulties
- History of moist cough
- Prominent dizziness, light-headedness, peripheral tingling
- Normal respiratory examinations when symptomatic
- Normal lung function tests (including peak flow) when symptomatic
- No response to asthma treatment
- Clinical features suggestive of an alternative diagnosis
542
What investigations should be done with a high probability of asthma?
- Trial of treatment and reassess in 2-3 months
| - Investigate further those who show a poor response
543
What investigations should be done with a low probability of asthma?
- Detailed investigations and referral especially when alternative diagnosis seems likely
544
What investigations should be done with an intermediate probability of asthma?
- Watchful waiting with review
- Trial of treatment with review- where beneficial, treat as asthma
- Spirometry and reversibility testing
545
What investigations should be done in general with asthma in children over 5?
- Spirometry
- Bronchodilator reversibility (in those with FEV1/FVC <70%)
- PEFR: monitor with diary and check reversibility
546
When should children be referred for a potential diagnosis of asthma?
- Unclear diagnosis
- Symptoms from birth
- Vomiting
- Severe URTI
- Productive cough
- FTT
- No response to treatment
547
What is the general management for childhood asthma?
- Allergen avoidance
- Smoking cessation of caregivers
- Education of family
548
What are the 4 steps of asthma management in under 5s?
Step 1: SABA (salbutamol)
Step 2: SABA + ICS (200-400mcg daily)
Step 3: Add LTRA (montelukast)
Step 4: Stop LTRA and refer to paediatrician
549
What are 5 steps of asthma management in 5-12s?
Step 1: SABA (salbutamol)
Step 2: SABA + ICS (200mcg)
Step 3: Add LABA (salmeterol). If no response, STOP LABA and increase ICS, and add LTRA/ slow release theophylline
Step 4: Increase ICS (400mcg)
Step 5: Oral corticosteroid and refer to paeds
550
What are the side effects of long term steroid use in childhood asthma?
1. Growth stunting
2. Immunosuppression
3. Thrush
551
What are the different routes of administration for asthma medication?
- NEBULISER (used in <2s and in severe attacks, delivered via face mask).
- SPACERS (toddlers 2-8, clean with warm soapy water)
- DRY POWDER SYSTEMS (school age child)
- METERED DOSE INHALER (only in >8)
552
What should be assess in an asthma review?
- Check family's understanding
- Inhaler technique
- Environment (smoking)
- Check diary, symptoms, treatment response, severe attacks, school absences and ADLs
- Height and wight
- Chest exam
- PEFR
553
What constitutes complete control in childhood asthma management?
- No daytime symptoms
- No night-time waking due to asthma
- No need for rescue medication
- No asthma attacks
- No limitations on activity including exercise
- Normal lung function
- Minimal side effects from medication
554
What are the 4 steps to take if a child is having an acute asthma attack?
1. Help them sit up straight and keep calm
2. Help them take one puff of their reliever inhaler (usually blue)
3. Call 999 if: symptoms get worse with inhaler
4. Repeat step 2 if ambulance takes longer than 15 mins
555
What should be assessed and recorded in an acute asthma attack?
- Pulse rate
- Respiratory rate
- Degree of breathlessness (ability to speak/feed)
- Use of accessory muscles of respiration
- Amount of wheezing
- Degree of agitation and conscious level
556
What constitutes a moderate asthma attack?
- Increasing symptoms
- PEF >50-75% of best or predicted
- No features of acute severe asthma
Age < 5 years
- Heart rate <140/min
- Resp rate <40/min
Age > 5 years
- Heart rate <125/min
- Resp rate <30/min
557
What constitutes an acute severe asthma attack?
- PEF 33-50% best or predicted
- respiratory rate >25/min
- heart rate >110/min
- inability to complete sentences in one breath
Age < 5 years
- Heart rate > 140/min
- Resp rate > 40/min
Age > 5 years
- Heart rate > 125/min
- Resp rate > 30/min
558
What constitutes a life-threatening asthma attack?
- PEF <33% best or predicted
- SpO2 <92%
- PaO2 <8kPa
- normal PaCO2 (4.6-6.0 kPa)
- silent chest
- cyanosis
- poor respiratory effort
- arrhythmia
- exhaustion, altered conscious level
- hypotension
559
What constitutes a near fatal asthma attack?
- Raised PaCO2 and/or requiring mechanical ventilation with raised inflation pressures
560
What investigations should be done in acute asthma attacks?
- PEFR (use best of three readings)
- O2 Saturations
- CXR and ABG
561
What is the management for an acute asthma attack?
ABCDE
Transferred to hospital ASAP
1. SpO2 <94%, then high flow oxygen via NRBM
2. Salbutamol (inhaler/ 2.5-5mg nebulizer)
3. Steroid therapy (oral pred [20mg 2-5yo], [30-40mg >5])
4. IV Salbutamol bolus (15ug/kg)
5. Nebulised ipratropium bromide
6. IV aminophylline with severe bronchospasm
7. IV magnesium sulphate
8. HDU/ICU admission
562
What is pneumonia?
Infection of the lung parenchyma. The alveoli fill up with fluid or pus, causing cough with phlegm or pus, fever, chills, difficulty breathing. Pathogens causing pneumonia vary according to the age of child.
563
What are the pathogens responsible for pneumonia in neonates?
- Female genital tract
- Group B strep,
- E.coli
- Gram negative bacilli
- Chlamydia trachomatis
564
What are the pathogens responsible for pneumonia in infants-preschool?
BACTERIAL (60%)
- Steptococcus pnuemoniae
- Staphylococcus aureus
- H. influenza
VIRAL (40%)
- Parainfluenza
- Influenza
- Adenovirus
- RSV
565
What are the pathogens responsible for pneumonia in older children- adolescents?
BACTERIAL (60%)
- Steptococcus pnuemoniae
- Staphylococcus aureus
- H. influenza
VIRAL (40%)
- Parainfluenza
- Influenza
- Adenovirus
- RSV
ATYPICAL
- Mycoplasma pneumoniae
- Chlamydia pneumoniae
566
What are the pathogens responsible for aspiration pneumonia in children?
- Enteric gram negative bacteria
- Steptococcus pnuemoniae
- Staphylococcus aureus
567
What are the pathogens responsible for pneumonia in the non-immunized?
- Haemophilus influenza
- Bordatella pertussis
- Measles
568
What are the pathogens responsible for pneumonia in the immunocompromised?
BACTERIAL
- Pneumocystis carinii
- Tuberculosis
VIRAL
- CMV
- VZV
- HZV
- Measles
- Adenoviruses
569
What conditions are associated with pneumonia?
CHRONIC LUNG DISEASE: Ex- prom, cystic fibrosis, sickle cell
CONGENITAL CARDIAC ABNORMALITY: Large left- right intracardiac shunt
- CHRONIC ASPIRATION: cerebral palsy, tracheooesophageal fistula, GORD
- KARTAGENER SYNDROME: Ciliary dysfunction, bronchiectasis, dextrocardia
570
What is the presentation for pneumonia?
- Fever
- Tachycardia
- Tachypnoea
- Cough
- Sputum
- Vomiting post coughing
- Poor feeding
- Diarrhoea
- Preceding URTI
571
What are the consolidation signs associated with pneumonia?
- Decreased breath sounds
- Dullness to percussion
- Increases tactile/vocal fremitus
- Bronchial breathing
- Coarse crepitations
572
What are the signs of respiratory distress?
- Cyanosis
- Grunting
- Nasal flaring
- Marked tachypnoea
- Chest indrawing (intercostal and suprasternal recession)
- Subcostal recession
- Abdominal see-sawing
- Tripod positioning
- Reduced oxygen saturation
573
What investigations should be done in pneumonia?
- CXR: Focal consolidation= bacterial cause, diffuse consolidation bronchopnuemonia= viral cause
- BLOODS: Increased WCC, increases ESR/CRP, U+Es, cultures, mycoplasma serology
- URINE: legionnaire's antigen
- MICROBIOLOGY: Blood and sputum MC and S
- BLOOD FILM: RBC agglutination by mycoplasma
- IMMUNOFLUORESCENCE: Can detect RSV on nasopharyngeal aspirate
574
What are examples of severe respiratory symptoms or showing signs of sepsis in pneumonia?
- Oxygen saturation <92%
- Grunting, marked chest recession, or RR >60
- Cyanosis
- Child looking seriously unwell, very lethargic
- A temperature of >38C if <3 months
- While waiting give oxygen
575
When should you consider referral for children with pneumonia?
- A temperature of >39C in aged 3-6months
- TACHYCARDIA:
- - >160 beats/min in a child <1 year
- - >150 beats/min in a child aged 1-2
- - >140 beats/min in a child aged 2-5
- Inadequate oral fluid intake (50-75% of usual)
- Pallor of skin, lips or tongue
- Abnormal response to social cues
- Waking only with prolonged stimulation
- Decreased activity
- Nasal flaring
- Clinical dehydration
576
For children who do not ned admission in pneumonia:
- ALL children with pneumonia should receive abx, as bacterial/viral are not easily distinguishable
- If <2 with mild symptoms of LRTI do NOT susally have pneumonia and do NOT need abx, but should be reviewed if symptoms persist
- Amoxicillin should be prescribed.
577
What advice should be given at home for children with pneumonia?
- Use either paracetamol of ibuprofen to treat a distressed child with fever.
- No more than 4 doses or either paracetamol or ibuprofen in a 24hr period.
- Encourage fluids regularly, advised continued breast feeding
578
How should parents be safety netted for children with pneumonia?
- Check on child regularly, including through the night
- Seek medical advice if:
- - breathing rate increases or there are any episodes of apnoea, cyanosis or increased respiratory effort
- - signs of dehydration
- - less responsive/ difficult to rouse
- - persistent/worsening fever.
579
Which antibiotics should be prescribed in pnuemonia?
- ORAL AMOXICILLIN OR ERYTHROMYCIN
- IV CEFUROXIME AND/OR ERYTHROMYCIN (in severe)
- METRONIDAZOLE (aspiration)
580
What should be done in respiratory failure from pneumonia?
- CPAP
- BiPAP
- PICU transfer
581
Which immunisations should be given to protect from pneumonia?
- Hib
- Pneumoncoccal
- Influenza
582
What are the complications associated with pneumonia in children?
- Pleural effusion
- Empyema
- Lung abscess
- Septic shock
- Acute respiratory distress syndrome
- Acute respiratory failure
583
What is bronchiolitis
- Lower respiratory tract infection in infants
- 2-6 months old.
- Viral infection of the bronchioles
584
Which virus is most likely to cause bronchiolitis?
RESPIRATORY SYNCYTIAL VIRUS
- hMPV
- adenovirus
- parainfluenza
585
What are the risk factors associated with bronchiolitis?
- older siblings
- nursery
- passive smoke
- prematurity
- low weight
- congenital heart disease
- hypotonia
- pharyngeal discoordination
- DM
- Down's
586
What are the clinical features associated with bronchiolitis?
- SOB
- cough
- decreased feeding
- irritability
- apnoea
- wheeze and bilateral crepitations
- signs of respiratory distress
- tachypnoea
- tachycardia
- fever
- cyanosis
- dehydration
587
What investigations are done in suspected bronchiolitis?
- Clinical diagnosis
- Pulse oximetry
- Nasopharyngeal aspirate: RSV and other viral cultures
- Routine bloods, cultures, ABGs
CXR not recommended
588
How do you determine the severity of the child's condition with bronchiolitis?
- Temperature
- Examine chest, heart rate, respiratory rate, pulse, BP
- Oxygen sats in room air
- degree of agitation and consciousness
- Signs of exhaustion, cyanosis
- Assess hydration status but CR time, skin turgor, dry mucous membranes
589
When should you admit for bronchiolitis?
- Apnoea
- Child looks seriously unwell
- severe respiratory distress
- central cyanosis
- persistent oxygen saturation of less than 92% when breathing air
590
When should you consider referral for bronchiolitis?
- Respiratory rate >60
- Difficult with breastfeeding or inadequate oral fluid intake
- Clinical dehydration
591
What would you say to parents when would the child not require hospital admission for bronchiolitis?
BRONCHIOLITIS IS SELF-LIMITING AND SYMPTOMS TEND TO PEAK AROUND 3-5 DAYS.
592
What advice should be given at home for children with bronchiolitis?
- Use either paracetamol of ibuprofen to treat a distressed child with fever.
- No more than 4 doses or either paracetamol or ibuprofen in a 24hr period.
- Encourage fluids regularly, advised continued breast feeding
593
How should parents be safety netted for children with bronchiolitis?
- Check on child regularly, including through the night
- Seek medical advice if:
- - breathing rate increases or there are any episodes of apnoea, cyanosis or increased respiratory effort
- - signs of dehydration
- - less responsive/ difficult to rouse
- - persistent/worsening fever.
594
What is the management for bronchiolitis?
```
It is a self limiting disease and can be managed at home.
- Give supportive treatment with attention to fluids
It usually lasts for 7-10 days
Refer to the hospital if:
- Poor feeding
- Lethargy
- Apnoea
- RR >70
- Respiratory distress
- Cyanosis
- Sats <94%
```
595
What is the available treatment at the hospital?
Very similar to treatment at home, with emphasis on supportive treatment
Oxygen (vapotherm- humidified warm oxygen up to 40L) and NG feeding (12mls/hr) where necessary.
596
When are vaccines against RSV given for babies?
- Premature babies
- lung, heart or neurological disease
- immunocompromised
597
What is croup?
Self limiting and mild viral URTI
- Nasopharyngel inflammation which can spread to the larynx and trachea
- Subglottal inflammation and can compromise the airway
- Affects children from 6 months to 3 years
- Lasts for 3-7 days
598
Which pathogen is most responsible for croup?
Parainfluenza virus
599
What are the clinical features of croup?
Start as symptoms of an URTI, but progresses to:
- BARKING COUGH
- Hoarseness
- Stridor
- Decreased air entry but normal sounds
- Respiratory distress
600
How is croup scored?
WESTLEY SCORING SYSTEM
601
What is the Westley scoring system for croup?
INSPIRATORY STRIDOR
- Not present= 0 pts
- When agitated= 1 pt
- At rest = 2pts
INTERCOSTAL RECESSION
- Not present = 0 pts
- Mild= 1 pt
- Moderate = 2 pts
- Severe = 3 pts
AIR ENTRY
- Normal = 0 pts
- Mildly decreased = 1 pt
- Severely decreased = 2 pts
CYANOSIS
- None = 0 pts
- With agitation/activity = 4 pts
- At rest = 5 pts
LEVEL OF CONSCIOUSNESS
- Normal = 0 pts
- Altered = 5 pts
602
What do the Westley scores for croup indicate?
0-3= MILD CROUP
4-6 = MODERATE CROUP
> 6 = SEVERE CROUP
603
When should you suspect croup in a child?
- SUDDEN ONSET
- seal like barking cough
- accompanied by stridor and intercostal/ sternal recession
- symptoms worse at night and increase with agitation
- prodrome
- hoarse voice
604
What are the differential diagnoses associated with croup?
1) BACTERIAL TRACHEITIS
2) EPIGLOTTITIS
3) FOREIGN NODY IN UPPER AIRWAY
4) RETROPHARYNGEAL/ PERITONSILLAR ABSCESS
5) ANGIONEUROTIC OEDEMA
6) ALLERGIC REACTION
605
What investigations should be done in someone with croup?
- Categorize the severity of the symptoms
| - Consider the need for hospital admission (all those with moderate, severe or impending respiratory failure and RR>60)
606
When would a child with mild croup symptoms require hospital admission?
- - chronic lung disease
- - haemodynamically stable
- - neuromuscular disorders
- - immunodeficiency
- - aged under 3 months
- - inadequate fluid intake
- - factors that might affect a carer's ability to look after a child with croup, such as adverse social circumstances
- - longer distance to healthcare in case of deterioration
607
What should be done while waiting admission for a child with croup?
- Give oxygen to all children
- ORAL DEXAMETHASONE (0.15mg/kg)
- Inhaled BUDESONIDE (2mg neb)
- IM DEXAMETHASONE (0.6mg/kg)
608
What should be given to a child with croup who does not require hospital admission?
Give a single dose of oral dexamethasone (0.15mg/kg) immediately.
609
What advice should be given to parents when safety netting for a child with croup?
- Check on child regularly, including through the night
- Seek medical advice if:
- - breathing rate increases or there are any episodes of apnoea, cyanosis or increased respiratory effort
- - signs of dehydration or fluid intake reduced to 50-75% of the norm.
- - less responsive/ difficult to rouse
- - persistent/worsening fever.
610
What supportive treatment should be given to manage a child with croup?
- Keep the child and family calm
- Symptomatic treatment and good fluid intake
- Oxygen therapy to maintain sats >93%
- 0.15mg/kg dexamethasone PO or 1mg pred once daily for three days
- Nebulised adrenaline in severe cases.
611
What is tonsillitis?
A very common infection of the tonsils, distinguishable from pharyngitis and laryngitis
612
What are the clinical features of tonsillitis?
- Sore throat which can be referred to ears
- Dysphagia
- Abdo pain
- Headache
- Voice changes
- Red throat with or without exudate
- Pyrexia
- Swollen lymph nodes (anterior cervical glands)
613
What are the differential diagnoses for tonsillitis?
- COXSACKIE INFECTION (blisters on tonsils and palate)
- GLANDULAR FEVER (affects teens, fatigue, splenomegaly)
- HSV in young adults
- EPIGLOTTITIS
- HIV
614
What are the investigations for tonsillitis?
- Clinical diagnosis with history and examination
| - CENTOR criteria
615
What is the CENTOR criteria?
HISTORY OF FEVER (1)
TONSILLAR EXUDATE (1)
TENDER ANTERIOR CERVICAL ADENOPATHY (1)
ABSENCE OF COUGH (1)
If <15 (1)
If >44 (-1)
616
What is the management for tonsillitis?
1) Reassurance that it is self-limiting
2) Avoid social contact
3) watchful waiting
4) Anti-pyretics and salt water gargles
5) Antibiotics
617
When should antibiotics be administered in treatment of tonsillitis?
- Systemic upset secondary to the acute sore throat
- Unilateral peritonsillitis
- History of rheumatic fever
- Increased risk from acute infection
- 3+ on the CENTOR
618
Which antibiotics should be given in the treatment of tonsillitis?
PHENOXYMETHYLPENICILLIN for 10 days
CLARITHROMYCIN for 10 days (in penicillin allergy)
619
When should a child be referred for a tonsillectomy?
- If they have had 5+ acute sore throats per year
- Symptoms have been occurring for at least a year
- Episodes of sore throat disrupt the child's normal behavior or day-to-day functioning
620
What are the complications associated with tonsillitis?
- Abscess
- Otitis media
- guttate psoriasis flare up
621
How long does tonsillitis usually last?
ONE WEEK
622
What is epiglottitis?
Potentially life threatening inflammation of the epiglottis which can lead to airway obstruction
623
What are the clinical features of epiglottitis?
- No prodrome
- Sore throat
- Odynophagia (painful swallowing)
- Drooling
- Hot potato voice
- Fever
- Tachycardia
- Tripod position
- Anterior neck tenderness over hyoid bone
- Ear pain
- Cervical lymphadenopathy
- Dyspnoea, dysphagia, dysphonia, respiratory distress
624
What are the investigations for epiglottitis?
DO NOT EXAMINE THE THROAT WITH TONGUE DEPRESSORS
- Refer for laryngoscopy
- Lateral XR if possible= thumb print sign
- Blood cultures and routine bloods
625
What is the management for epiglottitis?
- IV fluids
- Antibiotics (ampicillin)
- Intubation if the airway is compromised
626
What is a wheeze?
High pitched whistling sound that occurs on EXPIRATION when similar airways are narrowed by bronchospasm, swelling, secretions or an inhaled foreign body
627
What are the main causes of wheeze?
- RTIs
- Transient wheezing in infancy
- Asthma
- Bronchiolitis
- Croup
- Cigarette smoke or other air pollution
- GOR
- FB inhalation
628
What investigations should be done with a wheeze?
- Chest XR
- sweat test
- Allergy testing
- Barium swallow
- Spirometry (>6 years)
629
What is stridor?
A loud harsh high pitched sound. May start as a low pitched croaking sound, and progress to 'crowing'.
It is usually produced on inspiration but if severe can also be heard on expiration..
It occurs due to airway obstruction
630
What are the causes of stridor?
1) CROUP- the most common acute cause of stridor in children
2) INHALED FOREIGN BODY
3) TRACHEITIS
4) ABSCESSES
5) ANAPHYLAXIS
6) EPIGLOTTITIS
631
Which congenital conditions are associated with stridor?
1) Laryngomalacia
2) Vocal cord dysfunction
3) Subglottic stenosis
4) Laryngeal disorders
5) Tracheomalacia
6) Choanal atresia
7) Tracheal stenosis
632
What should be elicited in a history for a child with stridor?
- Age on onset
- Duration, progression and severity of stridor
- Precipitating factors
- Whether positional (worse right/left, prone/supine)
- Whether aphonia is present
- Other symptoms (cough, aspiration, drooling, choking, cyanosis, sleep)
- Severity (colour change, respiratory effort, apnoea)
- Perinatal history
- Developmental history
- Vaccination history
- Growth and weight gain
633
What should you observe on examination in a child with stridor?
- Fever and signs of toxicity suggesting bacterial infection
- Drooling from the mouth
- Character of cry, cough and voice
- In children, the craniofeatures, nasal patency and cutaneous haemangiomas
- positional preference that alleviates stridor
634
What should you palpate in a child with stridor?
- Crepitations or masses in the neck, face or chest
| - Deviation of the trachea
635
What should be auscultated in a child with stridor?
- Nose
- Oropharynx
- Neck
- Chest
636
What investigations should be done in a child with stridor?
- Pulse oximetry
- ABGs
- XR, CT, MRI
- Pulmonary function tests
- Laryngoscopy and bronchoscopy
637
What is the management for a child with stridor?
- ABCDE approach
- NBM
- Corticorticosteroids can be useful to reduce inflammation
- Treat underlying cause
638
What can you expect in the event of cessation of stridor with airway obstruction?
- Abrupt cessation of stridor may herald complete obstruction with chest movement but no breath sounds
- Patients will soon become unconscious
- Give oxygen
- If necessary, perform emergency endotracheal intubation, cricothyroidotomy or tracheostomy with mechanical ventilation
639
What is cystic fibrosis?
- Multi-organ disease characterized by thick mucus secretions and high salt content of sweat.
640
What causes cystic fibrosis?
- Mutation in the CFTR gene on CHR 7 and passed down via AUTOSOMAL RECESSIVE TRANSMISSION
641
What does the mutation do?
Reduces conductance of chloride ions and increases viscosity of secretions
642
What investigations should be performed in cystic fibrosis?
- SWEAT TEST: PILOCARPINE IONTOPHORESIS
- Mouthwash carrier testing
- Antenatal CVS at 10 weeks gestation
- Guthrie test (days 5-8)- if positive sweat test
- Meconium ileus
- CT head and thorax
643
What are the perinatal signs of cystic fibrosis?
- Screening
- Prolonged jaundice
- Meconium ileus
- Hemorrhagic disease
644
What are the signs of cystic fibrosis in infancy?
- Recurrent LRTI
- FTT
- Diarrhoea
- Nasal polyps
- Rectal prolapse
- Acute pancreatitis
645
What are the general clinical features of cystic fibrosis?
- Meconium ileus
- Recurrent LRTI
- FTT
- Chronic and productive coughs
- Nasal polyps
- Hyperinflation
- Malabsorption: FTT, bulky greasy stools, protuberant abdomen
- Acute pancreatitis
- Diarrhoea
- Rectal prolapse
- Diabetes
- Osteoporosis
- Congenital absence of vas deferens
- Liver disease
- Respirator signs: clubbing, cough, purulent sputum, bilateral wheeze, coarse basal crepitations
646
What is the management for cystic fibrosis?
THERE IS NO CURE
1) Tertiary CF centre
2) Chest physio
3) Saline nebs
4) Regular sputum samples
5) Prophylactic antibiotics
6) Creon and vitamin supplements ADEK
647
What is the specific respiratory management for cystic fibrosis?
- Chest physiological TWICE DAILY
- Sputum samples
- Prophylactic antibiotics (FLUCLOXACILLIN)
- Saline neb
- Bronchodilators (LABA and SABA)
- Offer rhDNase as FIRST choice of mucoactive agent
(MANNITOL dry powder if unable to tolerate rhDNase)
648
What is the management for a child with cystic fibrosis and a PSEUDOMONAS AERUGINOSA infection?
IF UNWELL
- IV antibiotics with inhaled antibiotics
- extended course of IV antibiotics
IF WELL
- commence eradication therapy- ORAL/IV antibiotics and inhaled antibiotic (NEB COLISTIMETHATE SODIUM)
649
What is the management of a child with cystic fibrosis and a NON-TUBERCULOUS MYCOBACTERIA infection?
- CT to look for changes of Non-TB mycobacteria
| - Refer to micro
650
What is the nutritional management for those with cystic fibrosis?
- Pancreatic enzyme (Creon), high fat and protein diet, vitamin supplements.
- They require 150% of normal energy diet
- Encourage increase in caloric intake
- Test for exocrine pancreatic insufficiency (stool elastase estimation)
- DIATRIZOATE MEGLUMINE and DIATRIZOATE SODIUM SOLUTION
- Ursodeoxycholic acid to breakdown gallstones.
651
How is cystic fibrosis related diabetes diagnosed?
- Continuous glucose monitoring
- Serial glucose testing over several days
- Oral glucose tolerance test
652
When should you consider a DEXA scan in a patient with CF?
- frequent or long term steroid use
- frequent IV abx use
- severe lung disease
- undernutrition
- previous low-impact fractures
- previous transplants
653
What is involved in the annual review for CF patients?
- Pulmonary assessment
- Assessment of nutrition and intestinal absorption
- Assessment for liver disease
- Testing for cystic fibrosis related diabetes from 10 years and older
- Assessment for other potential or existing cystic fibrosis complications
- Psychological assessment
- Review of exercise programme
654
What is the review frequency for those with CF?
```
WEEKLY- in the first month of life
EVERY 4 WEEKS when between 1-12 months
EVERY 6-8 WEEKS when between 1-5 years old
EVERY 8-12 WEEKS when they are >5
EVERY 3-6 MONTHS as adults
```
655
What are the complications associated with CF?
- Underweight
- Meconium ileus
- Fat soluble vitamin deficiencies (ADEK)
- Distal intestinal obstruction syndrome
- Muscle pains and arthralgia
- Male infertility
- Reduced female infertility
- Upper airway complications
- Chronic liver disease
- Urinary stress incontinence
- Cystic fibrosis related diabetes
- Reduced bone mineral density
656
What is the average life expectancy for a patient with CF?
40-50 years old
657
What is acute otitis media?
When pathogens reach the middle ear via the nasopharynx and cause inflammation?
658
What pathogens are responsible for otitis media?
- H. INFLUENZA
- STREP PNEUMONIA
- MOXARELLA CATARRHALIS
- PNEUMOCOCCAL VACCINE
RSV
RHINOVIRUS
659
What are the risk factors for acute otitis media?
- Younger, male
- Passive smoke
- Nursery
- Formula feeding
- Craniofacial abnormalities- cleft/ downs
- GORD
- Dummy use
- Supine feeding
660
What are the clinical features of acute otitis media?
- Pyrexia
- Red, yellow or cloudy tympanic membrane
- Bulging tympanic membrane
- Air fluid level
- Discharge in auditory canal secondary to TM perforation
- Red pinna
661
What investigations are done for acute otitis media?
NONE REQUIRED
BUT
can culture discharge
CT/MRI if complications
662
What is the management for for acute otitis media?
Most resolve spontaneously and without specific treatment symptoms improve within 24 hours.
663
When should you admit a patient with acute otitis media for assessment?
- <3 months of age with a temperature of 38C or more
- suspected acute complications e.g. meningitis, mastoiditis, or facial nerve paralysis
- Systemically very unwell
- 3-6 months of age with a temperature of 39C or more
664
When should antibiotic treatment for acute otitis media be given?
- Systemically unwell but do not require admission
- To those at high risk of complications
- Lasted >4 days or worsening
- < 2 years of age with bilateral acute otitis media
- Children with perforation/discharge in the ear canal
- AMOXICILLIN/ ERYTHROMYCIN/CLARITHROMICIN for FIVE DAYS
665
What are the complications associated with acute otitis media?
- Perforation
- Progression to chronic suppurative otitis media
- Labyrinthitis, meningitis, intracranial sepsis or facial nerve palsy
- Scarring of the eardrum with permanent hearing impairment
666
What is otitis media with effusion (glue ear)?
Collection of fluid in the middle war with signs only of chronic inflammation.
667
What are the risk factors of glue ear?
- Impaired Eustachian tube
- cleft palate infection
- colonization of adenoids
- male
- nursery
- URTI
- inflammation
668
What are the clinical features of glue ear?
HEARING LOSS
- lack of concentration
- impaired speech
- language and development problems at school
- ear pain
- recurrent otitis media
- opacification of the TM
- no signs of inflammation
- loss of the light reflex
- indrawn, retracted or concave TM
- absent mobility of the drum
- presence of bubbles/ fluid level
- yellow/ amber colour change to the drum
669
What investigations should be done in glue ear?
- Refer for a hearing test
| - Refer to ENT
670
What is the management for glue ear?
- Self limiting disease, but recurrence is common
- Advise parents to face child the speaking and to speak louder, slower, clearer and daily reading
- Hearing aid if surgery is contraindicated
671
When is surgical management for glue ear appropriate?
- Persistent bilateral glue ear for > 3mths
- Hearing loss >25dB
- Social, educational or developmental difficulties
- Grommets is FIRST LINE for surgery
672
What is tuberculosis?
Chronic granulomatous disease caused by MYCOBACTERIUM TUBERCULOSIS
Commonly spread by droplets
Miliary TB occurs when primary infection is not contained
673
When does secondary TB occur?
- Due to reactivation of semi-dormant bacteria and is usually preceded by immunocompromised.
Reactivation occurs in apices and can spread to distant sites
674
What are the risk factors of TB?
- TB contact
- South Asian
- Homeless
- Drug abusers
- HIV
- Elderly
675
What are the clinical features of TB?
- Fatigue
- Malaise
- Fever
- Night sweats
- Weight loss
- Anorexia
- PULMONARY (chronic, purulent ?bloodstained cough)
- GU (sterile pyuria, kidney lesions, salpingitis)
- MSK (pain, arthritis, osteomyelitis)
- CNS (TB meningitis)
- GI (ileocaecal lesions)
- Palpable lymph nodes
- SKIN (erythema nodosum)
- PERICARDIAL (pericardial effusion)
676
What investigations should be done for TB?
- CXR: Centrical apical portion with left lower lobe infiltrate/ pleural effusion, apical lesions, coin lesions
- SPUTUM SAMPLE
- MANTOUX TEST
677
What is the management for TB?
RIPE for two months
- Rifampicin
- Isoniazid
- Pyrazinamide
- Ethambutol
for four months:
Isoniazid + Rifampicin
678
What is the management for TB with Miliary spread?
RIPE for 3 months
- Rifampicin
- Isoniazid
- Pyrazinamide
- Ethambutol
for 12-18 months:
Isoniazid + Rifampicin
679
What is whooping cough?
Acute highly contagious respiratory infection caused by BORETELLA PERTUSSIS
Cyclical disease occurring every 3-4 years.
Incubation period is 1-3 weeks
680
What is the presentation of whooping cough?
1. CATARRHAL PHASE
- symptoms of mild respiratory infection
- malaise
- conjunctivitis
- nasal discharge
- sore throat
- dry cough
- mild fever
2. PAROXYSMAL STAGE
- Dry hacking cough- prolonged coughing spell followed by characteristic WHOOP
- Child chokes, gasps, and flails extremities
- post-cough vomiting
681
What investigations are done in whooping cough?
- Routine bloods
- Culture of nasopharyngeal/perinasal swabs
- Oral fluid testing- IgG anti pertussis
- PCR if very unwell
682
What is the management for whooping cough?
- Supportive treatment
| - Antibiotics: CLARITHROMYCIN
683
What are the complications associated with whooping cough?
- Pneumonia
- Apnoea
- Seizures
- Otitis media
684
What is acute abdominal pain in children?
VERY COMMON
| - important to quickly assess whether surgical intervention is required
685
What are the causes of acute abdominal pain in children?
- IBD
- ACUTE APPENDICITIS
- HSP
- UTI
- CONSTIPATION
- INTESTINAL OBSTRUCTION
- GASTROENTERITIS
- RENAL CALCULI
- PEPTIC ULCER
- LOWER LOBE PNEUMONIA
- DIABETES
- INTUSSCEPTION
- MESENTERIC ADENITIS
686
What should be elicited in a history of acute abdominal pain?
- Intermittent unexplained screaming
- Blood in the stool?
- Location of pain
- Associated features such as vomiting, diarrhea, recent viral infection, joint or urinary symptoms
- Loss of appetite
- Vomiting bile
687
What should be assessed in examination for acute abdominal pain?
- Assessment of how ill the child looks
- HR, CRT, temp
- Check face for guarding/pain when palpating the abdomen
- reluctance to move, rebound tenderness, guarding, rigidity
- Palpable lymphadenopathy (mesenteric adenitis)
688
What investigations should be done in acute abdominal pain?
- FBC (leucocytosis found in appendicitis and UTI)
- Urine dip (nitrites +++ in UTI, hematuria in HSP)
- Urine MCS (pyuria and organisms suggest UTI)
- Abdominal radiograph (dilated bowel loops, intestinal obstruction, abnormal gas pattern, intussusception, faecal loading)
- Abdominal USS (renal tract abnormality)
- Barium and air enema (intussusception)
- CRP/ESR (may be elevated in IBD or infection)
689
What are some causes for recurrent abdominal pain?
- IDIOPATHIC RECURRENT ABDOMINAL PAIN
- HEPATITIS
- GI (ibs, esophagitis, PU, IBD, constipation, malabsorption, giardiasis)
- UTI
- PANCREATITIS
- GYNAE (dysmenorrhoea, PID, haematocolpos, ovarian cyst)
OTHER- Psychogenic, abdominal migraine, sickle cell
690
What need to be elicited in a history of recurrent abdominal pain?
- Where is it worst?
- What time of day does it occur
- Does pain affect ADLs
- Associated constitutional symptoms (weight loss, anorexia)
- GI, GU, GYNAE symptoms?
- Emotional, anxiety or family problems?
691
What should be assessed in the physical examination for recurrent abdominal pain?
- Growth : weight loss/ fall- off in growth
- General examination: look for pallor, jaundice and clubbing
- Abdominal examination: hepatomegaly, splenomegaly, enlarged kidneys, distended bladder
- Anorectal examination
692
What investigations are done in recurrent abdominal pain?
- FBC (anaemia, eosinophilia, infection (leucocytes)
- CRP (H.pylori)
- LFTs (liver dysfunction)
- U+Es (renal failure)
- Amylase (pancreatitis)
- Urinalysis and culture (UTI)
- Stool for ova and parasites (GI parasites e.g. giardiasis)
693
What are the imaging investigations done in recurrent abdominal pain?
- Abdominal and pelvic ultrasound (urinary obstruction at all levels, organomegaly, abscesses, pregnancy, ovarian cyst, torsion)
- Plain abdominal radiography (constipation, renal calculi if radio-opaque, lead poisoning)
- Barium swallow and follow through (esophagitis and reflux, peptic ulcer, Crohn's disease, congenital malformations)
- Barium enema (ulcerative colitis)
- ENDOSCOPY
694
What is diarrhea?
Excessive stool and fluid loss from the rectum
| Loss of more than 5% of this water is significant dehydration.
695
What are the causes of acute diarrhoea?
- Viral gastroenteritis
- Bacterial gastroenteritis
- - shigella
- - E. coli
- - salmonella
- - campylobacter
- Antibiotic induced
- Food poisoning
- Any acute infection
696
What should be elicited in history and examination of a patient with acute diarrhoea?
- Any associated vomiting?
- Is the vomiting projectile?
- How many loose stools have there been?
- Passing urine as normal/less than normal?
- History of cystic fibrosis/diabetes?
697
What should be done in examination of a patient with acute diarrhoea?
- Weigh the child and compare with previous weights to assess dehydration
- In young infants feel for a pyloric mass during a test feed
- Assess the degree of dehydration
698
What factors should be assessed when checking for dehydration status?
- Mouth and lips
- urine output
- mental state
- pulse rate
- blood pressure
- capillary refill time
- fontanelle
- skin and eye turgor
699
What investigations should be done when assessing diarrhoea?
- U+Es for electrolyte imbalance and renal function
- Blood gas for metabolic acidosis/alkalosis
- Urinalysis for osmolality or specific gravity
- Blood sugar to exclude DKA
- Stool culture in gastroenteritis and food poisoning
700
What is the treatment for diarrhoea?
- Use oral rehydration therapy where possible
- Treat shock with bolus of IV fluids
- Rehydrate slowly to replace fluid loss over at least 24h
- Correct any electrolyte imbalance
701
What are the main features of chronic diarrhoea in children?
- Frequent stools are normal in early childhood
- Babies have 1-7 loose stools per day
- This usually stops after 12 months
- If the child is thriving and there are no other signs/symptoms, intervention is rarely necessary
- Chronic diarrhoea can be split into pathological and non- pathological
702
What are the main causes of pathological diarrhoea?
1. MALABSORPTION
2. INFLAMMATION
3. INFECTION
703
What are the non-pathological causes of chronic diarrhoea?
TODDLER DIARRHOEA
- Thriving toddler
- Loose stools containing undigested food
- May have large fluid intake
- Fast gut transit time
NON-SPECIFIC DIARRHOEA
- Loose watery stools
- Thriving child, may follow on from acute gastroenteritis
704
What are the malabsorptive causes of pathological chronic diarrhoea?
CYSTIC FIBROSIS
- starts in infancy
- FTT with chest infections
- Fatty stools
COELIACS DISEASE
- FTT with irritability
- muscle wasting, abdominal distension
- fatty stools
- Diagnosis by jejunal biopsy
SECONDARY LACTOSE INTOLERANCE
- baby or toddler
- follows acute gastroenteritis
- watery stools with low pH and reducing substances
705
What are the infective causes of pathological chronic diarrhoea?
PARASITES: Giardia Lamblia
- Weight loss and abdominal pain
- Watery stools
- Common in nurseries
706
What are the inflammatory cause of pathological chronic diarrhoea?
CROHN'S DISEASE
- Late childhood and adolescence
- weight loss and abdominal pain
- anorexia and fatigue
- exacerbations and remissions
COWS MILK PROTEIN INTOLERANCE
- Occurs in babies
- watery stools, may be bloody
- may have urticaria, stridor, bronchospasm or eczema
ULCERATIVE COLITIS
- late childhood and adolescence
- bloody stools and abdominal pain
- exacerbations and remissions
707
What should be elicited in a history of chronic diarrhoea in children?
1) Bowel pattern- volume, appearance, and consistency of stool. Blood/mucus present?
2) Precipitating factors- certain foods troublesome? others in the family/nursery affected?
3) Associated symptoms- weight loss/ abdo pain
4) Review of symptoms- non GI diseases?
708
What should be checked for during physical examination of a child with chronic diarrhoea?
- GROWTH- height, weight, head circumference.
| - ASSOCIATED FEATURES- hydration, pallor, abdominal distension, tenderness and finger clubbing
709
What investigations are done in a child presenting with chronic diarrhoea?
- PLASMA VISCOSITY/ESR- High in IBD
- COELIAC ANTIBODIES- test for coeliac disease
- URINE CULTURE
- SWEAT TEST (CF)
- BREATH HYDROGEN TEST- High H2 in carb malabsorption
STOOL SAMPLE
- ova and parasites- parasitic infection
- reducing substances and low pH- present in sugar intolerance
- faecal elastase- low in pancreatic insufficiency
- microscopy for fat globules- seen in fat malabsorption
- faecal calprotectin- present in IBD
IMAGING
- Jejunal biopsy- subtotal villous atrophy with crypt hyperplasia in coeliac disease
- Barium follow through- characteristic signs in small bowel Crohn's disease
- Endoscopy- Characteristic lesions in IBD
710
What is possetting?
Regurgitating a small amount of milk, is normal in babies
711
What is the definition of vomiting?
Complete emptying of the stomach
712
What are the causes of vomiting in children?
1) GASTROENTERITIS
2) FOOD ALLERGY
3) INFECTION (UTIs, middle ear infections, pneumonia, meningitis)
4) APPENDICITIS
5) POISON
713
What are the causes of vomiting in babies?
1) Gastroenteritis
2) Food allergy/ milk intolerance
3) GORD
4) Overfeeding
5) poison
6) Congenital pyloric stenosis (projectile)
7) strangulated hernia
8) Intussusception
9) Whooping cough
10) Constipation
714
What are the causes of vomiting in older children/adolescents?
1) Gastroenteritis
2) Migraine
3) Raised intracranial pressure (effortless vomiting, usually neuro signs, papilloedema)
4) Bullimia
5) Toxic ingestion/medication
6) Pregnancy
715
What are the red flags associated with vomiting in children?
- Repeatedly vomiting and unable to hold down fluids
- Dehydrated- dry mouth, crying without producing tears, urinating less/not wetting nappies
- Vomit is green/ contains blood
- More than a day or two
716
What should be elicited in a history of vomiting in a child?
- Differentiate between possetting and serious vomiting
- Thorough feeding history
- Ask about projectile vomiting (pyloric stenosis) and bile stained vomiting
- presence of diarrhoea
- fever suggests infection
- paroxysms of coughing (whooping cough)
- GORD especially in infants with cerebral palsy/Downs
717
What should be checked in examination of a vomiting child?
- Check for dehydration, especially with gastroenteritis
- Feel for a palpable pyloric mass in any young infant
- Check for abdominal distension which suggests intestinal obstruction
- Check for papilloedema and hypertension in cases of unexplained vomiting to exclude raised ICP
- Look for signs of meningitis
718
Which investigations should be done in a vomiting child?
1) U+Es- to assess electrolyte imbalance in dehydration and in pyloric stenosis
2) Chloride, pH and bicarb- to assess metabolic alkalosis in pyloric stenosis
3) pH monitoring and barium swallow- may show significant GOR
4) Upper GI contrast study- mandatory in bile-stained vomiting to exclude malrotation
719
What does bile stained vomiting in infants indicate?
CONGENITAL INTESTINAL OBSTRUCTION
| Duodenal or ileal atresia or volvulus of a malrotated intestine.
720
What investigations should be done in bile stained vomiting?
UPPER GI CONTRAST STUDY
- Contrast studies usually use a barium
- Contrast meal scans and contrast swallow scans are used to study the oesophagus and stomach in detail
721
What are some problems associated with feeding?
- Child may be easily distracted by TV/ run around during meal time (turn off TV, aim to all eat together)
- Child will change their mind about what they are willing to eat (Make only one meal for the whole family, allow child to help with prep)
- Child very slow eater/picks at food- (if weight satisfactory, do not worry that they are starved)
- Too much fluid (give drinks and snacks after/ not before food)
722
What investigations should be done in children with feeding problems?
- Weigh and measure accurately
| - Base all interventions on a food diary
723
What is the appropriate management for a child with feeding problems?
- Reassure parents
- Keep child relaxed and do not pressurize them into eating
- Small helpings that the child can finish
- Eating together as a family
724
What is the background for constipation and soiling?
- very common (30%)
- 95% is functional and is developmentally normal
- can be associated with major life events and neurodevelopmental disorders
725
According NICE guidance, when should constipation be diagnosed?
In a child presenting with two or more of the following:
STOOL PATTERNS
- <3 complete stools per week
- rabbit dropping stools
overflow soiling in children older than 1
Associated symptoms in children of any age
- distress/ pain on passing stool
- bleeding associated with hard stool
- straining
Associated symptoms in children >1
- Poor appetite that improves with passage of large stool
- Waxing and waning of abdominal pain with passage of stool
- Evidence of 'retentive posturing'- straight-legged, arched back
- Anal pain
- Past history of constipation
- Previous/current anal fissure
726
What are the features of idiopathic constipation?
- History of meconium being passed within 48 hours of birth (in a full term baby)
- Constipation begins at least a few weeks after birth
- Precipitating factors may be present such as weaning, poor fluid intake
- Abdomen is soft and not distended, normal appearance of the anus
- General health, growth and development are normal with normal gait, tone and power in lower limbs
727
What are the red flags in association with constipation?
- Symptoms that commence from birth or within the first few weeks
- Failure or delay in passing meconium
- Ribbon stools
- Leg weakness or locomotor delay
- Abdominal distension with vomiting
ABNORMAL EXAMINATION:
- Abnormal appearance of the anus
- Gross abdominal distension
- Abnormal gluteal muscles, scoliosis, sacral agenesis
- Limb deformities including talipes
- Abnormal reflexes
728
What are amber flags in association with constipation?
- Constipation with faltering growth
| - Possible maltreatment
729
What is important to elicit in history of constipation?
- Stool frequency, consistency
- Faecal incontinence
- Pain: likely to lead to withholding
- Stools block the toilet
- Associated behaviours
- Leg weakness and locomotor delay
730
What should be elicited in examination for constipation?
LOOK FOR
- sacral abnormalities
PALPATE
- faecal mass
- anal stenosis
- ectopia
Rectal examination and radiography are not routinely necessary
731
What are some of the organic causes of constipation?
1) ANORECTAL MALFORMATIONS
2) ANAL FISSURE- common and associated with painful defaecation. Passage of blood and sentinel pile on the anterior anus are characteristic
3) RECTAL PROLAPSE
4) HIRSCHSPRUNG'S DISEASE
5) NEURENTERIC PROBLEMS - Colonic motility (test of choice)
6) SPINAL CORD PROBLEMS
7) PELVIC FLOOR DYSSYNERGIA
8) METABOLIC/SYSTEMIC DISORDERS (Hypothyroidism, Coeliac disease, hypocalcaemia, cystic fibrosis)
9) TOXIC: lead levels, toxicology screen
10) COWS MILK ALLERGY
11) COELIAC DISEASE
732
What is the management for constipation in children?
- Disimpaction
- Maintenance
- Behaviour modification
- Reassurance
REFER TO SECONDARY CARE if:
- red flag symptoms
- treatment unsuccessful
- safeguarding/ child abuse concerns
733
What is the process for disimpaction in constipation management?
- Osmotc laxative such as MOVICOL
- If ineffective, add a stimulant such as SENNA or if < 6mths, DOCUSATE.
- BISACODYL SUPPOSITORIES can be used if >2
- Review weekly
734
What is the regime for MOVICOL in disimpaction?
Treat until impaction resolves, or for a maximum of 7 days.
1-5 years: 2-4-4-6-6-8-8 (sachets per day in succession)
5-12 years: 4-6-8-10-12-12-12 (sachets per day)
735
What is the maintenance advice given following disimpaction in constipation?
- Dietary advice= increase fibre and fluids
- Use bowel charts/diary
- Regular daily movicol
- Avoid stopping and starting treatment
- Prolonged laxative use- hypokalemia and atonic colon
736
What is the advice given for behaviour modification in consitpation management?
- Encourage regular unhurried toileting
| - Reward systems
737
What reassurance and supportive treatment can be given for incontinence?
- Explain involuntary nature of this
- Encourage regular toileting
- Involve school nurse and teachers to help
738
What are the potential complications of constipation?
- Faecal impaction
- Chronic constipation
- Megacolon (may predispose to, or result from constipation)
- Rectal prolapse
- Anal fissure
- Faecal soiling
- Psychological effects
739
What is the background for gastroenteritis?
Infective gastroenteritis is characterized by sudden diarrhoea and vomiting. It is mostly VIRAL and resolves within days, but care must be taken with dehydration.
740
What are the main causes of gastroenteritis?
VIRAL
- Rotavirus
- Norovirus
BACTERIAL
- campylobacter
- salmonella
- shigella
- E. coli
741
What are the risk factors associated with gastroenteritis?
- Poor hygiene
- Immunocompromised
- Poorly cooked food
742
What are the clinical features of gastroenteritis?
- Sudden onset D+V
- Fever
- previous antibiotics: C. Diff
- Blood in stool: campylobacter and e.coli
- Tender abdomen, distension
743
What are the red flags symptoms associated with gastroenteritis?
- Altered responsiveness
- sunken eyes
- tachycardia
- tachypnoea
- reduced skin turgor
744
What are the signs of shock that can result from gastroenteritis?
- Decreased consciousness
- Mottled skin
- Cold extremities and weak pulses
- Tachycardia and tachypnoea
- Prolonged CRT
- Hypotension
745
Who are the children at risk of developing dehydration?
- Children younger than 1 year of age, particularly younger than 6 months
- Infants who were of low birthweight
- Children who have passed >5 diarrheal stools in the previous 24 hrs
- Children who have vomited >2 in the previous 24hrs
- Children who have not been offered, or not been able to tolerate supplementary fluids before presentation
- Infants who have stopped breastfeeding during their illness
- Children with signs of malnutrition
746
How is gastroenteritis diagnosed?
Clinical symptoms and signs.
| Examination and culture of a stool sample may be necessary to determine the cause.
747
What is important to elicit when taking a history/examination of gastroenteritis?
- Document the frequency and consistency of stools, frequency of vomiting and presence of blood in the stool
- Ask about possible sources of infection (such as recent contact with someone with acute D+V, recent travel abroad, exposure to possible enteric infection (eating out))
748
When would you consider an alternative diagnosis if someone presents with symptoms similar to gastroenteritis?
- Fever (temp of >38C/39C whether > 3 months)
- Shortness of breath/ tachypnoea
- Altered conscious state
- Neck stiffness
- Bulging fontanelle in infants
- Non-blanching rash
- Blood and/mucus in stool
- Bilious green vomit
- Severe/localised abdominal pain
- Abdominal distension/rebound tenderness
749
What investigations should be done in gastroenteritis?
- FBC
- U+Es
- CRP
- Blood cultures
- Stool sample
750
Why would you send for an MCS stool sample in gastroenteritis?
- Septicaemia
- Blood/mucus
- Immunocompromised
- Recent travel abroad (test for ova, cysts and parasites)
- Recent hospitalisation/antibiotics
- Not improved by day 7
- Uncertain diagnosis
- Outbreaks in local community
751
What are the key points to note during stool sample collection?
- Do not allow urine to mix with the stool sample
- Pass stool onto potty/plastic wrap/ newspaper put over the toilet
- Small scoopfuls collected from area of mucus/blood
- Screw lid on tightly, add child details
- Deliver sample as soon as possible
752
What is the supportive management of gastroenteritis?
Manage at home but provide safety netting for parents
- Continue fluid intake, but NOT fizzy drinks/juices
- Wash hands often, do not share towels
- Avoid school for 48 hrs after last D+V
- Clean and launder all areas/items affected by faecal matter
- if CRYPTOSPORIDIOSIS is suspected, do not enter swimming pools for 2 weeks after the last episode of diarrhea.
753
What are the time frames through which symptoms of gastroenteritis should cease?
- Diarrhoea: the usual duration is 5-7 days, and in most children it stops within 2 weeks
- Vomiting: the usual duration is 1 or 2 days and in most children in stops within 3 days.
SEEK HELP IF SYMPTOMS PERSIST WELL OVER THESE TIMES
754
How should gastroenteritis be managed if there is accompanying dehydration?
- Use ORS solution (50ml/kg)
- Maintenance fluids
- Give ORS frequently and in small amounts
755
How is ORS used to rehydrate?
If UNDER 5
- Give ORS 50ml/kg for fluid deficit replacement over 4 hrs
- Give ORS maintenance volume (breastfeeding can continue)
- Small amounts frequently to reduce sickness
If OVER 5
- Give 200ml ORS solution (1 sachet) after each loose stool (in addition to child's normal fluid intake)
756
How are maintenance fluids prescribed?
100+50+20 RULE
100ml/kg for the first 10 kg
(1000ml +) 50ml/kg for the second 10 kg
(1500ml+) 20ml/kg for every kg over 20kg
757
What should be done if shock is suspected in gastroenteritis?
- IV 0.9% saline with 5% dextrose as maintenance
- If confirmed shock then add 100ml/kg to maintenance to reverse deficit. If not shocked add only 50ml/kg
- Measure U+Es
758
What drugs should be prescribed to children with gastroenteritis?
- DO NOT PRESCRIBE ANTIBIOTICS*
- DO NOT PRESCRIBE ANTI-DIARRHOEALS
- DO NOT PRESCRIBE ANTI-EMETICS
759
When should antibiotics be considered in gastroenteritis?
- Septicaemia
- Extra intestinal spread of bacterial infection
- Younger than 6 months or immunocompromised with salmonella
- C. diff, pseudomembranous enterocolitis, giardiasis, amoebiasis, cholera
760
What are the complications associated with gastroenteritis?
- Dehydration- shock- death
- Hemolytic uraemic syndrome in E. coli
- Lactose intolerance after viral infection
761
When should the local authorities be notified in gastroenteritis?
If any of the following are suspected:
- CHOLERA
- BLOODY DIARRHOEA
- FOOD POISONING (campylobacter, e. coli, salmonella, shigella, giardia, yersinia enterolytica, entamoeba histolytica, norovirus)
- Hemolytic uremic syndrome
762
What is the background of gastro-esophageal reflux?
Non forceful regurgitation of gastric contents which is asymptomatic
Does not require treatment
Should be distinguished from vomiting
GOR resolves in 90% before 1 year
It occurs due to incompetent gastro-oesophageal sphincter
763
What is gastro-esophageal reflux disease?
Disease is when reflux is persistent and symptomatic
764
What are the risk factors for GOR?
- Premature birth
- Parental history of heartburn or acid regurgitation
- Obesity
- Hiatus hernia
- History of congenital diaphragmatic hernia (repaired)
- History of congenital esophageal atresia (repaired)
- Neurodisability
765
What are the clinical features of GOR?
- HEARTBURN
- RETROSTERNAL PAIN
- EPIGASTRIC PAIN
- Recurrent regurgitation or vomiting
- Irritable
- Arching due to discomfort
- Witnessed episode of choking
- Respiratory problems (cough, apnoea, recurrent wheeze and aspiration pneumonia)
- Stops feedings (does not want pain)
- FTT
766
What investigations are done in GOR?
Diagnosis is predominantly clinical
- FBC (anaemia due to bleeding)
- 24hr esophageal pH study of complications occur
- barium meal if bile stained
- endoscopy- where oesophagitis is suspected
- manometry- to assess esophageal motility and lower esophageal sphincter function
767
When would you refer in cases of GOR?
- Haematemesis
- Melaena
- Dysphagia
- No improvement in regurgitation after 1 year
- Persistent, faltering growth associated with overt regurgitation
- Retreosternal, epigastric or upper abdominal pain that needs ongoing medical therapy
- Unexplained iron-deficiency anaemia
- A suspected diagnosis of Sandifer's syndrome
768
What is the conservative management given for GOR?
LIFESTYLE ADVICE
- Reassure it is very common (40%) begins at 8 weeks may be frequent and will resolve by 1 year
- Advise that sitting baby up during feeds and shortly after can help
- Do NOT overfeed baby (150ml/kg)
- Thickened formula milk
- Frequent but smaller feeds
- Trial of Gaviscon after feeds for 1-2 weeks
769
What signs for safety netting should be given in GOR?
- projectile
- bile stained
- blood stained
- FTT
- feeding problems
- lasts >1 year
770
What medical management can be used in GOR?
- 4 week trial of PPI or H2RA for children with communication difficulties, feeding difficulties, distressed FTT, persistent pain
- After 4 weeks, then can refer for endoscopy if not resolved
LAST RESORT
- metoclopramide, domperidone, erythromycin
771
What are the complications associated with GOR?
- Reflux oesophagitis
- Recurrent aspiration pneumonia
- Frequent otitis media (for example more than 3 episodes in 6 months)
- Dental erosion in a child/young person with a neurodisability, particularly cerebral palsy
772
What is the cause of GORD?
- Gastro-oesophageal sphincter immature, weakened
- Wider than usual opening in the diaphragm around the oesophagus
- Cerebral palsy or Down syndrome are more likely to have GORD
773
What is the presentation of GORD?
- Frequent spitting up or regurgitation after feeds
- Abdominal pain or general crankiness in the hours after feeding
- No weight gain as expected and may have frequent chest infections
- Inflammed surface of the oesophagus, leading to scarring and narrowing
774
When to suspect GORD in an infant under 1 with regurgitation?
If they have ONE or more of the following:
- Distressed behaviour show (excessive crying, crying while feeding, adopting unusual neck postures)
- Hoarseness and/or chronic cough
- A single episode of pneumonia
- Unexplained feeding difficulties
- Faltering growth
- Episodic torticollis with neck extension and rotation
- >1 year= heartburn, retrosternal pain, epigastric pain
775
What are the differentials that can be associated with GORD?
- PYLORIC STENOSIS- Frequent, forceful vomiting
- INTESTINAL OBSTRUCTION- bile stained, abdominal distension
- GI BLEEDS- hamatemesis
- RAISED ICP- bulging fontanelle, altered responsiveness, rapidly increasing head circumference
- GASTROENTERITIS
- COWS MILK PROTEIN ALLERGY
- CHRONIC DIARRHOEA
- UTI
- INFECTION
776
What investigations are done in GORD?
1) pH impedance study (probe inserted nasally to measure reflux)
2) Upper GI contrast study (barium) x ray photos taken
3) Gastroscopy
4) Manometry- catheter inserted nasally and sensor measures nerve and muscle activity
777
What is the conservative management for GORD?
- Feeding smaller amounts more frequently
- Head of the cot can also be raised by placing the legs on wooden blocks
- Switch formula to types less likely to cause reflux (Enfamil AR, SMA stay down)
- Add thickeners to instant formula (Instant Carobel)
778
What is the medical management for GORD?
1ST LINE- 2 weeks of Gaviscon
2ND LINE- 4 weeks of ranitidine or omeprazole
Erythromycin speeds up the rate at which feed passes from the stomach into the duodenum
779
What is appendicitis?
Sudden inflammation of the appendix, usually beginning with obstruction of the lumen. Leads to invasion of the wall by gut flora leading to infection.
This can then rupture and lead to life threatening peritonitis. Can cause abscess if surrounded by omentum causing the infection to become more localized.
Most common between 10 and 20
More common in males
780
What are the clinical features of appendicitis?
- Periumbilical pain which localized to RIF when peritonitis occurs. Movement and coughing aggravate it.
- Patient will lie still with shallow breathing
- Nausea, vomiting, anorexia
- Watery diarrhoea
- Low grade pyrexia and rising pulse
- ROSVINGS TEST- palpation of the LIF causes pain in RIF
- Rebound tenderness
- PSOAS TEST, OBTURATOR TEST
- Stage of illusion (just after perforation, child may look better)
- Can present as vague atypical pain
781
What are the scoring systems for appendicitis?
1) ALVARADO
- migratory RIF pain
- N+V
- anorexia
- tender RIF
- rebound tendernes
- pyrexia
- leukocytosis
- neutropenia
Give appendectomy if >7
2) RIPASA score
782
What investigations are done in appendicitis?
ABDOMINAL EXAM
- Urinalysis to exclude UTI
- FBC: milk leucocytes
- CRP
- US where diagnosis is doubtful
783
What is the management for appendicitis?
- Admit to hospital and APPENDECTOMY
- IV fluids and analgesia
- Preoperative antibiotics
784
What are the complications associated with appendicitis?
- Perforation
- Wound infection
- Appendix mass: omentum and SO adhere to appendix
- Abscess: US, drainage and then appendectomy
- Paralytic ileus and septicaemia
785
What is Coeliac disease
Immune mediated, inflammatory disorder porvoked by gluten, which can lead to malabsorption of nutrients.
It is a multi genetic disorder, associated with HLA and other genetic and environmental factors
786
Which HLA types are associated with coeliacs disease?
HLA DQ2
| HLA DQ8
787
What are the clinical features/presentations of coeliacs disease?
- Persisten GI symptoms (D+V, distension, irritability, steatorrhoea)
- FTT
- prolonged fatigue
- unexpected weight loss
- severe/persistent mouth ulcers
- unexplained iron, B12, folate deficiency
- type 1 diagnosis
- autoimmune thyroid disease
788
Which groups of people are at high risk of having coeliacs disease?
- Metabolic bone disorder
- Unexplained neurological symptoms
- Persistently raised liver enzymes with unknown cause
- Down's syndrome
- Turner's syndrome
789
What are some associated symptoms of coeliacs disease?
- Palor
- Gross motor delay
- ascites
- peripheral oedema
- anaemia
- arthralgia
790
What investigations are done for those with Coeliacs disease?
GLUTEN CONTAINING DIET MUST BE EATEN DURING DIAGNOSTIC PROCESS
- IgA tTG and total IgA first line
- FBC
- B12, FOLATE, FERRITIN, LFTs, CALCIUM, ALBUMIN
- BIOPSY (subtotal villous atrophy) from the jejunum
791
What is the management for coeliacs disease?
- Lifelong strike gluten-free diet
- provide information on the disease
- coeliac support groups
792
What should be done in the annual review of children with coeliacs disease?
- Measure weight and height
- Review symptoms
- Assess diet and need to specialist dietetic and nutritional advice
- Assess need for DEXA scan
- Routine bloods
793
What are the complications associated with coeliacs disease?
- Osteoporosis
- Growth failure
- Delayed puberty
- dental problems
- nutrient deficiencies
- malignancy
- ulcerative jejunitis
- functional hyposplenism
- infertility
- reduced bone mineral density
- inadequate energy intake
- inadequate calcium, B6 and D
794
What is dermatitis herpetiformis?
A chronic polymorphic pruritic skin disease (skin manifestation of coeliacs disease)
795
What is an inguinal hernia?
Protrusion of abdominal contents through fascia of abdominal wall through the internal inguinal ring.
Always contains peritoneal sac but may also contain viscera.
Males are more likely
Can be congenital or develop due to rupture of abdominal wall
796
What are the clinical features of an inguinal hernia?
- Swelling in the groin that may appear with lifting and be associated with sudden pain
- Indirect hernias are more prone to pain in scrotum (dragging sensation)
- Impulse palpable on coughing
- Reducible?
797
What are the three classifications of inguinal hernias?
1) Indirect
2) Direct
3) Sliding
798
What is the definition of an indirect inguinal hernia (80%)?
A protrusion through the INTERNAL INGUINAL RING possess along the inguinal canal through the abdominal wall running LATERALLY TO THE INFERIOR EPIGASTIC VESSELS
Associated with failure of the inguinal canal to close properly after passage of the testis in utero or during the neonatal period
799
What is the definition of a direct inguinal hernia?
Hernia protrudes directly through a weakness in the POSTERIOR WALL of the INGUINAL canal running MEDIALLY TO THE INFERIOR EPIGASTRIC VESSELS
800
What is the definition of a sliding inguinal hernia?
Portion of viscera slide BEHIND THE PERITONEAL SAC into the INGUINAL CANAL with the wall of the organ forming part of the hernial sac.
801
What investigations are done for inguinal hernias?
Clinical diagnosis
| US if the diagnosis is unclear
802
What is the management for inguinal hernias?
- Herniotomy with ligation and excision of the patent processus vaginalis.
- Surgical laparoscopic repair soon after diagnosis in healthy full term infant boys with asymptomatic reducible hernias
- Inguinal hernias in premature infants are usually repaired prior to discharge from the neonatal intensive care unit, but can be postponed until 2 months if necessary
803
What are the complications associated with inguinal hernias?
- Recurrence
- Infarcted testis or ovary with atrophy
- Wound infection
- A hydrocele from fluid accumulation in the distal sac usually resolves spontaneously but sometimes requires aspiration
804
What is intussusception?
- When one segment of the bowel invaginates into another leading to obstruction.
- Bowel may simply telescope onto itself or there may be pathology.
- The mesentery becomes compressed, the bowel wall distends and the lumen is obstructed.
- Peristalsis is disrupted and eventually ischaemia occurs
More common in males 5-10 MONTHS
Most common cause of obstruction in under 3 year olds.
805
What is the most common site for intussusception?
In most children it is ILEOCAECAL
806
What are the clinical features of intussusception?
- Paroxysms (about every 10-20 minutes) of colicky abdominal pain and crying
- Early vomiting, rapidly becoming bile-stained
- Neuro symptoms (lethargy, hypotonia, sudden alterations of consciousness)
- Palpable sausage shaped mass in the RUQ
- Absence of bowel in the right lower quadrant (Dance's sign)
- Dehydration, pallor, shock
- Irritability, sweating
- Mucoid, bloody, RED CURRANT stools
- Late pyrexia
807
What are the non-pathological lead points for intussusception?
- Viral in 50%: rotavirus, adenovirus and HHV6
- Ameobomata, shigella, yersinia
- Peyer's patch hypertrophy
808
What are the pathological lead points for intussusception?
- Meckel's diverticulum
- Polyps and Peutz- Jeghers syndrome
- Henloch-Schonlein purpura
- Lymphoma and other tumours
- Reduplication
- Cystic fibrosis
- An inflammed appendix
- Ascariasis
- Nephrotic syndrome
- Postoperative
- Exclusive breast feeding
- Weight above average
- Rotavirus vaccine
- Abdominal tuberculosis
809
What are the investigations done for intussusception?
- FBC- neutrophilia
- U+Es- dehydration
- Abdo XR
- US- doughnut/target sign, pseudokidney
- Barium enema
- CT/MRI
810
What is the management for intussusception?
- Refer urgently to secondary care
- Resuscitation- NG tube and IV fluids, (drip and suck)
RADIOLOGICAL
- Reduction if no signs of shock or peritonitis
- Air enema <120mmHg or barium enema
SURGICAL
- Laparotomy if peritonitis, perforation, prolonged, pathological lead point, failed enema
811
What are the complications associated with intussusception?
- Ischaemia
- Necrosis, haemorrhage, perforation
- Infection and peritonitis
- Chronic intussuscpetion- rare cause of FTT
812
What is jaundice?
Common sign of liver disease, but can also indicate haemolysis.
Its is the accumulation of bilirubin in the skin produced from the breakdown of haemoglobin.
Bilirubin is conjugated in the liver so it can be excreted
Jaundice occurs when serum bilirubin is >25-30mmol/L
813
What is the definition of unconjugated hyperbilirubinaemia?
Increased total serum bilirubin level with <15% in the conjugated form
814
What is the definition of conjugated hyperbilirubinaemia?
Increased total serum bilirubin level with >20% in the conjugated form
815
What are the causes of unconjugated hyperbilirubinaemia?
HAEMOLYTIC DISORDERS (excessive haemolysis)
- RBC membrane disorders
- Enzyme defects
- Haemoglobinopathies
INHERITED DISORDERS
- Gilbert's syndrome
- Criglar-Najjar
816
What are the causes of conjugated hyperbilirubinaemia?
INFECTIONS
- Hep A-E
- Epstein Barr Virus
- Cytomegalovirus
- Herpes Simplex virus
- Gram negative bacterial infections and sepsis
METABOLIC LIVER DISEASE
- Wilson's disease
- Alpha-1 Antitrypsin Deficiency
- Cystic Fibrosis
BILIARY TRACT DISORDERS
- Cholelithiasis
- Cholecystitis
- Choledocal cyst
- Sclerosing Cholangitis
AUTOIMMUNE LIVER DISEASE
HEPATOTOXINS
- Paracetamol
- Alcohol
- Anticonvulsants
- Anaesthetics
- Chemotherapeutic agents
- Antibiotics
- Oral contraceptives
VASCULAR CAUSES
- Budd Chiari syndrome
- Veno-occlusive disease
817
What should be elicited in a history for jaundice in children?
- Jaundice (duration, onset)
- Malaise
- Pruritis
- Anaemia symptoms
- Dark urine (conjugated)
- Steatorrhoea (cirrhosis)
818
What should be assessed during examination of a child with jaundice?
- Growth: FTT
- Excoriations due to pruritis
- Liver disease stigmata (spider naevi, clubbing, caput medusa, ascited, dupuytrens contracture, liver flap)
- Kaiser fleischer rings in Wilsons
- Hepatosplenomegaly
- Conscious level (encephalopathy)
819
What are the investigations for jaundice in children?
- FBC, blood film, reticulocyte count
- Coagulation studies
- U+Es, SBR, LFT, albumin, total protein, TFT
- Viral serology (Hep a-e, ebc, cmv), blood culture
- Copper levels
- Abdominal US, Abdominal CT, biliary scintigraphy
- Liver biopsy
820
What is the management for jaundice in children?
- Mainly supportive
- Correct any clotting abnormality
- Phototherapy may be helpful only if jaundice has a significant unconjugated component,
- Treat any associated anaemia if due to hemolysis
- Correct fat soluble vitamin deficiencies (A,D,E,K) and nutritional supplements
821
What is mesenteric adenitis?
It is the result of an intercurrent viral infection.
| It is caused by an inflammation of intra-abdominal lymph nodes following an URTI or gastroenteritis
822
How does mesenteric adenitis present?
- No peritonism or guarding, and there may be evidence of infection in the throat or chest
- Diagnosis of exclusion
- Fever
- Malaise
- Central abdominal pain
823
What is the management of mesenteric adenitis?
- Period of monitoring to make sure the pain is not progressing in the same way as appendicitis.
- ANALGESIA
824
What is pyloric stenosis?
Stenosis of the pylorus
- Caused by hypertrophy and hyperplasia of the smooth muscle of the stomach and pylorus.
- It is most common in FIRST BORN MALES
- Most commonly between 2-8 weeks
825
Early exposure to which drug increases the risk of pyloric stenosis?
ERYTHROMYCIN
826
What is the presentation of pyloric stenosis?
VOMITING
- 2-8 WEEKS
- non-bilious, projectile, and usually 30-60 minutes after a feed with the baby remaining hungry
- Vomiting increases in frequency over several days
- Slight haematemesis
- Persistent hunger
- weight loss
- dehydration
- lethargy
- infrequent or absent bowel movements
- Stomach wall peristalsis
827
What can be seen on examination in a child with pyloric stenosis?
- "OLIVE' shaped mass in the RUQ or epigastrium
- best palpated at the start of a feed
- With the infant supine gently palpate the liver edge near the diploid process
- Then displace the liver superiorly; downward palpation should reveal the pyloric olive right of the midline
828
What investigations should be done for pyloric stenosis?
- US= should show a thickened and elongated pyloric muscle
| - Blood gas: will show HYPOCHLORAEMIC, HYPOKALAEMIC METABOLIC ALKALOSIS
829
How is pyloric stenosis managed?
- Rehydration with careful correction of the electrolyte imbalance before definitive surgery
- Rehydration may take at least 24 hours
SURGICAL
- splitting the pylorus muscle without cutting through the mucosa (Ramstedt's pyloromyotomy)
- can be laparoscopic
- oral feeds can be commenced soon after surgery
830
What are the complications associated with pyloric stenosis?
- Dehydration
- Weight loss
- Hypokalaemia and hypochloraemic alkalosis
831
What are examples of testicular problems?
- INGUINAL HERNIA
- TESTICULAR TORSION
- INGUINAL LYMPHADENOPATHY
- HYDROCELE
832
What are some examples of impalpable testes?
- RETRACTILE TESTIS (brought down by careful palpation, examine the child warm hands and cross legged or squatting
- UNDESCENDED TESTES
- ECTOPIC TESTES (testes descend but along abnormal path, may be in superficial inguinal pouch or on perineum)
833
What is torsion of the testis?
PAEDIATRIC EMERGENCY
| Causes occlusion of testicular blood supply and viability of the testis is compromised.
834
What are risk factors for testicular torsion?
- Genetic factors may predispose
- A high insertion of the tunica vaginalis, producing 'bell-clapper testis' with a horizontal resting position high up in scrotum
835
What are the main features of presentation for testicular torsion?
- SUDDEN ONSET
- SEVERE SCROTAL PAIN
- SWELLING
- Associated with nausea and vomiting
- TENDER TESTIS with overlying skin reddened/ oedematous
836
What is the presentation for congenital testicular torsion?
- Rare perinatal event
- Newborn infant has a hard painless scrotal mass
- Tested have invariably infarcted- does not require further investigation
837
What is the presentation for torsion outside the perinatal period?
- Result of abnormally mobile mesentery of the testis inside the tunica vaginalis
- This anomaly is bilateral and allows gonad to twist on its vascular pedicle
838
What investigations are done in testicular torsion?
ULTRASOUND INTEGRATED WITH COLOUR DOPPLER
A significant finding is detection of intratesticular blood flow for early identification
- Urinalysis to exclude UTI
839
What are the differential diagnoses associated with testicular torsion?
- Torted hyatid
- Epididymo-orchitis
- Hydrocele
- Testicular trauma
- Malignancy
- Mumps
- Idiopathic scrotal oedema
840
What is the appropriate management for testicular torsion?
- Immediate scrotal exploration to salvage the testis, which should then be fixed to prevent recurrence- ORCHIDOPLEXY
- The contralateral testis should also be fixed
- May be possible to reduce manually, but also do ORCHIDOPLEXY
841
What is the definition of undescended testes?
- Failure of the testes descended from the inguinal canal into the scrotum during the first trimester (CRYPTOCHORDISM)
- The aeitology is multifactorial (genetic, maternal and environmental factors)
842
What are the main possible causes of absence of the testes in the scrotum?
- Testicular agenesis
- Retractile testes
- Ascending testis syndrome
- Testicular maldescent (unilateral, more common in preterm, can descend within the 1st three months.
843
Absence of testes in the scrotum can be broadly divided into two subgroups:
- palpable undescended testes (80%)
| - impalpable testes (20%)
844
What are the features of palpable undescended testes?
- Usually at external inguinal ring
| - These testes can be brought down into the scrotum with an orchidoplexy performed through inguinal incision
845
What are the features of impalpable testes?
- Intra-abdominal (either inside the inguinal canal or absent)
- Risk of malignant degeneration in intra-abdominal testis
- If in abdomen then it must be brought down in a two stage orchidoplexy.
846
Which genetic syndromes are associated with cryptochordism?
1) PRADER WILLI SYNDROME
2) KALLMAN'S SYNDROME
3) CONGENITAL ADRENAL HYPERPLASIA
847
What should be done in examination of a child with undescended testes?
- Child should be supine in cross-legged position
- Visual examination of the scrotum
- Inhibit the cremasteric reflex with one hand above the symphysis in the groin region before the scrotum
- 'Milking' of the groin region towards the scrotum may help to move the testis
- A retractile testis can usually be moved into the scrotum and will remain there until it retracts back into the groin again with a cremasteric reflex
- Look at the femoral, penile and perineal region for ectopic testes
848
What investigations should be done in undescended/impalpable testes?
- LAPAROSCOPY is the investigation of choice
| - Abdominal and pelvic ultrasonography may be required if intersexuality is suspected
849
What is the management for undescended testes?
This should be documented in medical records
- Initial follow up with GP because the majority will descend within the first 6 months of life
- GP REVIEW: 6-8 weeks
- GP REVIEW: 3 months
- If undescended before 6 months- refer to surgeon
ORCHIDOPLEXY is required by 12 months to avoid infertility, torsion and malignancy
850
What are the complications associated with undescended testes?
- Increased risk of torsion
- Malignancy
- Trauma
- Reduced fertility
851
What is biliary atresia?
Condition of uncertain cause where part, or all, of the extrahepatic bile ducts are obliterated by inflammation and subsequent fibrosis, leading to biliary obstruction and jaundice
852
How does biliary atresia present?
- Persistent jaundice with rising conjugation fraction
- Pale, chalky stools
- Dark urine
- Splenomegaly (late sign due to portal HTN)
- FTT
853
What is Kasai classification?
TYPES OF OBSTRUCTION
854
What is Type 1 biliary atresia?
- CBD is obliterated, proximal bile ducts are patent
855
What is Type 2 biliary atresia?
Atresia of the HEPATIC duct
TYPE 2A:
- cystic and CBD are patent
TYPE 2B
- cystic, CBD and hepatic ducts are all obliterated
856
What is Type 3 biliary atresia?
- Atresia refers to discontinuity of both R+L hepatic ducts to the level of the porta hepatis
857
What investigations are done in biliary atresia?
Any baby jaundiced after 2 wks needs to have conjugated and unconjugated bilirubin measured
- LFTs and GGT (will be high)
- US
- Biopsy
- ERCP (if diagnosis is unclear)
858
What is the management for biliary atresia?
- KASAI PROCEDURE (if detected within 6 weeks), replaces bile duct with intestine
- Antibiotics to prevent cholangitis
859
What are the viral causes of hepatitis?
Hep A-E and G
860
Features of Hepatitis A
- Incubation 2-6 weeks
| - FAECAL-ORAL transmission
861
Features of Hepatitis B
```
- Incubation 6 weeks to 6 months. Endemic in Far East and Africa
Transmission from:
- BLOOD PRODUCTS
- SEXUAL INTERCOURSE
- CLOSE DIRECT CONTACT
- VERTICAL TRANSMISSION
```
862
Features of Hepatitis C
```
- Incubation 2 weeks to 6 months
Transmission from:
- BLOOD PRODUCTS
- SEXUAL INTERCOURSE
- CLOSE DIRECT CONTACT
- VERTICAL TRANSMISSION
```
Usually causes mild severity acute illness or is asymptomatic
Rarely causes acute hepatitis
863
Features of Hepatitis D
Requires previous HBV infection
864
Features of Hepatitis E
FAECAL ORAL TRANSMISSION (endemic in India)
865
Features of Hepatitis G
PARENTERAL TRANSMISSION
866
What other organisms can cause hepatitis as part of their systemic manifestations?
- Epstein Barr Virus
| - TORCH (toxoplasmosis, other (syphilis), rubella, cytomegalovirus, HSV)
867
What are some non-infective causes of hepatitis?
- POISONS AND DRUGS- paracetamol, isoniazid, halothane
- METABOLIC DISEASE- Wilson's disease
- AUTOIMMUNE HEPATITIS- May present with acute hepatitis
- REYE'S SYNDROME- a rare acute encephalopathic illness associated with aspirin therapy
868
What are the symptoms of hepatitis?
- Fever
- Fatigue
- Malaise
- Anorexia
- Nausea
- Arthralgia
- RUQ pain
- Jaundice + hepatomegaly
- Splenomegaly
- Adenopathy
- Urticaria
869
What investigations should be done in hepatitis?
- LFTs
- Decreased blood glucose (especially in Reye's syndrome)
- Viral serology
870
What is the management for hepatitis?
- Supportive measures
- Alcohol avoidance in teenagers
- No antivirals unless child is immunocompromised
- Fulminant hepatitis requires referral
871
What is the prevention of viral hepatitis?
- Proper hygiene
- Vaccinations for Hep A and B
- Blood transfusions should be screened to reduce risk of infection
872
What is Hirschsprungs Disease?
Absence of parasympathetic ganglion cells (myenteric plexus) in the bowel wall leading to functional bowel obstruction as cells can't relax
It is more common in males
Can be associated with Down's syndrome
873
Which genes have been listed to be associated with Hirschsprungs Disease?
X
13
10
19
874
What is the presentation of Hirschsprungs Disease?
- Usually presents in the new born period with delayed passage of meconium for >48 hours after birth, and abdominal distension
- If only a short segment of bowel is affected, it may present later with chronic constipation and FTT
- overflow incontinence
875
What investigations should be done in Hirschsrungs disease?
- FBC : infection could lead to necrotizing enterocolitis
- ABDO XR: dilated loops of bowel with airless rectum
- Diagnosis made with barium enema and then rectal biopsy
876
What are the management options for acute problems with Hirschsprungs disease?
PRESENCE OF INTESTINAL OBSTRUCTION
- IV fluids
- gastric and intestinal decompression
- cessation of oral feeding
PRESENCE OF ENTEROCOLITIS
- Requires broad spectrum antibiotics and aggressive intravenous rehydration
877
What are the surgical options for management of Hirschsrungs disease?
SWENSON'S PROCEDURE- remove defective distal colon and perform an end-end anastomosis.
878
What are the complications associated with Hirshsprungs disease?
Enterocolitis
879
What is inflammatory Bowel disease?
A cause of chronic diarrhoea in late childhood and adolescence.
880
What are the two types of IBD?
- CROHNS
| - ULCERATIVE COLITIS
881
Features of Crohn's disease?
- Twice as common as UC
- May affect ANY PART of the GI tract but TERMINAL ILEUM, PROXIMAL COLON are the commonest sites of involvement
- Skip lesions (bowel involvement is not continuous)
882
What does Crohn's disease present with?
- Recurrent abdominal pain
- Anorexia
- Growth failure
- Fever
- Diarrhoea
- Anaemia
- Oral/ perineal ulcers
- Arthritis, eye and skin complications
883
How is Crohn's disease diagnosed?
- ENDOSCOPIC BIOPSY
884
What is the treatment for Crohn's disease?
REMISSION CAN BE INDUCED BY
- Mesalazine (aminosalicylates)
- Steroids
- Azathioprine
- Immunosuppressive therapies; infliximab
- Surgical resection
885
Features of ulcerative colitis?
- Involves the colon only
| - Rectal (proctors) is the most common, or may extend continuously up to whole colon (pan colitis)
886
How does ulcerative colitis present?
- Bloody diarrhoea
- abdominal pain
- weight loss, fever, arthritis
- eye and skin complications
887
How is ulcerative colitis diagnosed?
- Barium studies
| - Biopsy
888
What is the treatment for ulcerative colitis?
- Mesalazine (aminosalicylates)
- Steroid enema
- Azathioprine
- Immunosuppressive therapies, infliximab or even colectomy
889
When are anti-diarrhoeals contra-indicated in IBD?
- During active colitis, for fear of TOXIC MEGACOLON
| - They CAN be used in stable disease
890
What is Toddler's diarrhoea?
- 3-4 bowel actions per day for > 3 weeks
- Frequent loos stools no pathology
- Presents 6 months to 5 years
- More common in boys
891
What is the presentation of Toddler's Diarrhoea?
- Chronic non-specific diarrhoea
- Colicky intestinal pain
- Increased flatus
- Abdominal distension
- Loose stools with undigested food (peas and carrot stools)
All examinations and investigations are normal- child is otherwise well and thriving
892
What is the treatment for Toddler's Diarrhoea?
REASSURE- will settle in 2-5 days
| Diet advice with 4 F's (fat, fluid, fibre, fruit juice)
893
What are the four F's of diet advice in Toddler's diarrhoea?
1) HIGH FAT
- low fat foods cause diarrhoea
- fat slows speed of digestion
- milk, yogurts, cheese
2) LOW FLUID
- avoid excessive fluid intake
- 6-8 cups (200ml) water per day is recommended
3) HIGH FIBRE
- low fibre diet- does not soak up water- loose stools
- wholemeal bread, cereal, brown rice/pasta
- high fibre diet= causes irritation
- white bread, reduce fruit and veg intake (child portion is what fits in their hand
4) LOW FRUIT JUICE
- High levels of fructose- poorly absorbed- faster stomach emptying and transit
- no added sugar often have artificial sweeteners
- dilute juice (1:10 - juice:water)
- NO clear juices (apple, grape, blueberry)
894
What is a volvulus?
Torsion of a malrotated intestine and present with severe abdominal pain and bilious vomiting.
It is a complete twisting of a loop of intestine around its mesenteric attachment site.
895
What is midgut rotation?
EMBRYOLOGICALLY
- jejunum, ileum, cecum, appendix, ascending colon and 2/3 transverse colon develop, as the mid gut herniates through the umbilical ring during umbilical herniation
- The loop then ROTATES 270 degrees counterclockwise around the superior mesenteric artery (SMA) and returns to the abdominal cavity
896
What is malrotation?
When the midgut does not complete rotation prior to returning to the abdomen
897
How does volvulus present?
- Rapid onset bilious vomiting
- metabolic acidosis
- lactataemia
- oliguria
- hypotension
- shock with advancing ischemia
- palpable abdominal mass, distension, signs of peritonitis
- blood/ sloughed tissue passed per rectum
- Tachycardia, hypovolemia, septic shock
898
What investigations should be done in suspected volvulus?
- ROUTINE BLOODS: hyponaetraemia, hyperkalaemia, metabolic acidosis, increased urea and creatinine, hypochloraemia, lactic acidosis
- ABDOMINAL X RAY: duodenal obstruction- DOUBLE BUBBLE
- UPPER GI CONTRAST: dilation of the proximal duodenum with 'bird-beak' obstruction and a spiral or corkscrew duodenal configuration
- CT SCAN: 'coffee bean' in sigmoid volvulus
- COLOUR DOPPLER: 'whirlpool sign'
899
What is the management for malrotation?
- Observation and GI decompression with NG tube
| - Ladd's procedure
900
What is the management for volvulus?
- Ladd's procedure is the treatment of choice
901
What are the complications associated with volvulus?
- Short gut syndrome (post-operative)
- Intestinal ischaemia
- Mucosal necrosis
- Sepsis
- Perforation
- Peritonitis
- Death
902
What is the etiology for insulin dependent diabetes mellitus type 1?
- It occurs in 1 in 500 (0-14yrs)
- Destruction of beta islet cells in pancreas leads to insulin deficiency
- Onset determined by genetic predisposition plus some trigger factor (possibly viral infection related)
- incidence is increasing, particularly in children
903
What is the early presentation of type 1 diabetes?
CLASSIC TRIAD
1. Excessive drinking (polydipsia)
2. Polyuria
3. Weight loss
OTHERS
- fatigue
- recurrent infections
- blurred visions
- necrobiosis lipidica
- enuresis
- Candida and other infections
904
What is the late presentation of type 1 diabetes?
DKA
- smell of acetone on breath (pear drops)
- vomiting
- dehydration
- abdominal pain
- hyperventilation due to acidosis (kussmaul breathing)
- hypovolaemic shock
- drowsiness
- coma and death
905
What symptoms can be manifest in those already diagnosed and on insulin? (HYPOGLYCAEMIA)
- sweating
- tremor
- palpitations
- irritability
906
What investigations are carried out in type 1 diabetes?
Diagnosis is heavily based on the presence of the main symptoms:
- polyuria
- polydipsia
- weight loss
- excessive tiredness
AND
- random venous plasma glucose >11.1mmol
- fasting glucose >7
- OGTT >11.1mmol
907
What are the main principles of Type 1 Diabetes management?
- Referral to pediatrician if just presenting with classic triad
- Parental and patient education
- Referral to CNS, dietician and social worker
- Dietary advice
- Blood glucose measurements
- Insulin regimens
908
What is important to mention in education for parents and patients with type 1 diabetes?
1) Nature of diabetes
2) Recognition of hypoglycemia (BM measuring)
3) Insulin regime and technique
4) Diet and maintenance of active lifestyle
5) Inform school
6) Coping with crises with either short acting insulin or glucose gel/snack/glucagon and to have these on their person at all times.
7) Support from Diabetes association
909
What is important to consider in diet in management of type 1 diabetes?
- Eating a normal diet with high fibre
- Take meals at regular times and regular snacks
- High sugar foods kept to a minimum
- Carb counting- one CP (carb portion) is usually = 10g of carb
- Administer insulin dependent on your insulin:carb ratio
- They should not fast (for religious purposes, be vigilant with check levels, and break fast if levels below 3.5mmol/L)
910
What should be considered in for blood glucose measurements in management of type 1 diabetes?
Blood glucose measurements should be performed at least 5 TIMES DAILY
- increase frequency when sick, exercising, high risk activities
911
What are the optimum targets for short term blood glucose control- when self monitoring?
1) FASTING: 4-7mmol/L on waking
2) BEFORE MEALS: 4-7mmol/L
3) AFTER MEALS: 5-9mmol/L
For those of driving age
WHEN DRIVING: >5mmol/L
HbA1c: 48mmol/L or lower to minimize risk of long-term complications of type 1 diabetes.
912
What are the main options for insulin administration?
1) TWICE DAILY REGIMEN
2) BASAL BOLUS REGIMEN
3) CONTINUOUS SUBCUTANEOUS INSULIN INFUSION
913
What is involved in the twice daily regimen for T1DM management?
- Insulin is given as a mixture of rapid- acting insulin (peak at 2-4 hours) and intermediate-acting isoprene insulin (peak at 4-12 hours)
- Administered subcutaneously in the arms, thighs, buttocks or abdomen
- BEFORE BREAKFAST and BEFORE EVENING MEAL
Adjust doses according to the general trend in pre-meal, bedtime and occasional night time blood glucose levels
914
What is involved in the basal bolus regimen for T1DM management?
- Provides a long-acting insulin at night and...
- Rapid acting insulin given before each meal (reduces blood glucose levels after meals and optimizes blood glucose control)
- Based on calculated carbohydrate intake
915
What is involved in the continuous subcutaneous insulin infusion (CSII) of rapid acting insulin for TD1M management?
- Programmable pump and insulin storage reservoir that gives a regular/continuous amount of insulin (rapid/short acting) by a subcutaneous needle/cannula
- Can deliver differential basal rates at different times of the day/night with higher infusion rates triggered by the push of a button
- Insulin requirement can be matched more closely to the person's basal requirement, preprandial glucose, carb intake and physical activity.
916
When should insulin pump therapy be recommended?
NICE recommends:
- 12 years or older
- multiple daily injection is ineffective, or has resulted in hypoglycemia
- children younger than 12 where multiple daily injection is inappropriate/impractical
917
What are the three forms of insulin available in the UK?
- HUMAN INSULIN (produced by recombinant DNA)
- HUMAN INSULIN ANALOGUE (insulin modified to produce a specific desired kinetic characteristic)
- ANIMAL INSULIN- extracted and purified from animal sources (rarely used)
918
How are insulins characterized?
1) SHORT ACTING INSULIN- rapid onset of action and short duration of action
2) SOLUBLE INSULINS- (injected 30 mins before food , onset 30-60 mins, duration up to 8hrs) e.g. HUMAN ACTRAPID/HUMALIN S.
3) RAPID ACTING INSULIN ANALOGUES- onset within 15mins, duration (2-5 hrs) e.g. HUMALOG, NOVORAPID
4) INTERMEDIATE ACTING- used to mimic the effect of the basal insulin secreted continuously throughout the day. Onset 1-2 hrs, maximal effects between 4-12hrs, duration 16-35 hrs e.g. HUMALIN I, INSUMAN BASAL, HUMAN INSULATARD
5) LONG ACTING INSULIN ANALOGUES- Mimic basal insulin but has longer duration. Used once or twice a day and achieve a steady state level after 2-4 days to produce a constant level of insulin e.g. LANTUS, LEVEMIR, TRESIBA
919
How should insulin be stored?
In the refrigerator 2-8C
920
What are the main injection site problems that can occur with insulin administration?
- Signs of infection
- Swelling
- Bruising
- Lipohypertrophy- injecting the same small area. can be minimized by rotating injection sites
921
Why is insulin given subcutaneously?
- it is destroyed by gastric acid and is not absorbed across the gut mucosa.
922
What factors increase insulin absorption?
- FAT MASS (slower with large amounts of subcutaneous fat)
- AGE (faster in young children)
- INJECTION SITE
- EXERCISE (increased blood flow at injection site of exercised region
- ACCIDENTAL INTRAMUSCULAR INJECTION
- LARGER DOSE (slower absorption)
923
Where are the main sites for insulin injection?
1) Abdomen
2) Outer thigh
3) Buttocks
4) Arm (not usually recommended)
924
Which drugs enhance the hypoglycemic effects of insulin and therefore REDUCE insulin requirement?
- ALCOHOL (can mask hypos)
- ANABOLIC STEROIDS
- ACE INHIBITORS
- BETA BLOCKERS
- FIBRATES
- LANREOTIDE AND OCTREOTIDE
- MAOI (selegiline, rasagiline)
- SALICYLATES
- SULPHONAMIDES
925
Which drugs antagonize the hypoglycemic effects if insulins and therefore INCREASE insulin requirement?
- CORTICOSTEROIDS
- DANAZOL
- DIURETICS
- GLUCAGON
- GROWTH HORMONE
- LEVOTHYROXINE
- ORAL CONTRACEPTIVES
- SYMPATHOMIMETIC DRUGS (salbutamol, adrenaline, terbutaline)
926
What is DKA (diabetic ketoacidosis)?
The metabolic state characterized by the triad of:
- MARKED HYPERGLYCAEMIA
- ACIDOSIS
- KETONAEMIA
It is a medical emergency because it leads to dehydration and electrolyte imbalances
927
When should you suspect DKA in a person?
If they have KNOWN DIABETES or SIGNIFICANT HYPERGLYCAEMIA (finger prick glucose level >11mmol/L)
If they have certain precipitating factors
If they have ketones present in blood/urine
928
What are precipitating factors that could be associated with DKA?
- infection (pneumonia/UTI)
- physiological stress (trauma/surgery)
- inadequate insulin or non-adherence to insulin treatment
- other medical conditions (pancreatitis/hypothyroidism)
- Drugs (corticosteroids, diuretics, sympathomimetic drugs e.g. salbutamol)
929
What constitutes a high ketone level in the urine?
> 2+
930
What constitutes a high ketone level in the blood?
> 3mmol/L
931
What are the symptoms of DKA?
- Increased thirst and urinary frequency
- Weight loss
- Inability to tolerate fluids
- Persistent vomiting and/or diarrhoea
- Abdominal pain
- Lethargy and/or confusion
932
What are the clinical signs of DKA?
- Fruity smell of acetone on the breath (pear drops)
- Acidotic breathing (deep sighing, Kussmaul respiration)
- Dehydration (mild, moderate [dry skin and mucus membranes, reduced skin turgor], severe [sunken eyes, prolonged CRT])
- Shock- tachycardia, poor peripheral perfusion, hypotension
- Lethargy, drowsiness, decreased level of consciousness
- Reduced urine output
933
What is the appropriate management for DKA?
- FLUID RESUSCITATION with IV normal saline
- INSULIN SLIDING SCALE: short acting soluble insulin via syringe driver- (0.25 units/kg/hr)
- HOURLY U+Es and K+ REPLACEMENT on passing urine
- SWITCH TO IV DEXTROSE when glucose levels fall to 12mmol/L
934
What is the process for reviewing children with diabetes?
CHILDREN SHOULD ATTEND CLINIC 4 TIMES A YEAR as regular contact helps to optimize blood glucose control
935
What should be ascertained in a diabetes review history?
- review diary
- ask about blood glucose levels
- symptoms of hypo/hyperglycaemia
- dietary difficulties
- problems at school related/unrelated to diabetes
- if in poor control, assess compliance with injections, diet and any new stresses
936
What should be done in a diabetes review physical examination?
- height and weight
- injections sites for lipoatrophy/hypertrophy
- fundi and blood pressure
937
What investigations should be done in a diabetes review?
- HbA1c
- TFTs (if thyromegaly, or fall off in growth
- Opthalmological examination
938
What is the management for hypoglycemia?
- GLUCOSE 10-20g PO then snack repeat if needed
- IM GLUCAGON 500mcg (if unconscious)
- IV DEXTROSE INFUSION 10% (2-5ml/kg) with careful monitoring
939
What is the definition of Failure To Thrive (FTT)?
Significant interruption in the expected rate of growth compared with other children of similar age and sex during early childhood.
940
When should an evaluation be carried out concerning FTT?
When:
- Weight or height is BELOW THE 2ND CENTILE
- Crossing down 2 CENTILE CHANNELS for height and weight
- If infants lose more that 10% of their birth weight, or do not return to birth weight by THREE WEEKS
941
What are the main causes of FTT?
1) PSYCHOSOCIAL
- difficulties at home, disturbed attachment, postnatal depression, neglect, eating difficulties.
2) REFLUX
- vomiting and posseting. esophagitis, anorexia and pain
3) MALABSORPTION
- diarrhoea and colic, COELIAC and CF most common
4) CHRONIC ILLNESS
5) CONSTITUTION
- small parents, growth will be steady along lower centiles
6) IUGR
- length, weight and HC will be affected. reduced growth potential but can show catch up
7) GENETIC
- Dysmorphic features e.g. short stature e.g. Turner's
8) ENDOCRINE
- congenital hypothyroidism
942
What should be elicited in the presenting complaint portion of a FTT history?
FEEDING/EATING PATTERNS
- what is eaten, how much and how often (food diary)
- issues with breastfeeding?
- how formula is prepared
- excess liquid leading to satiety before meals
- environmental/ family eating patterns
- child seem content with feed? dissatisfied? craving more? uninterested?
BOWEL and URINE HABITS
- nature of stool
- chronic diarrhoea
- frequency of wet nappies
943
What associated symptoms should be check in FTT history?
- fever
- pain
- coughing
- SoB
- dysphagia
- vomiting
- diarrhoea
944
What should be asked about pregnancy/ birth in FTT history?
- Smoking, alcohol drugs, illness
- placental insufficiency
- prematurity
- IUGR
- birth weight
945
What should be asked about PMH in FTT history?
- Congenital abnormalities (cerebral palsy, Down's)
- Developmental delay
- GI, respiratory, immunological, metabolic, endocrine
- allergies, food intolerances
946
What psychosocial factors should be asked during FTT history?
- Social circumstances (who is at home, access to food)
- Family stress (divorce, housing, financial problems)
- Substance and domestic abuse
- health beliefs
- maternal depression/anxiety
947
What family factors should be asked in FTT history?
- familial disorders (coeliacs, IBD, endocrine disorders)
- parental mental health
- constitutional growth delay in parents
- biological parent's height (to calculate mid-parental height centile)
948
What should be checked during examination for a child with suspected FTT?
INSPECTION
- does the child look lively, active and well?
- features suggestive of syndrome (Down's, Turner's)
- well nourished/ starved
- alert/ responsiveness
- normal tone?
PLOT HEIGHT, WEIGHT AND HEAD CIRCUMFERENCE
FULL SYSTEMIC EXAMINATION
949
Other than decrease in growth, what physical signs may be seen on examination in FTT?
- oedema
- hepatomegaly
- rash/ skin changes
- hair color and texture abnormalities
- signs go vitamin deficiency
- dehydration
950
What investigations should be carried out in FTT?
- FBC/ FERRITIN- iron deficiency is common in FTT and can cause anorexia
- U+Es- unsuspected renal failure
- STOOL FOR FECAL ELASTASE- low levels suggest malabsorption
- COELIAC ANTIBODIES, JEJUNAL BIOPSY, SWEAT TEST- coeliac disease, CF are main causes of malabsorption
- THYROID HORMONE and TSH- Congenital hypothyroidism causes poor growth
- KARYOTYPE- chromosomal abnormalities are often associated with short stature and dysmorphism
951
What are the management options for FTT?
Most children can be managed with ADVICE and support.
It is important to treat the underlying cause
DIETARY
- 3 meals and two snacks each day
- increase the number and variety of foods offered
- increase energy density of foods by adding cheese, margarine/cream
- limit milk intake to 500ml per day
- avoid excessive juice drinking
BEHAVIOURAL
- offer meals at regular times with other family members
- praise when food is eaten, ignore when not
- limit meal time to 30mins
- mealtime conflict should be avoided
- NEVER force feed
952
When should referrals be made for FTT?
PAEDIATRICIAN
- when there are features suggesting an associated illness, or where severe FTT not responsive
SOCIAL WORK
- where family has major social problems, e.g. substance abuse, or where direct evidence suggests abuse/neglect
PSYCHOLOGY
- indications include pronounced food refusal, or very anxious, stressful mealtimes
953
What are disorders to sex development (DSDs)?
A group of conditions where the reproductive organs/genitals do not develop normally
954
What are the steps involved in normal puberty?
1) BRAIN BEGINS THE PROCESS- brain makes GnRH
2) PITUITARY GLAND RELEASES MORE HORMONES- GnRH causes the pituitary gland to release luteinizing hormone (LH) and follicle stimulating hormone (FSH)
3) SEX HORMONES ARE PRODUCED. LH and FSH cause the ovaries to produce hormones involved in the growth and development of female sexual characteristics (estrogen) and the testicles to produce hormones responsible for the growth and development of male sexual characteristics (testosterone).
4) PHYSICAL CHANGES OCCUR- the production of estrogen and testosterone causes the physical changes of puberty
955
What is delayed puberty?
GIRLS
- lack of breast development by 13
- more than 5 years between breast growth and menstrual period
- lack of pubic hair by age 14
- failure to menstruate by 15-16
BOYS
- lack of testicular enlargement by age 14
- lack of pubic hair by age 15
- more than 5 years to complete genital enlargement
956
What are the causes of delayed puberty?
CENTRAL- INTACT HYPOTHALAMO-PITUITARY AXIS
- Constitutional delay in growth and puberty (CDGP)
- chronic illness- CKD, IBD
- malnutrition
- excessive physical exercise
- psychosocial deprivation
- steroid therapy
- hypothyroidism
IMPAIRED HYPOTHALAMO- PITUITARY AXIS
- Pituitary tumours
- congenital anomalies- sept-optic dysplasia, congenital panhypopituitarism
- irradiation treatment
- trauma
- IHH- Kallmanns (loss of smell)
PERIPHERAL
- bilateral testicular damage
- Noonan's
- Prader-Willi
- Kleinfelter (boys)
- Turner's (girls)
- Complete androgen insensitivity syndrome, congenital adrenal hyperplasia
- PCOS
957
What investigations should be done in delayed puberty?
- routine bloods, ferritin
- coeliac and CKD screen
- karyotyping
- FSH, LH, serum testosterone/estradiol
- prolactin
- TFTs
- GnRH test and growth hormone
- Pelvic USS in girls
- bone age (wrist XR)
- MRI of pituitary
958
What is the management for delayed puberty?
CDGP
- reassurance
- puberty can be induced by short course hormones (testosterone by depot or PO 3/12, oestrogen with cyclical progression therapy until levels have been achieved)
PRIMARY GONADAL FAILURE
- pubertal induction followed by ongoing hormone replacement
- early gonadotrophins in neonatal period/infancy (for severe congenital hypogonadism)
CENTRAL DELAY
- treat underlying cause
959
What is precocious puberty?
Secondary sexual characteristics occur before the age of 8 in girls and 9 in boys
Motor common in women
960
What are the causes of precocious puberty?
CENTRAL/TRUE
- idiopathic
- tumours
- CNS trauma
- hypothalamic hamartoma
- hydrocephalus
- arachnoid cyst
PRECOCIOUS PSEUDOPUBERTY
- CAH
- tumours
- McCune Albright
- Silver Russell
- Hypothyroidism
- Exogenous hormone exposure
961
What is the presentation of precocious puberty in girls?
NORMAL TANNER STAGES- but occurring earlier leading to psychosocial problems and reduced height
- breast enlargement
- pubic and axillary hair
- menarche does not occur until 2-3 years after onset of breast enlargement
- growth spurt
962
What is the presentation of precocious puberty in boys?
NORMAL TANNER STAGES- but occurring earlier leading to psychosocial problems and reduced height
- testicular enlargement
- growth of the penis and scrotum- typically occurs a year after testicular enlargement
- growth spurt
963
What investigations should be carried our in precocious puberty?
- hormone levels
- LF and FSH
- TFTs
- Adrenal steroid precursors
- HCG
- Pelvic US
- Wrist XR for bone age
- Brain MRI
- GnRH stimulation test
- Karyotyping
964
What is the management for precocious puberty?
- surgical resection of tumors
| - GnRH agonists which reduce release of LH and FSH
965
What is Congenital Adrenal Hyperplasia (CAH) and ambiguous genitalia?
A group of autosomal recessive disorders which results in a block of corticosteroids, leads to a build up of androgenic precursors and ambiguous genitalia
966
What is CAH and ambiguous genitalia in males?
MALE APPEARANCE
- severe hypospadias with bifid scrotum
- undescended testes with hypospadias
- bilateral non-palpable testes in a full term apparently male infant
967
What is CAH and ambiguous genitalia in females?
FEMALE APPEARANCE
- clitoral hypertrophy of any degree
- non-palpable gonads
- vulva with single opening
968
What are the causes of CAH and ambiguous genitalia?
- 21- hydroxylase deficiencies CORTISOL DEFICIENCY
- 11-BETA-HYDROXYLASE DEFICIENCY
- CAH is the most common cause of ambiguous genitalia
969
What is the female presentation of CAH?
- Ambiguous genitalia with an enlarged clitoris
- common urogenital sinus in place of a separate urethra and vagina
- internal female organs are normal
PRADER scale (1-5) has been developed to describe this
970
What is the male presentation of CAH?
- No signs at birth
- subtle hyperpigmentation
- possible penile enlargement
those with 'salt losing form' of CAH present at 7-14 days with:
- vomiting
- weight loss
- lethargy
- dehydration
- hyponatraemia
- hyperkalaemia
- shock
boys with non salt-losing form present with
- EARLY VIRILISATION at 2-4yrs
971
How do patients with non-classic CAH present?
- early pubarche
- young women with infertility
- hirsutism
- oligomenorrhoea
- amenorrhoea with PCO and acne
972
What investigations are done in CAH?
- U+Es- will show hyponatremia, hyperkalaemia, hypoglycemia
- Serum 17-hydroxyprogesterone: HIGH
- corticotropin stimulation test
- pelvic US
- bone age
- karyotype
973
What is the management for CAH?
- Glucocorticoid replacement therapy
- Mineralocorticoid therapy for salt wasting form of CAH
- urogenital surgery (clitoral reduction and vaginoplasty)
974
What is congenital hypothyroidism?
A lack of thyroid hormones present from birth. If not detected and treated early, it is associated with CRETINISM- irreversible neurological problems and poor growth.
975
What are the causes of congenital hypothyroidism?
- THYROID GLAND DEFECTS (75% of all cases)
- DISORDERS OF THYROID HORMONE METABOLISM
- HYPOTHALAMIC-PITUITARY DYSFUNCTION
- TRANSIENT HYPOTHYROIDISM
- MATERNAL MEDICATIONS (carbimazole)
976
What are the clinical signs associated with congenital hypothyroidism?
- large fontanelle
- myxoedema (swelling) with coarse features and a large head
- nasal obstruction
- macroglossia (large tongue)
- low temperature with cold and mottled skin on the extremities
- jaundice
- umbilical hernia
- hypotonia
- hoarse voice
- cardiomegaly
- bradycardia
- pericardial effusion
- goitre
977
What features in the growing child will be apparent with congenital hypothyroidism?
- short stature
- hypertolerism (increased distance between eyes)
- depressed bridge of nose
- narrow palpebral fissures
- swollen eyelids
- refractory anaemia
978
What symptoms are associated with congenital hypothyroidism?
- feeding difficulties
- somnolence (excessive desire to/periods of sleep)
- lethargy
- low frequency of crying
- constipation
979
What investigations should be done in suspected congenital hypothyroidism?
- Guthrie heel prick screening test at birth (T3+T4). The blood is also analyzed for PKU, CF and sickle cell
- A HIGH TSH and LOW T4 confirm the diagnosis
- Thyroid auto-antibodies are also measured
980
What is the management for congenital hypothyroidism?
- Early detection and early thyroid hormone replacement to ensure that infants do not develop irreversible neurological disability
- L-thyroxine is given once daily and titrated to TFTs
- TFTs need to be monitored on a regular basis
981
What is an example of acquired hypothyroidism?
LYMPHOCYTIC THYROIDITIS (HASHIMOTO'S AUTOIMMUNE THYROIDITIS)- the most common cause go hypothyroidism.
982
What are some rarer causes of acquired hypothyroidism?
- acute suppurative thyroiditis
| - iatrogenic and subacute non-suppurative thyroiditis (de Quervain's disease)
983
What is the presentation for acquired hypothyroidism?
- short stature and delay in bone age
- fall-off in school performance and low energy
- constipation
- dry skin
- delayed puberty
- cold intolerance
- goitre (smooth and non-tender)
- slow relaxing reflexes
- bradycardia
- obesity
984
Which investigations should be done in suspected acquired hypothyroidism?
- LOW T4 and HIGH TSH levels are found
| - ANTITHYROID ANTIBODIES if the cause is autoimmune
985
What is the management for acquired hypothyroidism?
- THYROXINE (lifelong)
| - monitor TFTs
986
What is obesity and how is obesity classified?
Obesity implies increased CENTRAL ABDOMINAL FAT MASS and can be calculated using
1) BMI
2) WAIST CIRCUMFERENCE
3) WAIST:HIP RATIO
Classified in children as:
- overweight- BMI>91st centile
- Obesity- BMI>98th centile
987
What are the causes of obesity?
1) Nutritional
2) Endocrine
3) Genetic
4) Iatrogenic
988
What are the features of nutritional obesity?
- Family history of obesity is common
- Social and emotional difficulties
- Tall height
- Early puberty
- Boys may appear to have small genitals
989
What are the endocrine causes of obesity?
- Hypothyroidism
- Cushings syndrome/disease
- Growth hormone deficiency
- PCOS
- Pseudohypoparathyroidism
990
What are the genetic causes of obesity?
- Prader-Willi syndrome
- Bardet-Biedl syndrome
- Monogenic causes (leptin deficiency)
991
What drugs can lead to obesity in children?
- Antidepressants
- Anticonvulsants
- Antipsychotics
- Corticosteroids
992
What is the presentation of an obese child?
- HIGH BMI
| - may have symptoms of organic disease depending on cause
993
What should be elicited in a history of an obese child?
- LIFESTYLE AND DIET- ask about physical activity and sedentary activities. take dietary history
- EMOTIONAL AND BEHAVIOURAL PROBLEMS- social/school issues
- COMPLICATIONS- MSK symptoms, snoring, lethargy
- LEARNING DIFFICULTIES
- ENDOCRINE symptoms
- FAMILY HISTORY
994
What investigations for cause should be carried out in an obese child?
- TFTS (low T4, high TSH in hypothyroidism)
- URINARY FREE CORTISOL (high in Cushings)
- KARYOTYPE and DNA ANALYSIS (genetic syndromes (PW)
- MRI of the BRAIN (hypothalamic cause)
995
What investigations for consequences of obesity should be carried out in children?
- URINARY GLUCOSE, OGTT- diabetes
- FASTING LIPID SCREEN- dyslipidaemia
- LFTs- fatty liver
996
What should be checked in examination of an obese child?
GROWTH
- nutritional obese children are generally tall
- short stature or fall off in height suggests pathological cause
ENDOCRINOLOGICAL SIGNS
- signs of hypothyroidism
- steroid excess
SIGNS OF DYSMORPHIC SYNDROME
- short stature
- microcephaly
- hypogonadism
- hypotonia
SIGNS OF COMPLICATION
- blood pressure
- - acanthosis nigricans
997
What is the management for obesity in children?
- SUPPORT
- Encouraging physical activity and reducing sedentary behaviour
- Balanced healthy diet
- Monitoring of comorbidity and management when needed
- Group programmes
998
What is phenylketonuria (PKU)?
An inborn error of amino acid caused by absent or virtually absent PHENYALANINE HYDROXYLASE (PAH) ENZYME ACTIVITY
999
What is the pathophysiology of PKU?
- PAH converts PHENYLALANINE to TYROSINE.
- The products of this are important in the formation of catecholamines, neurotransmitters and melanin.
- High concentrations of phenylalanine lead to concentrations of phenylpyruvic acid and phenylethylamine which are neurotoxic above a certain threshold.
1000
What is the presentation for PKU?
- Normal at birth- diagnosis comes from abnormal heel prick
- Fair with pale blue eyes
- Musty odour
- progressive developmental delay and mental retardation
- MRI evidence of demyelination
1001
What are the presentations in later childhood for PKU?
- Recurrent vomiting
- Eczematous skin eruptions
- Scleroderma-like skin lesions
- Seizures
- Self-mutilation
- Severe behavioural disturbance
- Hyperactive with rhythmic rocking and writhing movements affecting hands, face and tongue.
1002
What investigations are done in PKU?
- Guthrie heel prick assay for phenylalanine >12 hours after birth
1003
What are the values for high phenlyalanine?
> 120umol/L- suggests diagnosis and referral for lab testing
1004
What is the management for PKU?
- Specialised metabolic/paediatric clinic
- Dietary protein restriction combined with substitution of protein containing balanced mixture of amino acids
- Regular assay of plasma phenylalanine level
- Careful weaning is very important.
1005
How is short stature defined?
a height 2 STANDARD DEVIATIONS or more below the mean for the population.
On a standard growth chart, this represents a height <2nd gentile. Also >2SDs below mid parental height
1006
What is growth velocity disorder?
Abnormality slow growth rate across two major centile lines
1007
What are the causes of STEADY GROWTH below centiles?
CONSTITUTIONAL
- short parents
- normal history and exam
- no delay in bone age- no need for treatment
MATURATIONAL DELAY
- delayed onset of puberty
- family history of delay
- delayed bone age
TURNER'S SYNDROME
- 45XO
- no pubertal signs
- no delay in bone age
IUGR
- low birth weight
SKELETAL DYSPLASIAS
- body disproportionate with shortened limbs
- achondroplasia is most common
1008
What are the causes of FALL-OFF IN GROWTH across centiles?
CHRONIC ILLNESS
- identifiable on history and exam
- Crohn's and chronic renal failure, coeliac
- some delay in bone age
ACQUIRED HYPOTHYROIDISM
- clinical feature of hypothyroidism
- goitre may be present
- low T4, high TSH, thyroid antibodies
- delayed bone age
- Hashimoto's most commonly
CUSHINGS DISEASE
- usually iatrogenic due to prescribed steroids
- cushingoid features
- delayed bone age
GROWTH HORMONE DEFICIENCY
- congenital or acquired
- may occur with other hormone deficiencies
- delayed bone age
PSYCHOSOCIAL
- neglected appearance
- behavioural problems
- catch-up growth occurs when child is removed from home
1009
What should be elicited in a history of short stature?
- medical history and review of systems
- family history (mid parental height centile)
- birth history- SGA
- Psychosocial history- emotional neglect and abuse
1010
What should be checked in examination of a child with short stature?
- pattern of growth
- anthropometric mesures (obtain accurate measures of length, height and weight, plot on chart
- general examination for signs of hypothyroidism, body disproportion, stigmata of Turner's
- head circumference
1011
What investigations should be done in a child with short stature?
- FBC and PLASMA VISCOSITY- IBD
- U+Es- chronic renal failure
- COELIAC ANTIBODIES- screening for coeliac disease
- TFTs - hypothyroidism
- KARYOTYPE - Turner's
- GROWTH HORMONE TESTS (serum IGF-I) - hypopituitarism, growth hormone deficiency
- WRIST X RAY- bone age
1012
What is the management for a child with short stature?
DEPENDS ON UNDERLYING CAUSE
| Always offer information and support
1013
What is cerebral palsy?
A disorder of movement caused by a permanent, non-progressive lesion in the developing brain
1014
What are the general risk factors for cerebral palsy?
- Maternal age >35
- IUGR
- TORCH infection
1015
What are the antenatal risk factors for cerebral palsy?
- preterm birth (risk increases with decreasing gestational age)
- chorioamnionitis
- maternal respiratory tract or genito-urinary infection treated in hospitals
1016
What are the perinatal factors for cerebral palsy?
- low birth weight
- chorioamnionitis
- neonatal encephalopathy
- neonatal sepsis (with birth weight below 1.5kg)
- maternal respiratory tract or genito-urinary infection treated in hospital
1017
What are the post-natal factors for cerebral palsy?
- meningitis
- infections
- head injury
1018
How can cerebral palsy be classified?
In terms of movement:
1) SPASTIC CEREBRAL PALSY
2) ATHETOID CEREBRAL PALSY
3) ATAXIC CEREBRAL PALSY
4) MIXED CEREBRAL PALSY
In terms of distribution:
1) MONOPLEGIA (one limb)
2) DIPLEGIA (both lower/upper limbs)
3) HEMIPLEGIA (spasticity on one side of the body)
4) TRIPLEGIA (3 limbs)
5) QUADRIPLEGIA (4 limbs)
1019
What are the features of spastic cerebral palsy?
- Most common
- jerky movement
- intermittent increased tone
- pathological reflexes
- Motor cortex and pyramidal tract damage
1020
What are the features of athetoid cerebral palsy?
- constant motion of body and eyes
- increased activity (hyperkinesia)
- stormy movement
- basal ganglia damage
1021
What are the features of ataxic cerebral palsy?
- falls and stumbles
- loss of orderly muscular coordination
- movements are performed with abnormal force, rhythm and accuracy
- cerebellar damage
1022
What are the features of mixed cerebral palsy?
A combination of several forms
1023
When would you suspect diagnosis of cerebral palsy in a neonate?
- difficulty sucking
- irritability
- convulsions
- abnormal neurological examination
1024
What features are present for clinical diagnosis of cerebral palsy to be made in infancy?
1) ABNORMALITIES OF TONE- initially reduced, then spasticity develops
2) DELAY IN MOTOR DEVELOPMENT- marked head lag, delays in sitting and rolling over
3) ABNORMAL PATTERNS OF DEVELOPMENT- movements delayed and abnormal in quality
4) PERSISTENCE OF PRIMITIVE REFLEXES- Moro, grasp and asymmetric tonic neck reflex
1025
What are the causes of cerebral palsy?
- Spastic cerebral palsy: white matter damage (born in preterm, may occur in any functional level)
- Athetoid cerebral palsy: basal ganglia/deep grey matter damage
- congenital malformations- associated with higher levels of functional impairment than other causes
Sometimes it is not possible to identify a cause
1026
How do patients with cerebral palsy present?
1) Strongly associated with low APGAR score 5 mins after birth
2) delayed milestones
3) epilepsy (36%)
4) FTT
5) incontinence
6) drooling
7) behavioral problems
8) joint contractures
1027
What other conditions can be associated with cerebral palsy?
- Preterm birth
- TORCH
- congenital malformations
- maternal toxins
- maternal trauma and illness
- intracranial haemorrhage
- hyperbilirubinaemia
- hypoxia
- seizures
1028
What is a General Movement Assessment (GMA)?
Routine neonatal follow-up assessments for children between 0-3months who are at risk of developing cerebral palsy.
1029
What early motor features can be recognized in presentation of cerebral palsy?
- Unusual fidgety movements or other abnormalities of movement
- asymmetry/ paucity of movement
- abnormalities of tone, (hypotonia, spasticity, dystonia)
- abnormal motor development, including late head control, rolling and crawling
- feeding difficulties
1030
What are the most common delayed motor milestones in children with cerebral palsy?
- Not SITTING BY 8 MONTHS
- Not WALKING BY 18 MONTHS
- Early ASYMMETRY OF HAND FUNCTION BEFORE 1 YEAR
1031
What are the five levels of severity in cerebral palsy (GMFCS)?
LEVEL ONE: Fully independent, can perform most physical activities normally with only slight problems in balance or coordination
LEVEL TWO: Trouble balancing on uneven surfaces, requires use of railings when climbing stairs, but can walk independently for the most part; minimal ability in running and jumping.
LEVEL THREE: crutches or a wheelchair; may climb stairs using railing
LEVEL FOUR: Walk is severely affected, wheelchair use
LEVEL FIVE: Significant restrictions in voluntary control; cannot walk, sit or stand independently
1032
What investigations can be done in children with cerebral palsy?
- CLINICAL DIAGNOSIS
- MRI can demonstrate cerebral injury or malformations- but no always needed if diagnosis and extent of disease is clear.
- Thyroid studies
- Genetic testing
- CSF testing
- EEG
1033
Who is most likely to be involved in care for the child with cerebral palsy?
- Physiotherapists
- occupational therapists
- SALT
- dietetics
- psychology
- paediatricians
1034
What are the medical management options for cerebral palsy?
- BACLOFEN is helpful in relieving muscle spasm. PO, but can be intrathecal cannula in diffuse spasticity
- TIZANIDINE
- BOTULINUM TOXIN A
second line:
- DIAZEPAM
- DANTROLENE
- GABAPENTIN
1035
What are the surgical management options for cerebral palsy?
- Repair of scoliosis
- tendon lengthening or transfer to decrease the imbalance from muscle spasticity
- Osteotomy to realign a limb
1036
What alternative supportive management can be offered in cerebral palsy?
- mobility aids- orthotic devices, wheelchairs, powered mobility walkers
- deep brain stimulation in severe dystonic quadriplegic cerebral palsy
- physical methods of spasticity relief include heat, cold and vibration
- splinting can help to improve range of movement
1037
What other factors are important to consider in cerebral palsy patients?
PHYSICAL AND PSYCHOLOGICAL HEALTH OF CARERS
1038
What complications are associated with cerebral palsy?
- contractions
- GI symptoms: reflux, oropharyngeal disorders FTT
- osteoporosis
- pulmonary complications- aspiration pneumonia, bronchopulmonary dysplasia
- dental problems
- moderate/severe learning difficulties
- hearing loss
1039
What are the main types of fits and faints in infants and toddlers?
- Apnoea and acute life threatening events
- Febrile convulsions
- Breath holding spells (cyanotic)
- Reflex anoxic spells
- Infantile spasms
- Hypoglycemia and metabolic conditions
1040
What are the main types of fits and faints in school age children?
- Epilepsy
- Syncope (vasovagal)
- Hyperventilation
- Cardiac arrhythmias
1041
What do you need to elicit from a history of a fitting/fainting child?
- Description of the episode (precipitating factors, length of time, loss/altered consciousness)
- Home video recording
- A developmental history
- Family history
1042
What are the main causes of convulsions?
1) HEAD INJURY- history of trauma, intracranial bleeding
2) HYPOGLYCAEMIA- diabetes or inborn errors of metabolism, responds to glucose
3) MENINGITIS- fever and meningism
4) ASPHYXIAL INJURY- hypoxic episode (e.g. near-drowning/cardiac arrest)
5) EPILIEPSY
6) DRUG INGESTION
7) ELECTROLYTE IMBALANCE- hyponatraemia, hypocalcaemia
8) FEBRILE CONVULSIONS- high fever, generalised convulsion
1043
What is the general treatment for any type of convulsion?
- Give oxygen and maintain a patent airway
- Place the child in the recovery position
- Give buccal midazolam or rectal diazepam
- Correct any metabolic disturbance
- Give dextrose if hypoglycaemia is likely
- Consider IV anticonvulsants (lorazepam, phenytoin, phenobarbital)
- If prolonged status epilepticus, THIOPENTAL infusion and ventilation may be required
1044
What investigations should be carried out in a convulsion?
- BLOOD GLUCOSE: must always be checked in any fitting child
- U+Es, CA, MG: hyponatraemia, hypocalcaemia and hypomagnesaemia can cause fits
- LUMBAR PUNCTURE- If meningitis is suspected, but beware raised ICP
- CT/MRI scan- if history of trauma or focal neurological signs
- BLOOD, URINE CULTURES, THROAT SWAB, CXR- to look for any source of infection
- URINE TOXICOLOGY: in case of drug overdose
1045
What is a febrile convulsion?
Seizure with fever
- Most common between 6 MONTHS AND 5 YEARS
- Usually arise from infection/inflammation outside the CNS in an otherwise neurologically normal child.
Fevers arising from meningitis or encephalitis are NOT included in the definition for febrile seizure.
1046
What are the three classifications of febrile convulsion?
1) Simple febrile seizure
- isolated, brief, generalized tonic/clonic seizure (<15mins)
2) Complex febrile seizure
- (>15mins), focal features, repeat features with the same illness or incomplete recovery from seizure after one hour
3) Febrile status epilepticus
- duration is more than 30mins
1047
What are the risk factors for febrile convulsions?
- first degree relative who has had a febrile seizure
- relative with epilepsy
- infection with Human Herpes Virus 6 (HHV-6)
- iron and zinc deficiencies
1048
What should be elicited in history and examination of a patient with a febrile seizure?
- Assess cause of fever: respiratory distress, ENT examination, UTI
- Check blood glucose
- Exclude signs of CNS infection
- Check vital signs
1049
What investigations should be done in a child with a febrile seizure?
BLOOD TESTS: FBC, ESR, glucose, U+E, coagulation, culture
URINE: microscopy/culture if <18months, complex seizure or no infection found
LUMBAR PUNCTURE: child <12months (unless reviewed by senior)
1050
How should febrile convulsion be managed?
If child looks well, can be managed at HOME
1051
What advice should be given to parents after a child has had a febrile seizure?
- What a febrile seizure is
- remove excess clothing, give fluids, give antipyretics if child is uncomfortable. EXCESSIVE COOLING IS NOT RECOMMENDED
- Check for non-blanching rash, dehydration and stay with the child at night
- First aid if the child has a fit, turn the child into the recovery position, semi prone, with the knee flexed and the hand under the head
- Call 999 if the seizure lasts >5mins
1052
What are the criteria for admission to hospital following a febrile seizure?
- first febrile convulsion
- diagnostic uncertainty about the underlying cause of the seizure
- complex febrile seizure
- less than 18 months of age
- pretreatment with antibiotics (masked meningitis)
- unwell child
- social circumstance
1053
How is the convulsion terminated?
- Majority require no additional medical intervention.
| - Rectal diazepam/buccal midazolam if seizure persists longer than 3-4 minutes
1054
What febrile seizure prophylaxis can be offered to those with a history of prolonged/multiple seizures?
- Rectal diazepam at onset of febrile illnesses
| - Regular antiepileptics are rarely indicated
1055
What information should be given to parents about the prognosis of febrile seizures?
- 30% of children will experience 1 recurrence
| - 1-2% will develop epilepsy
1056
What are the doses for rectal diazepam in management of seizure?
- less than one month of age: 1.25-2.5mg
- 1 month to 1 year of age: 5mg
- 2-11 years of age: 5-10mg
1057
What are the doses for buccal midazolam in management of seizure?
- less than 6 months of age: 300mcg/kg (MAX. 2.5mg)
- 6 months- 11 months of age: 2.5mg
- 1-4 years of age: 5mg
- 5-9 years of age: 7.5mg
1058
What is a breath holding attack?
A period of time for which a child is holding their breath
The cause is not known- children who go through a stage of breath-holding fo not have a serious problem and are not epileptic
1059
What are the classifications for breath-holding attacks?
- CYANOTIC
- PALLID
and also:
- SIMPLE
- COMPLEX
1060
What is involved in a cyanotic breath holding attacks?
- Common in babies and toddlers- resolve by 18months
- Precipitated by crying due to pain or temper
- The child cries, takes a deep breath, stops breathing, becomes deeply cyanotic and the limbs extend
- Transient loss of consciousness and convulsive jerks may occur
- The child then becomes limp, resumes breathing and after a few seconds, is completely alert.
- This can last up to a minute
1061
What is involved in a reflex anoxic seizure (pallid)?
- Pallid spells or 'white' breath holding attacks
- 6 months to 2 years
- Bump on the head or other minor injury that triggers an excessive vagal reflex, causing transient bradycardia and circulatory impairment
- Child may or may not cry. then turns pale and collapses
- Transient apnoea and limpness followed by rapid recovery after 30-60 seconds
- child may roll eyes or experience incontinence
- poetical drowsiness helps distinguish these spells from epilepsy
- attacks disappear before school age
1062
What investigations should be done in breath holding attacks?
- EEG (hypsarrythmia in infantile spasms, 3 per second spike (absence seizures), epileptiform activity (epilepsy)
- ECG
- 24 hr ECG
- BLOOD CHEMISTRY
- pH MONITORING
1063
What management options are there for breath holding attacks?
- Spells may occur several times a week
| - REASSURE AND TREAT CHILD AS NORMAL
1064
What is epilepsy?
A common neurological disorder in childhood. Seizures and epilepsy affect infants and children more than any other age group.
1065
How is epilepsy defined?
TWO MORE MORE SEIZURES UNPROVOKED BY ANY IMMEDIATE IDENTIFIABLE CAUSE
1066
How are epileptic seizures classified?
- GENERALISED SEIZURES
| - FOCAL SEIZURES
1067
What are the causes for epilepsy?
- MALFORMATIONS (e.g. tuberous sclerosis and other hamartomas)
- INFECTIONS: meningitis and encephalitis; parasitic infections
- ELECTOLYTE DISTURBANCE e.g. hypernatraemia, hyponatraemia, hypoglycaemia, hypocalcaemia, hypomagnesamia, toxins
- TRAUMAS
- METABOLIC DISORDERS
- Triggers can include watching TV, flashing lights and lack of sleep
1068
What are the risk factors for epilepsy?
- Seizure triggers
- Family history of epilepsy
- Comorbid conditions (cerebrovascular disease/ cerebral tumors)
1069
What is important to elicit in a history of suspected epilepsy?
- Risk factors suggesting predisposition for epilepsy
- Any symptoms of auras (unexpected tastes, smells, paraesthesia etc)
- Specific features of generalized seizures (tonic/clonic/absence/myoclonic/atonic)
- Specific features of focal seizures (focal motor, focal sensory)
- Post-ictal period
1070
What symptoms can be found in the post-octal period following a seizure?
- Drowsiness/amnesia
- Injury (including bites to the sides of the tongue
- aching limbs or headache
- focal neurological deficit that slowly recovers
1071
What are the different types of generalized seizures?
1) Absence seizures
2) Myoclonic seizures
3) Atonic seizures
4) Clonic seizures
5) Tonic seizures
6) Generalised tonic-clonic seizures
7) Status epilepticus
1072
What are the features of absence seizures?
- Onset 4-12 years old
- Short episodes <20sec child stares and blinks with no apparent awareness of surroundings
- Can occur over 100 times a day
- No aura/post-ictal phase
- May present as daydreaming in school
- Usually undergo spontaneous remission in adulthood
EEG shows bursts of 3second spike and wave
1073
What are the features of myoclonic seizures?
- Sudden brief muscle contractions
- Often cluster within a few minutes
- Results in sudden falls
- If they evolve into rhythmic jerking movements they are classified as clonic seizures
1074
What are the features of atonic seizures?
- Brief loss of postural tone
- Results in falls and injuries
- Occurs in people with SIGNIFICANT NEUROLOGICAL SYMPTOMS
1075
What are the features of clonic seizures?
Rhythmic jerking movements
1076
What are the features of tonic seizures?
- Sudden onset extension/flexion of the head, trunk and/or extremity for several seconds
1077
What are the features of generalized tonic-clonic seizures?
- Tonic extension lasting for a few seconds followed by clonic rhythmic movements and a prolonged post-octal phased
- Often associated with a tongue biting, urinary or faecal incontinence
1078
What are the features of status epilepticus?
- A generalised convulsion lasting >30 mins or ...
| - Repeated convulsions occurring over 30mins without recovery of consciousness between each convulsion
1079
What are the different types of partial onset seizures?
1) Simple partial seizures
2) Complex partial seizures
3) Partial seizures with secondary generalization
1080
What are the features of simple partial seizures?
- Seizure with preservation of consciousness; includes sensory, motor, autonomic and psychic experiences
- tonic or clonic movements are initially localized but may move to different parts of the body
1081
What are the features of complex partial seizures?
Similarly to simple partial seizure but consciousness is impaired
1082
What are the features of partial seizures with secondary generalization?
Focal seizure followed by GTCS
1083
What are the different types of epilepsy syndromes?
1) Infantile spasms
2) Lennox-Gastau syndrome
3) Benign childhood centrotemporal spike
4) Juvenile Myoclonic Epilepsy
5) Panayiotopoulos syndrome
6) Benign Rolandic epilepsy
1084
What are the features of infantile spasms?
- Affects infants aged 4-8 months
- Clusters of myoclonic spasms; 'jack-knife' attacks where the child jerks forward with arms flexed and hands extended.
- Usually have chronic epilepsy and developmental delay
- poor prognosis
EEG: hypsarrhythmic pattern
1085
What are the features of Lennox-Gastau syndrome?
- Affects children aged 1-3 years
- Characterised by multiple seizure type
- Tonic-axial
- Atonic
- Absence seizures
- Developmental regression and learning disability
- Often chronic epilepsy resistant to therapy
1086
What are the features of benign childhood epilepsy with centrotemporal spikes?
- Older children may have focal or generalised seizures
- Large spike discharges over Rolandic area of one hemisphere
- Not associated with any structural lesion
1087
What are the features of juvenile myoclonic epilepsy?
- Affects adolescents
- Idiopathic generalised epileptic syndrome characterized by myoclonic jerks GTCS and sometimes absence seizures
- Usually occurs on awakening
- Requires lifelong treatment
- NOT associated with intellectual impairment
1088
What are the features of Panayiotopoulos syndrome?
- Common multifocal autonomic childhood epileptic disorder
- affects otherwise normal children
- onset 3-6 years
- prolonged seizures with autonomic symptoms and ictal vomiting
EEG: shifting and multiple foci, occipital dominance
1089
What are the features of benign Rolandic epilepsy?
- Benign focal epilepsy
- Children aged 4-10 years
- Common in boys
- Nocturnal seizures with facial twitching and aphasia
- May also have GTCS
1090
What are the differential diagnoses associated with epilepsy?
- Syncope
- Night terrors (children aged 6-8 years who suddenly awaken from a sound sleep, they are then wide-eyed, screaming, inconsolable, but have no recollection the following morning)
- Reflexive anoxic seizures
- febrile convulsions
- fabricated/induced illness by carers
- cardiac arrhythmias
- migraine
1091
What investigations are done in suspected epilepsy?
- EEG: epileptiform spike and wave activity correlates with different forms of epilepsy
- MRI: to rule out underlying pathology
- LUMBAR PUNCTURE: if infective cause is suspected
- ECG/ECHO/ lying and standing BP
1092
What are the main management options for epilepsy?
- REFER to neurology specialist including detailed description of the seizure
- STOP DRVING
- AVOID SWIMMING
- TAKE CARE IN THE BATH
- Medical management (see separate card)
- Ketogenic diet: high fat, low carbs and protein
- Vagal nerve and deep brain stimulation
1093
What are the medical management options for epilepsy?
Therapy should be started at the lowest limit and gradually increased.
If this drug doesn't work, initiate a second the same way, whilst decreasing the dose of the first.
- SODIUM VALPROATE
- CARBMAZEPINE
1094
What medication should be used in infantile spasms?
- VIGABATRIN
1095
What medication should be used in children with absence seizures only?
- ETHOSUXAMIDE as an alternative to valproate
1096
How are seizures managed acutely?
- ABCDE
- Check airway
- Lie the child in the recovery position
- Do not insert anything in their mouths
- Rectal diazepam or buccal midazolam if lasts longer than 5 mins
- If not responding, then status epilepticus algorithm
1097
What is the algorithm for the treatment of status epilepticus?
- AIRWAY, HIGH FLOW O2, CHECK GLUCOSE
If there is IV access:
- IV LORAZEPAM
- 2nd dose IV LORAZEPAM
- PARALDEHYDE PR
- PHENYTOIN IV/IO (give phenobarbital is already on phenytoin)
- CALL ANAESTHETIST: rapid sequence induction with TIOPENTONE
If there is no IV access:
- RECTAL DIAZEPAM or BUCCAL MIDAZOLAM
- if IV established... give 2nd dose LORAZEPAM
- PARALDEHYDE PR
- PHENYTOIN IV/IO (give phenobarbital is already on phenytoin)
- CALL ANAESTHETIST: rapid sequence induction with TIOPENTONE
1098
How often should children with epilepsy be reviewed?
every 3-12 months
1099
What are the side effects of valproate?
- vomiting
- anorexia
- lethargy
- hair loss
- hepatotoxicity
1100
What are the side effects of ethosuxamide?
- abdominal discomfort
- skin rash
- liver dysfunction
- leucopenia
1101
What are the side effects of clonazepam?
- drowsiness
- irritability
- behavioural abnormalities
- excessive salivation
1102
What are the side effects of carbamazepine?
- dizziness
- drowsiness
- diplopia
- liver dysfunction
- anaemia
- leucopenia
1103
What are the side effects phenytoin?
- hirsutism
- gum hypertrophy
- ataxia
- skin rash
1104
What is a seizure?
A clinical event with sudden disturbance of neurological function caused by an abnormal or excessive neuronal discharge. May be classified as epileptic or non-epileptic
1105
What are the causes of epileptic seizure?
- IDIOPATHIC (70-80%)
- SECONDARY
- - cerebral dysgenesis/malformation
- - cerebral vascular occlusion
- - cerebral damage e.g. congenital infection, HIE
- - cerebral tumour
- - neurodegenerative disorders
- - neurocutaneous syndromes
1106
What are the causes of non-epileptic seizures?
- FEBRILE SEIZURE
- METABOLIC
- - hypoglycaemia
- - hypocalcaemia/hypomagesaemia
- - hypo/hypernatraemia
- HEAD TRAUMA
- MENINGITIS/ENCEPHALITIS
- POISONS/ TOXINS
1107
How should diagnoses of fits be made?
Clinical decision made based on combination of description of attacks and symptoms.
1108
What investigations should be made in fits/seizures?
- EEG: only to SUPPORT a clinical diagnosis of epilepsy where the clinical history suggests epileptic seizure.
- NEUROIMAGING: only used to identify structural abnormalities that cause certain epilepsies
1109
What is a childhood headache?
Common complaint in older children and mostly due to non-specific viral infection, local infection or related to tension.
If a headache is acute and severe and the child is ill, serious pathology should then be considered.
1110
What are the main causes of headache in children?
- Tension headache (band like pressure, worse late in the day, precipitated by stress)
- Eye strain
- Migraine
- Sinusitis (bony tenderness)
- Dental issues
- Hypertension
- Analgesic headache (with frequent NSAID use)
- Infections
- Raised ICP
1111
What should be elicited in the history of a headache in childhood?
- SOCRATES
- concerning features?
- Associated emotional/behavioural problems
- Family history of migraine
- Affecting the child's ability to perform at school or attendance
- BINDS (birth, immunizations, neonatal period, development, school)
1112
What are potential causes for concern in childhood headaches?
- Acute onset of severe pain
- Worse on lying down
- Associated vomiting
- Developmental regression or personality change
- Unilateral pain
- Hypertension
- Papilloedema
- Increasing head circumference
- Focal neurological signs
- Bradycardia and hypertension
1113
What should be done in examination for childhood headache?
- ABCDE
- CNS, PNS, GALS examination
- Look for petechial rash, or any sign of trauma
- Check or otorrhoea/rhinorrhoea
- Examine the pupils and the funds looking for papilloedema
1114
What are the features of a tension headache?
CHARACTER: constricting band across front
TIMING: end of day
ASSOCIATED FEATURES: nil
O/E: normal
MANAGEMENT: reassurance and simple analgesia
1115
What are the features of a migraine with aura?
CHARACTER: throbbing, unilateral
TIMING: lasts a few hours, relieved by sleep, has triggers
ASSOCIATED FEATURES: AURA (visual disturbance, sensory/motor changes, nausea)
O/E: normal
MANAGEMENT: rest and analgesia, prophylaxis with PIZTIFEN/PROPRANOLOL, SUMITRIPTAN in older children
1116
What are the features of migraine without aura?
CHARACTER: bilateral, pulsatile over temporal/frontal area
TIMING: aggravated by physical activity
ASSOCIATED FEATURES: GI disturbance, photophobia, phonophobia
O/E: Normal
MANAGEMENT: Keep migraine diary, rest and analgesia, prophylaxis with PIZTIFEN/PROPRANOLOL, SUMITRIPTAN in older children
1117
What are the features of cluster headaches?
CHARACTER: sudden onset, severe, unilateral, peiorbital pain, non-pulsatile
TIMING: clusters a few times a day for a period of weeks
ASSOCIATED FEATURES: unilateral eye redness, orbital swelling/ tears
O/E: Normal
MANAGEMENT: SUMITRIPTAN acutely, CCBs (nifedipine)
1118
What are the features of raised ICP?
CHARACTER: worse on lying down, localised to lesion
TIMING:
ASSOCIATED FEATURES: morning vomiting, nocturnal pain, seizures
O/E: bradycardia, HTN, papilloedema, ataxia, torticollis, focal signs, visual field defects
MANAGEMENT: CT/MRI and treat cause
1119
What are the features of headache in meningitis?
```
CHARACTER: severe, acute
TIMING:
ASSOCIATED FEATURES: meningism
O/E: signs of infection and meningism, Kernig's sign, irritability, drowsiness
MANAGEMENT: meningitis management
```
1120
What is migraine?
Recurrent headache the lasts from 30 minutes to 48 hours- most common cause of primary headache in children
1121
What are uncommon forms of migraine?
1) FAMILIAL (linked to dominantly inherited calcium channel defect)
2) SPORADIC HEMIPLEGIC MIGRAINE
3) BASILAR TYPE MIGRAINE- vomiting w/ nystagmus and cerebellar signs
4) PERIODIC SYNDROMES: cyclical vomiting, abdominal migraine, benign paroxysmal vertigo of childhood
1122
What causes migraine in children?
- Imbalances in brain chemicals (serotonin)
| - Causes trigeminal system to release neuropeptides, which cause pain in the meninges
1123
What are the red flag symptoms for migraines?
- A short history
- Accelerated course, change in character over weeks/days
- Headache suggesting raised ICP
- Associated symptoms of personality changes, weakness, visual disturbances, confusion, focal weakness, seizures/ fever
- Underlying history of neurocutaneous syndrome, history of systemic illnesses
- Young age of child (<3)
1124
What investigations should be done for migraine in children?
HISTORY
| EXAMINATION
1125
What is the appropriate management for migraine in children?
ANALGESIA
- NSAID and paracetamol taken as early as possible
ANITEMETICS
- prochlorperazine and metoclopramide
SEROTONIN ANTAGONISTS e.g. SUMITRIPTAN
1126
What prophylactic agents should be given for children with migraine?
- Pizotifen (5-HT antagonist)
- Beta blockers- PROPRANOLOL
- Sodium channel blockers- VALPROATE or TOPIRAMATE
1127
When should a child be admitted to hospital following a suspected injury?
- History of loss of consciousness
- Neurological abnormality, persistent headache/vomiting
- Clinical/radiological evidence of skull fracture or penetrating injury
- Difficulty in making a full assessment
- Suspicion of NAI
- other significant medical problems
- Not accompanied by a responsible adult/ safeguarding concerns
1128
How does a subdural haemorrhage appear on CT?
CRESCENTIC SHAPE
1129
What are the causes of subdural haemorrhage?
- Secondary head trauma (shearing forces)
1130
What are the risk factors for subdural hemorrhage?
- Long term anticoagulants
| - high fall risk (epileptics)
1131
What is the pathology of a subdural haemorrhage?
- Tearing of bridging veins between cortex and venous sinuses
1132
What is the clinical presentation of a subdural haemorrhge?
- Insidious onset
- Fluctuating consciousness
- Physical/intellectual slowing
- Drowsiness
- Headache
- Change in personality
- Unsteadiness
1133
What investigations should be sone in suspected subdural haemorrhage?
- CT head: crescent shaped haematoma, possible midline shift
1134
What is the management for subdural haemorrhage?
- Surgical evacuation via burr holes
1135
What does an epidural haemorrhage looking like on CT scan?
- LENTICULAR SHAPE
1136
What is the cause of an epidural haemorrhage?
Secondary trauma
1137
What is the pathology for an epidural haemorrhage?
Fractured temporal/parietal bone following trauma to the temple just lateral to the eye.
- laceration of the middle meningeal vessel
- accumulation of blood between bone and dura
1138
What is the clinical presentation of an epidural haemorrhage?
- Lucid interval pattern of consciousness
- Eventual decrease of consciousness as ICP raises
- headache, vomiting
- altered mental state, seizures, hemiparesis, hyper-reflexia, upgoiing planters
- bradycardia
- hypertension
1139
What are the investigations for an epidural haemorrhage?
- XR SKULL- look for fracture crossing course of middle meningeal artery
- CT HEAD- lens shaped haematomas
- LP CONTRAINDICATED
1140
What is the management for an epidural haemorrhage?
Clot evacuation and ligation of bleeding vessel
1141
What is hydrocephalus?
Increase in the volume of CSF occupying the cerebral ventricles leading to dilatation of the ventricles.
CSF then permeates through the EPENDYMAL lining into white matter.
This causes damage and scarring which left untreated can lead to death
1142
What is arrested hydrocephalus?
When ICP returns to normal despite dilated ventricles and normal development can resume.
1143
What are the classifications of hydrocephalus?
- NON-COMMUNICATING (obstruction in the ventricular system)
| - COMMUNICATING (failure to reabsorb the CSF)
1144
What are the causes of non-communicating hydrocephalus?
- Congenital malformation
- Aquaduct stenosis (Bickers-Adams)
- Atresia of the outflow foramina of the 4th ventricle (Dandy-Walker malformation)
- Arnold-Chiari malformation
- Posterior fossa neoplasm
- Vascular malformation
- Intraventricular haemorrhage in preterm infant
- Toxoplasmosis
1145
What are the causes of communicating hydrocephalus?
- Subarachnoid haemorrhage
- Meningitis (pneumococcal, tuberculosis)
- Increased CSF production or viscosity
1146
What are the clinical signs of hydrocephalus in infants?
- Irritability, vomiting, impaired conscious level
- Rapid increase in head circumference
- Dysjunction of sutures, dilated scalp veins, tense fontanelle
- SETTING- SUN SIGN
- MACEWENS'S SIGN ( a cracked pot sound on percussing the head)
- Increased limb tone
- High pitched cry, seizures
- Cushings triad of BRADYCARDIA, HYPOTENSION, RESPIRATORY DEPRESSION
- Gradual onset manifests itself as failure to thrive and developmental delays
1147
What is the presentation of hydrocephalus in ACUTE ONSET?
- Headache and vomiting
| - Papilloedema and impaired upward glaze
1148
What is the presentation of hydrocephalus in GRADUAL ONSET?
- Unsteady gait due to spasticity
- Large head (although the sutures are closed)
- Unilateral or bilateral sixth nerve palsy secondary to increased ICP
1149
What investigations should be done in hydrocephalus?
CT SCAN:
- dilated lateral and 3rd ventricle
- - with NORMAL 4th ventricle: aqueduct stenosis
- - with ABNORMAL 4th ventricle: posterior fossa
- generalized ventricular dilatation suggests a communicating hydrocephalus
CENTILE CHARTS
- head circumference should be monitored over time on centile charts
1150
What is the management for hydrocephalus?
- LP can help if acute deterioration and it is a communicating cause
- FUROSEMIDE and ACETAZOLAMIDE inhibit secretion of CSF by the choroid plexus
- ISOSORBIDE promotes reabsorption
- VENTRICOPERITONEAL SHUNT
1151
What is plagiocephaly?
A disorder that affects the skull, making the bacl or side of the baby's head appear flattened.
Sometimes called deformational plagiocephaly
1152
What the causes of plagiocephaly?
- Pressure- most commonly from the the baby's sleeping position
1153
What is the presentation of plagiocephaly?
- Asymmetry of the skull with a normal head circumference
- No symptoms
- Does not cause any pressure on the baby's brain
- Development will not be affected later in life
1154
What us brachycephaly?
Flattening across the back of the skull, causing the head shape to appear much wider than usual
1155
What is the management for plagiocephaly?
- Early recognition will lead to better chances of improvement
- More time spent on tummy but still sleep on back
- Alter the position of toys or mobiles.
- Rolled up towel under the mattress can help the child sleep with less pressure on the flattest part of the head
- Physiotherapy for children with difficulty turning the head in one direction
1156
What are tics?
Rapid, repetitive, brief, involuntary movements.
| These can include blinking, jerking, facial grimacing
1157
What are the causes of tics?
- Extreme stress
- Medications: Ritaline, Dexedrin, Aderall, Tegretol
- rarely: encephalitis or meningitis
- viral infection and steptococcal
- PANDAS (paediatric autoimmune neuropsychiatric disorders)
1158
What is the presentation of tics?
- Eye linking, grimacing, nasal flaming, mouth opening
- tics become worse when people are stressed
- usually fluctuate in intensity
- vocal tics: humming, grunting, swearing
1159
How can tics be classified?
SIMPLE: purposeless (e.g. grunt, eye blink, muscle twitch)
COMPLEX: muscle movemnt with purpose (scratching)
VOCAL COMPLEX: one that produces a word
1160
What are the red flag symptoms associated with tics?
- Child is unable to stop
- child is frustrated
- has intense anxiety or pain when resisting
- Behaviour causes physical discomfort (neck strain/sore muscles)
- Behaviour interferes with functioning (in the middle of sports event/test)
1161
What investigations should be done in tics?
- FULL PHYSICAL AND NEUROLOGICAL EXAMINATIONS
atypical presentation (e.g. adult onset, learning difficulties, unusual physical features or autism spectrum) leads to referral to paediatrician, neurologist and clinical geneticist
1162
What is the management for tics?
Most children with tics do not require treatment but there are medical options
Conservative management includes: psychoogical counselling, behavioral modification, support groups.
1163
What is the medical management for tics?
- CLONDINE: available in patch form
| - centrally acting BP medication that may benefit tics and calm hyperactivity
1164
What is muscular dystrophy?
A group of congenital inherited disorders characteriesed by PROGRESSIVE MUSCLE WASTING and WEAKNESS.
They affect muscles in different patterns and are characteristically associated with a raised creatine kinase enzymes.
1165
What is Duchennes Muscular Dystrophy?
- X linked recessive condition
- Classically presents within the first 4 years of life with delayed motor milestones and mild speech delay
- Confined to wheelchair by 12
- Often die in early 20s
1166
What are the clinical features of DMD?
- motor delay
- inability to run
- cannot hop or jump and increased falls
- speech delay
- FTT
- fatigue
1167
What is seen on examination of DMD?
- Waddling lordotic gait
- Calf hypertrophy
- GOWERS SIGN: Weakness in limb girdles (lower more than upper)
- Sparing of the facial, extra-ocular and bulbar muscles
1168
What investigations are done in DMD?
- Markedly raised creatinine (10-100x)
- EMG
- Genetic analysis
- Muscle biopsy
1169
What are the differential diagnoses for DMD?
- Beckers muscular dystrophy- similar, but progresses slowly
1170
What is the management for DMD?
- Information and support for family
| - Genetic counselling as 50% of sons will be affected
1171
What is the management for early stages of DMD (up to 11 years old)?
PHYSIOTHERAPY
- stretching to prevent contractures
- knee-foot- ankle orthoses
- casting of ankles
CORTICOSTEROIDS: may prolong walking by 24 months and help with cardiorespiratory function
VIT D, CALCIUM, BISPHOSPHONATES
1172
What is the management for late stage DMD (> 11 years old)?
- Help with mobility- electric wheelchair
- Orthotics or surgery for contractors and scoliosis
- Cardiorespiratory surveillance (NIV d assist airway clearance)
- Support at school- Counselling?
1173
What is the management for the end stages of DMD?
- Wheelchair and other living adaptations
- Optimize respiratory and cardiac treatment
- Nutritional advice
- Respite and palliative care
- Planning ahead and end of life directives
1174
What is usually the cause of death in DMD?
Respiratory muscle failure
1175
What are the complications associated with DMD?
- Contractures
- Respiratory muscle failure: hypoventilation, loss of cough, infections and death
- Cardiomyopathy
- GI dilation or obstruction
- Learning difficulty
- Steroid complications
1176
What is Becker's Muscular Dystrophy?
- X linked recessive
- Same as DMD but LSOWER and MILDER
- Walking difficulties begin >16 years old
1177
How does BMD present?
- Presents as delayed walking
- muscle cramps
- weakness of proximal muscles in limbs during their mid 20s
1178
What is usually the cause of death in BMD?
Dilated cardiomyopathy
1179
What is spina bifida?
Failure of the neural tube (midline fusion of the dorsal vertebra) to close
1180
What is anencephaly?
Most severe type of spina bifida where there is complete failure of development of the cranial neural tube so brain does not develop
1181
What is the investigation for anencephaly?
- Raised alpha-fetoprotein (AFP) at 16-18 weeks gestation
- The 18-20 week fetal anomaly screening ultrasound
- CT/MRI for hydrocephalus or SC tethering
- Gait analysis
1182
What is the management for anencephaly?
Surgical closure of defect. MDT approach to help with disability
1183
What is the prognosis for anencephaly?
Death
1184
What is myelomeningocele?
Open lesion with malformed and exposed spinal cord covered by meninges
1185
What is the presentation of myelomeningocele?
- Severe neuro abnormality of legs, bladder and anus
| - Hydrocephalus in 90%
1186
What is the investigation for myelomeningocele?
- Raised alpha-fetoprotein (AFP) at 16-18 weeks gestation
- The 18-20 week fetal anomaly screening ultrasound
- CT/MRI for hydrocephalus or SC tethering
- Gait analysis
1187
What is the management for myelomeningocele?
Surgical closure of defect. MDT approach to help with disability
1188
What is the prognosis for myelomeningocele?
Major disability
1189
What is meningocele?
Spinal cord intact but defect involves exposed meninges which ruptures easily
1190
What is the presentation for meningocele?
Defect in back
1191
What is the investigation for meningocele?
- Raised alpha-fetoprotein (AFP) at 16-18 weeks gestation
- The 18-20 week fetal anomaly screening ultrasound
- CT/MRI for hydrocephalus or SC tethering
- Gait analysis
1192
What is the management of meningocele?
Surgical closure of defect. MDT approach to help with disability
1193
What is the prognosis for meningocele?
Normal if repaired
1194
What is spina bifida occulta?
Small defect covered with skin
Can lead to SC tethering
Leads to bladder, bowel and leg dysfunction
1195
What is the presentation of spina bidifa occulta?
- Naevus congenital sinus
- hypertrichosis (a hairy patch of skin)
- Asymmetrical gluteal cleft
- Scoliosis
1196
What is the investigation for spina bifida occulta?
- Raised alpha-fetoprotein (AFP) at 16-18 weeks gestation
- The 18-20 week fetal anomaly screening ultrasound
- CT/MRI for hydrocephalus or SC tethering
- Gait analysis
1197
What is the management for spina bifida occulta?
Surgical closure of defect. MDT approach to help with disability
1198
What is the prognosis for spina bifida occulta?
Normal
1199
What is nocturnal enuresis?
Bedwetting at night
1200
What are the normal ages for staying dry?
- Dry by day : 2 YEARS
- Dry by night: 3 YEARS
By 4 YEARS: 75% of children are dry by day and night
1201
What is primary enuresis?
If the child has never achieved dryness and is over 5 years old
1202
What is secondary enuresis?
A relapse after 6 months dry
1203
What are the risk factors for nocturnal enuresis?
- Family history
- Gender (BOYS)
- Delay in attaining bladder control
- Being obese
- Developmental delay (physical or intellectual)
- Constipation, face incontinence and day time urinary incontinence
- Psychological or behavioural disorders
- Sleep apnoea and upper airway obstructive symptoms
1204
What are the causes of primary bedwetting without daytime symptoms?
- Sleep arousal difficulties (inability to wake to noise, sensation of full bladder, bladder contractions)
- Polyuria- a larger than normal production of urine at night
- Bladder dysfunction- small bladder capacity or overactive bladder
1205
What are the causes of primary bedwetting with daytime symptoms?
Usually caused by disorders of the lower urinary tract such as:
- an overactive bladder
- structural abnormalities
- neurological disorders
- chronic constipation
- urinary tract infection
1206
What are the causes of of secondary bedwetting?
Often has an underlying cause:
- diabetes
- UTI
- constipation
- psychological problems
- family problems
1207
What should be elicited in a history of bedwetting?
1) PATTERN - how many nights per week, how many times a night, large amount of urine? Does the child wake up after bedwetting
2) FLUID INTAKE
3) PREVIOUS DRYNESS?
4) SYSTEMIC SYMPTOMS?
5) METHODS USED SO FAR?
6) BINDS ( birth, immunisations, nutritional, development, school)
1208
What should be assessed in physical examination for nocturnal enuresis?
- Examine the child's back for signs of congenital spinal malformations such as dimples/ hairy patch
- Palpable faecal mass (constipation)
- Evidence of renal disease
- Check for hypertension
1209
What investigations should be done for nocturnal enuresis?
- Urine microscopy and culture (UTI)
- Urine dipstick (to exclude glycosuria)
- Renal US if ectopic ureter is suspected
1210
What is the behavioural management for enuresis?
- STAR CHARTS: rewards for dry nights
- ENURESIS ALARM: (triggered my moisture and wakes child to go to the toilet)
- Ensure there is no excessive fluid intake
- Use the toilet before bed
1211
What is the medical management for nocturnal enuresis?
- DESMOPRESSIN- 1st line to children >7 years old where alarm has not worked
- given at night in tablet or nasal spray form
- IMIPRAMINE: only for resistant cases
1212
What is diurnal enureses?
- Lack of bladder control during the day in a child >3 years old
1213
What are the causes of diurnal enuresis?
- UTIs
- Neurogenic bladder
- Congenital
- Urgency incontinence
- Psychogenic
- Constipation
1214
What are the features of neurogenic bladder?
FEATURES: distended bladder, abnormal perinanal sensation and anal tone. Abnormal neurological findings in legs
MANAGEMENT: Refer, can cause long term kidney damage
1215
What are the features of congenital causes of diurnal enuresis?
FEATURES: continuous leakage, distended bladder
MANAGEMENT: refer for surgery
1216
What are the features of urgency incontinence in diurnal enuresis?
MANAGEMENT: void frequently and train child with stream interruption exercises.
1217
What are the features of psychogenic causes of diurnal enuresis?
FEATURES: Behavioural problems, recent stress
MANAGEMENT: reduce stress and positive encouragement
1218
What are the features of constipation in diurnal enuresis?
FEATURES: faecal mass palpable, worse when running, coughing lifting
MANAGEMENT: faecal disimpaction routine
1219
What should be done in examination for a child with daytime enuresis?
- ABDOMEN- bladder distension, pain or masses
- GENITALIA- inspect for seepage of urine
- BACK AND LEGS- midline lipoma, hairy patch, spinal deformity, reduced power and sensation in eg
- ANUS- for perianal sensation, but do not PR
1220
What are the medicines used in the management of diurnal enuresis?
DESMOPRESSIN - (first line, AVP analogue)
OXYBUTININ- anticholinergic
IMIPRAMINE- tricyclic antidepressant
1221
What are the recommended doses for desmopressin?
SHORT TERM: Desmotabs 200mcg initially at bedtime
LONG TERM: Desmotabs 200mcg increase to 400mcg
Stop after 3 months of use, for one week, to see if dryness has been achieved.
If not continue treatment for another 6 months
1222
What are the different types of UTI?
A) Lower UTI- a UTI involving the bladder and urethra
B) Upper UTI- a UTI involving the renal pelvis and/kidney pyelonephritis
C) Undifferentiated UTI- not possible to distinguish
D) Recurrent UTI
E) Asymptomatic bacteriuria
F) Atypical UTI
1223
What are the causes of UTIs in children?
RENAL ANOMALIES
UROLOGICAL ABNORMALITIES
BACTERIAL CAUSES
1224
What are the renal anomalies associated with UTIs in children?
- Solitary kidney: unilateral renal agenesis
- Ectopic kidney
- Multicystic dysplastic kidney
- Autosomal dominant polycystic kidney disease (ADPKD)
- Autosomal recessive polycystic kidney disease (ARPKD)
1225
What are the urological abnormalities associated with UTIs in children?
- Pelviuteric obstruction
- Posterior urethral valves
- Hypospadias
- Phimosis
- Paraphimosis
- Vesicoureteric reflux
1226
What are the bacterial causes of UTI in children?
- E. COLI- 85-90% of paediatric UTIs
- PROTEUS MIRABILIS
- STAPH. SAPROOPHYTICUS
- PSEUDOMONAS
1227
What are the risk factors for UTIs in children?
- Aged <1 year
- Female sex
- Caucasian race
- Previous UTI
- Voiding dysfunction
- Vesicoureteral reflux (VUR)
- Sexual activity
- No history of breastfeeding
- Immunosuppression
1228
What are the clinical features of UTI in infants <3 months?
- Pyrexia
- vomiting
- lethargy
- irritable
- poor feeding
- FTT
- abdominal pain
- jaundice
- haematuria
- Offensive urine
1229
What are the clinical features of UTI in infants >3 months (preverbal)?
- pyrexia
- abdo pain
- loin tenderness
- vomiting
- lethargy
- haematuria
- FTT
1230
What are the clinical features of UTI in infants and children >3 months (verbal)?
- frequency and dysuria
- dysfunctional voiding
- continence changes
- abdo pain
- loin tenderness
- malaise
- vomiting
- haematuria
- offensive/ cloudy urine
1231
What are the investigations for children with UTI?
- Child <3 months with sepsis: FULL SEPTIC SCREEN, urine sample for MC+S
- Child 3 months- 3 years with possible UTI: urine sample MC+S
- Child >3 years- URINALYSIS
- - leucocyte (+), nitrite (+): UTI
- - nitrite (+): probable UTI
- - leucocyte (+), nitrite (-): equivocal, seng urine MC+S
1232
What bottle is used for urine MC+S?
RED CAPPED
| containing BORIC ACID to preserve
1233
What are the interpretations of microscopy results?
Pyuria (+), Bacteriuria (+): UTI
Pyuria (+), Bacteriuria (-): Antibiotic treatment if clinical UTI
Pyuria (-), Bacteriuria (+): UTI
Pyuria (-), Bacteriuria (-): NO UTI
1234
What are the radiological investigations for UTI in children?
- Renal USS
- Radioisotope scanning
- MCUG (bladder filled with contrast via urethral catheter) detects reflux.
1235
What are the differential diagnoses for UTI in children?
- vulvovaginitis
- kawasaki disease
- voiding dysfunction
- sepsis with no urinary tract source
- threadworms
- meningitis
- possibility of child abuse
1236
What is the management of upper UTI in children?
ALL THOSE <3 months, refer
Consider referral in those >3 months
oral antibiotics- ciprofloxacin/co-amoxiclav 7-10 days
1237
What is the management of lower UTI in children?
ALL THOSE <3 months, refer
Oral antibiotics for 3 days according to local guidelines AND results of urine culture
- Trimethoprim
- Nitrofurantoin
- Cephalosporin
- Amoxicillin
1238
What are the doses for medicines in treatment of UTI in children?
oral CO-AMOXICLAV: 250/125mg every 8h
IV CO-AMOXICLAV: (child 1-2months)- 30mg/kg ever 12h
(child 3months-17y)- 30mg/kg every 8h
NITROFURANTOIN: (3months-11years) 750mcg/kg QDS
(12-17 yrs) 50mg QDS or 100mg BDS
1239
What are the complications of UTI in children?
- Chronic pyelonephritis
- Chronic renal failure
- hypertension
- Recurrence more likely in young girls
1240
What are the causes of haematuria in children?
1) Post-streptococcal glomerulonephritis
2) Polycystic kidneys
3) Renal stone
4) Renal tumour
5) Sickle cell disease
6) Renal trauma
7) UTI
1241
What are the causes of proteinuria in children?
1) Nephrotic syndrome
2) Acute renal failure
3) Orthostatic proteinuria
4) UTI
1242
What should be elicited in a history for haematuria/proteinuria?
- clear about what is described
- colour of urine (brown suggests renal, clots suggest bladder, red could be beetroot/rifampicin)
- other urinary symptoms (frequency, dysuria)
- precipitating factor?
- family history of renal disease
1243
What investigations should be done in haematuria/proteinuria?
- Urinalysis + culture: will identify blood/protein/infection
- FBC: for anaemia and to exclude HUS
- ASOT/throat swab: for evidence of of strep infection
- U+E: renal function
- Serum albumin: low in nephrotic syndrome
- Urinary protein/creatinine ratio: high in nephrotic syndrome
- Triglycerides and cholesterol: high in nephrotic syndrome
- Renal US: may show stones
- Renal biopsy: may show HTN, proteinuria, haematuria
1244
What is haemolytic uremic syndrome (HUS)?
Commonest cause of AKI in children, associated with thrombocytopenia, acute renal failure and hemolytic anaemia due to fragmentation of red blood cells
- Follows BLOODY DIARRHOEA is associated with E.Coli O157:H7
1245
What are the risk factors for HUS?
- Rural population > urban population
- Warmer summer months (june-September)
- Young age (6months-5 years)
- Older people or those with altered immune response
- Contact with farm animals
1246
How does HUS present in children?
- PROFUSE DIARRHOEA turns bloody 1-3 days later
- Fever
- Abdominal pain
- Vomiting
1247
What are the investigations for HUS in children?
- FBC and film
- Renal function and electrolytes
- Lactate dehydrogenase
- CRP
- CLotting screen
- Stool culture
- Urinalysis
1248
What is the management for HUS?
NOTIFIABLE DISEASE
| - Supportive: fluid and electrolyte management, antihypertensive therapy
1249
What are the complications associated with HUS?
- intestinal strictures and perforations
- intussception and rectal prolapse
- pancreatitis
- severe colitis
- altered mental state
- cerebrovascular accident
- seizures
- AKI
- CKD
- haematuria
- hypertension
- proteinuria
1250
What is nephrotic syndrome?
A combination of:
1) HYPOALBUMINAEMIA
2) OEDEMA
3) PROTEINURIA > 3-3.5g/24hr
4) HYPERLIPIDAMEIA
Occurs due to increased capillary wall permeability in the glomerulus which allows protein to leak urine
1251
What are the two main causes of nephrotic syndrome?
1) PRIMARY GLOMERULAR DISEASE
| 2) SECONDARY GLOMERULAR DISEASE
1252
What are the types of primary glomerular disease?
- Minimal change glomerular disease
- Focal segmental glomerulosclerosis
- Membranous glomerular disease
- Membranoproliferative glomerulonephritis
1253
What are the causes of secondary glomerular disease?
- Infection- HIV, hepatitis b and hepatitis C, mycoplasma
- Collagen vascular disease
- Metabolic diseases
- Inherited disease
- Malignancy
- Nephrotoxic drugs
- Pregnancy (pre-eclampsia)
- Transplant rejection
1254
What is the presentation of nephrotic syndrome?
- After viral URTI
- Insidious onset of oedema, initially perorbital and facial before becoming more generalized with pitting oedema
- Frothy urine
- Periorbital oedema
- Ascites and pleural effusion may subsequently develop
- HTN
- Increased risk of infection
- Leukonychia
1255
Which investigations of urine should be carried out in nephrotic syndrome?
- Urinalysis and protein (+++)
- Microscopy: haematuria and casts
- Culture
- Protein:creatinine ratio
1256
What investigations of blood should be carried out in nephrotic syndrome?
- Serum albumin
- U+E: decreased Ca and Na
- Normal C3/C4
- High cholesterol and TG
- ANF, ASOT, ANC, Ig
- Hb may be decreased
1257
What imaging investigations should be done in nephrotic syndrome?
- CXR
- Renal US
- Biosy
1258
What is the proposed management for nephrotic syndrome?
- Admit to hospital
- Fluid restriction, diuretics, low salt diet and corticosteroids
- Prednisolone is continued until there is remission of proteinuria
- Prophylactic penicillin is given until the proteinuria has cleared
1259
What are the complications associated with nephrotic syndrome?
- Increased risk of infection and thrombosis due to loss of antithrombin, plasminogen and Ig
- AKI and CKD
- Pulmonary oedema
1260
What is glomerulonephritis?
- Immune mediated disorder that causes inflammation within the glomerulus and other compartments of the kidney
1261
What are the classifications of glomerulonephritides?
MINIMAL CHANGE
DIFFUSE: affecting all glomeruli
FOCAL: affecting only some of the glomeruli
SEGMENTAL: only affecting parts of an affected glomerulus
1262
What are the post infectious causes of glomerulonephritis?
- Bacteria (strep, staph. aureus)
- Mycoplasma pneumonia, salmonella
- Virus- herpes viruses, EBV, CMV
- Fungi- Candidia, aspergillus
- Parasites- toxoplasma, malaria, schistomiasis
1263
What are less common causes of glomerulonephritis?
```
- IgA nephropathy
MPGN
- SLE
- Subacute bacterial endocarditis
- Shunt nephritis
```
1264
What is the clinical presentation of glomerulonephritis?
- Asymptomatic haematuria and/or proteinuria which occurs 1-2 weeks post throat infection
- Smoke/coke colored urine
- Oliguria
- Malaise, headache and loin discomfort
- Periorbital oedema
1265
What are the investigations for glomerulonephritis?
URINALYSIS: haematuria and proteinuria
URINE MICROSCOPY: granular and red cell casts
THROAT SWAB: strep infection
LOW C3
1266
What is the management of glomerulonephritis?
- Penicillin (10 days) if streptococcus infection was the cause
- Monitoring of haematuria and proteinuria
- Treatment of oedema with diuretics and potassium supplementation
- Fluid and salt restriction
- Anti-hypertensives
- Lipid lowering therapy
- Immunosuppression: corticosteroids, alkylating agents, cytotoxic
- Antithrombotics
- Intravenous immunoglobulin
- Dialysis
1267
What are the complications of glomerulonephritis?
- hypertension
- hyperkalaemia
- acidosis
- seizures
- hypocalcaemia
1268
What is hypospadias?
When the urethral meatus is on the underside of the penis
| Severe forms associated with chord (curvature of the penile head)
1269
How is hypospadias treated?
SURGERY:
- before the age of 2
- Use the foreskin (no circumcision)
- Straighten any cord if present and reconstruction of the urethra to the glans
1270
What is vulvovaginitis?
- Inflammation of the vulva/vagina and usually occurs secondary to infection related to bad hygiene
1271
Why is the risk of vulvovaginitis high in prepubertal girls?
- The proximity of the vagina to the anus
- Lack of estrogen- leads to thinning of the vaginal mucosa
- Lack of pubic hair to protect the area
- Lack of labial fat pads
1272
What is the presentation for vulvovaginitis?
- Vaginal discharge: white, yellow, green, foul odor
- Pruritis
- Dysuria
- Erythema
- Bleeding
1273
What is the aetiology for vulvovaginitis?
Infectious: Strep. pyogenes and staphylococcus aureus
Candidia infection- associated with antibiotic use
- *Chlamydia trachomatis
- *N. gonorrhea
- *Trichomonas
- *Herpes simplex
*suspicion of sexual abuse
1274
How is vulvovaginitis diagnosed?
- swabs and cultures
| - full STI panel if abuse is suspected
1275
What is the management for vulvovaginitis?
- improving hygiene
- wearing loose fitting underpants made out of cotton
- avoid use of bubble baths, perfumed soaps, fabric softeners
- If the vulval area is swollen/tender, cool compresses can be used to relieve pain
- If the child has not improved- 10 day course of amoxicillin/ co-amoxiclav
1276
What are examples of skin lesions in children?
- DESQUAMATION
- MACULOPAPULAR
- VESICLES
- WHEALS
- PAPULES
- PURPURA AND PETECHIAE
- MACULES
1277
What are the features of desquamation?
- Loss of epidermal cells producing scaly eruption
| - Post scarlett fever, Kawasaki's disease
1278
What are the features of maculopapular?
- mixture of macules and papules
- tend to be confluent
- seen in measles and drug rashes
1279
What are the features of vesicles?
- raised fluid filled lesions(<0.5cm)
- bullae are large vesicles
- seen in chicken pox
1280
What are the features of wheals?
- Raised lesions with a flat top and pale centre
| - seen in urticaria
1281
What are features of papules?
- Solid palpable projection above skin surface
| - seen in insect bites
1282
What are the features of purpura and petechiae?
- purple lesions caused by small haemorrhages in the skin
- do not fade with pressure
- seen in meningococcaemia, ITP, HSP, leukaemia
1283
What are the features of macules?
- flat pink lesions
| - seen in rubella, roseola, cafe-au-lait spot
1284
What is atopic eczema?
A chronic, relapsing, inflammatory skin condition characterized by an ITCHY RED RASH that favors the SKIN CREASES (flexor surfaces).
1285
What are the triggers for atopic eczema?
- soaps and shampoos
- infections (s.aureus)
- winter
- sweating
- contact allergens
- food allergens
- inhaled allergen (pollen, dust)
- stress
1286
How is diagnosis of eczema made?
MUST HAVE AN UTCHY SKIN CONDITION and 3+
- visible flexural dermatitis
- history of flexural dermatitis
- dry skin in the last 12 months
- history of asthma or hay fever or 1st degree relative with atopy
- visible flexural eczema
- <2 years old
1287
What is the presentation of eczema in children?
In infants- red and weepy on the cheeks which then extend to face, neck, wrists and hands
- marked pruritis which can lead to weeping, crusting and secondary infections
- preschool= often remission, but some children will persist with lesions in antecubital and popliteal fossae, behind ears, on face and neck
- School years= dry thickened and the face can become white.
- Hyperpigmentation, scaling and lichenification become prominent
1288
What investigations are done in atopic eczema?
Rarely required
Can swab and culture if evidence of secondary infection
Can measure IgE and specific RASTs to confirm atopic nature of child
1289
What is the management for mild eczema?
EMOLLIENTS
| MILD TOPICAL CORTICOSTEROIDS: 1% hydrocortisone
1290
What is the management for moderate eczema?
EMOLLIENTS
MODERATE TOPICAL CORTICOSTEROIDS: Eumovate
TOPICAL CALCINEURIN INHIBITORS: topical tacrolimus
BANDAGES
1291
What is the management for severe atopic eczema?
```
EMOLLIENTS
POTENT TOPICAL CORTICOSTEROIDS
TOPICAL CLACINEURIN INHIBITOR
BANDAGES
PHOTOTHERAPY
SYSTEMIC THERAPY
```
1292
What are the signs of eczema herpeticum?
- Areas of rapidly worsening, painful eczema
- Clustered blisters consistent with early stage cold sores
- Punched-out erosions (circular, depressed, ulcerated lesions)
- Possible fever, lethargy or distress
1293
What are the complications associated with childhood eczema?
- Flare ups are common, often for no obvious reason
- Infection with Staphylococcus or Streptococcus or HSV
- Psychosocial impact
1294
What is impetigo?
- Impetigo is very contagious so child should be excluded from school until it has fully healed.
- Nasal carriage is often the source of infection
1295
What is the causative organism for impetigo?
Staphylococcus aureus or beta-hemolytic streptococcus
1296
How is impetigo classified?
- BULLOUS
| - NON-BULLOUS
1297
What is the non-bullous presentation of impetigo?
- Start as tiny pustules that evolve into honey-colored crusted plaques <2cm.
- Usually on the face (particularly around the mouth and nose)
- Satellite lesions may occur as a consequence of autoinoculation.
- Itching
- Regional lymph nodes can be enlarged
- Can develop into necrotic ecthyma
1298
What is the bullous presentation of impetigo?
- Thin roof and tend to rupture spontaneously.
- Face, trunk, extremities, buttocks or perineal regions
- More likely to occur on top of other disease like atopic eczema
- Little erythema, no regional lymphadenopathy
- Bullous lesions are more common in neonates
- More likely to be painful with systemic symptoms
1299
What is the management for impetigo?
- Stay off school until lesions are dry or on on abc for 48hrs
- Keep area clean and do not scratch
- Do not share towel
- Topical antibiotics if <5 lesions applied after crusts have been soaked off with water and soap
- if extensive, FLUCLOXACILLIN/ERYTHROMYCIN PO for 7 days
1300
What is nappy rash?
- Irritative rash
| - commonly candidiasis superimposed
1301
What are the different types of nappy rash?
- Ammoniacal dermatitis
- Candidal nappy rash
- Seborrhoeic nappy rash
- Psoriatic nappy rash
1302
What are the features of ammoniacal dermatitis?
- Erythematous or papulovesicular lesions, fissures and erosions
- Skin folds spared
- caused by irritation from excretions and chemicals
- unusual with modern disposable nappies
- treat by regular washing, changing and exposure to air and use of protective creams
1303
What are the features of candid nappy rash?
- bright red rash with clearly demarcated edge
- satellite lesions beyond border
- inguinal folds usually involved
- may also have oral thrush
- treatment with nystatin cream and orally if necessary
1304
What are the features of seborrhoeic dermatitis?
- pink, breast lesions with yellow scales
- often in skin folds
- cradle cap may be present
- treat with mild topical corticosteroids
1305
What are the features of psoriatic nappy rash?
- appearance similar to seborrhoeic dermatitis
| - positive family history of psoriasis
1306
What are the management options for nappy rash?
- frequent nappy changes and washing
- barrier cream: zinc and castor oil cream
- mild hydrocortisone cream
- topical antifungal: nystatin ointment 6 hourly (if severe, may benefit from oral anti fungal simultaneously)
1307
What is infantile seborrhoeic dermatitis (cradle cap)?
- Affects infants and causes yellowfins crusty, greasy scaling
- Papulosquamous disorder affecting the areas with most sebum, such as the scalp, face and trunk.
- Extremely common in infants
- Presents in the first 6 weeks
1308
What is the presentation of cradle cap?
- Greasy yellow scaling patches
loss of small amounts of hair in the area of scalp affected
- Some areas of redness around the plaques
- Tends to be very scaly on the scalp, whereas in the flexural areas and on face it may be more erythematous
1309
What are the investigations for cradle cap?
- Diagnosis is made on clinical appearance alone
- Be wary of tinea capitis
- Can look very similar to psoriasis
1310
What is the management for cradle cap on the scalp?
- Reassurance for the parents
- Regular washing of the scalp with baby shampoo, followed by brushing with a soft brush to loosen scale
- soften scales with baby oil
- if necessary crusts may be soaked overnight before washing, with white petrolleum jelly
1311
What is the management for infantile seborrhoeic dermatitis on other areas of skin?
- Baby should be bathed at least once a day and an emollient used as a soap substitute
Imidazole cream may be used once or twice a day
- If widespread, severe and unresponsive to treatment.
1312
What are the complications associated with infantile seborrhoeic dermatitis?
- Secondary infection
| - Leiners disease
1313
What is Steven-Johnsons Syndrome?
An immune-complex-mediated hypersensitivity disorder.
It ranges from mild skin and mucous membrane lesions to a severe and sometimes fatal systemic illness.
It is more common in females
More common in those with HIV
1314
What are the risk factors/triggers for SJS?
- Drugs (75%) (anticonvulsants and antimicrobials)
- Infections (25%)
- Genetic factors (HLA B) are associated with predisposition to SJS
1315
What is the systemic presentation of SJS?
- Non-specific URTI with fever, sore throat, chills, headache, arthralgia, vomiting and diarrhoea and malaise)
- Mucocutaneous lesions develop suddenly
- Severe oromucosal ulceration of mouth
- Cough productive of a thick purulent sputum
- Dysuria or an inability to pass urine
- Ocular symptoms: painful red eye, purulent conjunctivitis, photophobia, blepharitis
- General examination: fever, tachycardia, hypotension, altered level of consciousness, seizures, coma
1316
What is the dermatological presentation of SJS?
- lesions affecting the palms, soles, dorsum of hands and extensor surfaces
- the rash can begin as macules that develop into papules, vesicles, bullae, urticarial plaques or confluent erythema.
- Target lesions
- The skin becomes susceptible to secondary infection
- Urticarial lesions are usually not pruritic
- Nikolsky sign
1317
What is Nikolsky sign?
- present when slight rubbing of the skin results in exfoliation of the outermost layer.
- Dislodgement of intact superficial epidermis by a shearing force, indicating a plane of cleavage in the skin at the dermal-epidermal junction
1318
What are the best investigations for SJS?
- Assess level of dehydration with routine bloods and glucose
- Skin biopsy
1319
What is the best management for SJS?
- ABCDE
- Supportive, as for severe burns, hydration, airway protection
- Identifying causative antigen and remove/treat
- Frequent emollient ointment
- Specialist eye care
- Systemic corticosteroids or immunoglobulin used in the 1st 2-3 days
1320
What are the complications of SJS?
TOXIC EPIDERMAL NECROSIS (TEN)
1321
What is erythema nodosum?
A type of panniculitis: inflammation of the fat lying underneath the skin.
It causes red nodules to form just below the skin surface (usually on the shins)
1322
What are the associated diseases and causes of erythema nodosum?
- STREPTOCOCCAL INFECTION
- TB
- Mycoplasma infection
- IBD
- Sulphonamides
- Viruses
- Idiopathic (30%)
1323
What is the presentation of erythema nodosum?
- Begins with fever, aching, arthralgia whilst a painful rash usually appears in 2 days
- Lesions are poorly defined, red, tender nodules.
- In the first week, the lesions become tense, hard and painful
- In the second week, they may become fluctuant- rather like an abscess- but they do not suppurate or ulcerate.
- Individual lesions last around2 weeks but occassionally, new lesions continue to appear for 3-6 weeks.
- Aching legs and swollen ankles may persist for many weeks
- Commonly in the anterior aspect of the lower leg.
1324
What investigations are done for erythema nodosum?
- THROAT SWAB for strep and anti-strep
- FBC, ESR: ESR often very high
- Stool examination for Y. enterocolitica, Salmonella and Campylobacter
- Blood cultures
- In sarcoidosis, calcium and ACE are often raised
- CXR: bilateral hilar lymphadenopathy
- Intradermal skin tests
- Excisional biopsy
1325
What is the management for erythema nodosum?
- Treat underying disease
- Cool compresses and bed rest
- NSAIDs
Usually resolves in 6 weeks if infectious
1326
What are scabies?
Itchy rash caused by the parasitic mite SARCOPTES SCABEI.
1327
What is the presentation of scabies?
- Itchy papular rash with visible burrows affecting finger and toe webs.
Also affects palms, soles, wrists, groins, axillary folds, and buttocks.
1328
What is Crusted Norwegian scabies?
HYPERINFESTATION due to insufficient immune response.
- Develop hyperkeratotic lesions
- Lymphadenopathy
1329
What are the risk factors for scabies?
- Overcrowding
- Poverty
- Poor hygiene
- Care homes
- Sexual contact
1330
What investigations are done in scabies?
CLINICAL DIAGNOSIS
| but confirmed with SKIN SCRAPINGS
1331
What is the management for scabies?
All contacts must apply treatment on the same day
TOPICAL APPLICATION OF PARASITICIDAL PREP overnight to the whole body except the face. This should be repeated a week later
1. 1ST LINE: PERMETHRIN 5% DERMAL CREAM
2. 2ND LINE: MALATHION 0.5% AQUEOUS LIQUID
1332
What are the sign of joint inflammation in children?
- swelling
- pain
- heat
- reduced range of movement
1333
What are some of the causes of joint inflammation in children?
1) Septic arthritis
2) Juvenille idiopathic arthritis
3) Hemophilia
4) Viral infection
5) Psoriasis
6) Trauma
7) Crohn's disease
8) HSP
1334
What should be elicited in a general history of joint inflammation?
- Main joint symptoms (SOCRATES)
- Presence of systemic symptoms?
- Past medical and family history
1335
What investigations should be done in joint inflammation?
- FBC: bacterial infection, anaemia, haemoglobinopathies
- CRP: elevated in bacterial infection, IBD, collagen vascular disease
- BLOOD CULTURE: positive in septic arthritis
- ASOT: high in reactive arthritis
- VIRAL TITRES: viral arthritis
- RHEUMATOID FACTOR: negative in most forms of juvenile arthritis
- RADIOGRAPHY of the joint
- JOINT ASPIRATION: microscopy and culture
- MRI of joint
1336
What should be examined in a child with joint inflammation?
- MSK system: all four limbs and spine, look for skin changes, heat, tenderness, range of motion and asymmetry
- OBSERVE the gait
- general examination
- look for focus of infection
1337
What are the malignant causes of leg pain and limp in childhood?
- neuroblastoma
- ewings sarcoma
- osteosarcoma
1338
What are the infective cause of leg pain and limp in childhood?
- septic arthritis
- TB and malaria
- Lyme disease
- Viral
1339
What are the benign tumour causes of leg pain and limp in childhood?
osteoid sarcoma
1340
What are the trauma causes of leg pain and limp in childhood?
- NAI
- sprains and contusions
- ill fitting shoes
1341
What are the neurological causes of leg pain and limp in childhood?
- cerebral palsy
- DMD
- Poliomyelitis
- Spina bifida
1342
What are the congenital causes of leg pain and limp in childhood?
- congenital limb deficiency/ shortening
1343
What are the haematological causes of leg pain and limp in childhood?
- Acute lymphocytic leukaemia
- HSP
- Sickle cell disease
- Thalassaemia
1344
What is the rheumatological causes of leg pain and limp in childhood?
Juvenile idiopathic arthritis
1345
What are some other causes of leg pain and limp in childhood?
- Transient synovitis
- Perthes disease
- Growing pains
1346
What should be elicited in a joint history?
- Any injury, trauma, surgery, known inflammatory joint disease, IBD, psoriasis, arthropathies?
- Underlying developmental conditions
- Possibility of tick exposure
- Developmental history (late onset of walking, unusual gait/clumsiness)
- family history of gait problems
- systemic symptoms, inflammatory symptoms, current level of function
- previous treatment/response
1347
What red flags may indicate serious disease in a child with acute limp?
- PAIN WAKING CHILD AT NIGHT (malignancy)
- REDNESS, SWELLING, STIFFNESS (infection/IFD)
- WEIGHT LOSS, ANOREXIA, FEVER, NIGHT SWEATS (infection/malignancy)
- UNEXPLAINED RASH OR BRUISING (IFD, hematological, NAI)
- LIMP and STIFFNESS WORSE IN THE MORNING (IFD)
- SEVERE PAIN, ANXIETY and AGITATION (evolving compartment syndrome)
1348
What is septic arthritis?
Serious infection of the joint space, which can lead to bone destruction.
- Most common in <2
- Can occur following a puncture wound or infected skin
- usually only one joint is affected
- hip concern in infants and young children
1349
What are the causative organisms for septic arthritis?
- STAPH AUREUS
- H. Influenzae (less common)
- GONORRHOEA in adolescents
1350
What is the typical presentation for septic arthritis?
- Erythematous
- warm
- acutely tender joint
- reduced range of movement
- acutely unwell, febrile
- joint effusion (patella tap)
- in neonates- limb is immobile
1351
What investigations should be done in septic arthritis?
- WCC, CRP, ESR (all raised)
- BLOOD CULTURES
- US HIP (identify an effusion)
- XRAYS (exclude trauma and other bony lesions)
- MRI (may indicate adjacent osteomyelitis/abscess)
- JOINT ASPIRATION for culture, polarising microscopy to exclude crystal arthropathy
- LYME DISEASE BLOOD TEST
1352
What are the differentials associated with septic arthritis?
- Primary rheumatological disorders (gout, psuedogout, vasculitis, osteoarthritis)
- Drug induced arthritis
- Reactive arthritis, post-infectious diarrhoeal syndrome, post-meningococcal and post-gonococcal arthritis, arthritis associated with intrinsic bowel disease
- Lyme disease
- Viral arthritis (often symmetrical and smaller joints)
- Infective endocarditis
1353
What are the criteria of urgent assessment for a limping child?
- <3 years
- >9 years with painful/restricted hip movements
- unable to weight bear
- has fever or other red flags
- severe pain/agitation, reduced peripheral pulses/ muscle weakness
- NAI suspicion
1354
What is the treatment for septic arthritis?
- IV FLUCLOXACILLIN FOR 4-6 WEEKS (CLINDAMYCIN)
- MRSA= VANCOMYCIN
- Gonococcal= IV CEFOTAXIME switch to oral after 2 weeks
- Washing out of the joint or surgical drainage
- Joint immobilisation in a functional position, but then mobilised
- Splint joint in position of function
- Physiotherapy to restore function
1355
What is Developmental Dysplasia of the Hip (DDH)?
The acetabulum is shallow and does not adequately cover the femoral head, leading to the hip joint being dislocatable or dislocated
1356
What are the 4 F's (risk factors for DDH)?
1) FRANK BREECH POSITION
2) FAMILY HISTORY
3) FEMALE SEX
4) FIRST BORN
1357
What are additional risk factors for DDH?
- oligohydramnios
- spina bifida
- cerebral palsy
- talipes
- twins
1358
What is the presentation for DDH in <3 months?
- Hip dislocates on Barlow's and Ortolani's
1359
What is BARLOW'S test?
- Hips in adducted position, slight gentle posterior pressure is applied to the hips
- "CLUNK" is felt as the hip subluxates out of the acetabulum
1360
What is ORTOLANI'S test?
- Examiner's thumb is placed over the patient's inner thigh, and the index finger is gently placed over the greater trochanter.
- The hip is abducted and gentle pressure is placed over the greater trochanter.
- In DDH there will be a "CLUNK" when the hip is reduced
1361
What is the presentation for DDH in >3months?
- One leg appears shorter than the other in Galeazzi's test
1362
What is GALEAZZI SIGN?
- patient lies supine and the hips/knees are flexed
| - One leg appears shorter than the other
1363
What investigations should be done in DDH?
Should be picked up in 6-9 week baby check
- observation of symmetrical skin creases
- observation of symmetrical leg length
- Barlow's test
- Ortolani's test
1364
What is the management for DDH?
- Principle is to immobilize the hip joint with a splint (PAVLIK harness) for 3 months to allow development of the ACETABULAR RIM.
This should be help in flexion and abduction
SURGERY
- for children in whom non-operative treatment has failed, and in children diagnosed over 6 months of age
1365
What are the complications associated with DDH?
- Osteoarthritis
- lower back pain
- surgical issues
- hip replacements in later life
- degenerative joint disease
1366
What is reactive arthritis?
An acute inflammatory arthritis occurring with or following intercurrent infection, but without evidence of causative organisms in joint
1367
What are the causes of reactive arthritis?
- Typically follows 7-10 days after gastroenteritis involving lower limb large joints (knee>ankle>hip)
- Infections due to Shigella, Salmonella, Yersinia, Campylobacter.
- Adolescents (gonoccoal/chlamydia) infections
- Reiters disease
1368
What is Reiter's disease?
TRIAD OF:
- Urethritis
- Conjunctivitis
- Arthritis
1369
What is the presentation of reactive arthritis?
- Limp
- Inflammation and effusion can cause sudden hip pain
- UNILATERAL but may travel to other joints on the same side
- Child keeps the hip and leg flexed and externally rotated and abducted
- Developed 2-4 weeks after GU/GI infection
- No pain at rest
- The onset is most often acute with malaise, fatigue and slight fever
- Low back pain
- Heel pain is common
- Skin changes (erythema nodosum), apthous ulcers
1370
What are the investigations for reactive arthritis?
- blood and synovial fluid cultures are NEGATIVE
- ESR, CRP very high
- FBC- normocytic normochromic anaemia, mild leukocytosis and thrombocytosis
- HLA-B27 positive in the majority of those affected
- High WCC in joint aspiration
- Stool culture to identify causative organisms
- GUM referral and serology for detection of chlamydia
- XRAYS- normal to start but eventually show periosteal reaction
- ECG in patients with prolonged disease to assess for conduction disturbances
1371
What is the management for reactive arthritis?
- Rest and aspirate effusion
- Physio
- NSAIDs
- Intra-articular steroid injection
1372
What is the prognosis for reactive arthritis?
- SELF LIMITING usually resolves within 12 months
| - May develop long term arthritis particularly if HLA-B27 positive
1373
What is juvenile idiopathic arthritis?
A group of conditions that presents in childhood with joint inflammation lasting 6 weeks for which no other cause is found.
Treatment is aimed at treating the pain and inflammation and maintaining good joint mobility.
1374
How is juvenile idiopathic arthritis classified?
1) SYSTEMIC (Still's disease)- 9%
2) POLYARTICULAR - 19%
3) PAUCIARTICULAR - 49%
4) SPONDYLO-ARTHROPATHIES (HLA B27)- 7%
5) JUVENILE PSORIATIC ARTHRITIS- (7%)
6) OTHER
1375
What is the presentation of Pauciarticular arthritis (up to 4 joints)?
- 70% ANA positive
- usually < 6 years old
- usually female
- swollen joint with reduced ROM but not much pain
- child feels well
- commonly affects knee and ankle
- high risk of chronic uveitis
1376
What is the presentation of Still's disease?
- Arthritis with at least two weeks of daily fever
- There must be at least one of:
- - RASH
- - LN ENLARGEMENT
- - HEPATOSPLENOMEGALY
- - SEROSITIS
Other features include:
- daily high spiking fever
- salmon rash with fever
- symmetrical affecting several joints
1377
What is the presentation for polyarticular arthritis (more than 4 joints)?
RF NEGATIVE
- toddlers and pre-adolescent
- females
- stiffness with minimal swelling
- destructive arthritis
RF POSITIVE
- symmetrical involvement of small joints
- swelling and stiffness
- rheumatoid nodules
Systemic symptoms:
- fever
- hepatosplenomegaly
- lymphadenopathy
- serotisis
1378
What are the investigations for JIA?
- CLINICAL DIAGNOSIS
- ESR raised
- ANA positive
- XR
- ECHO
1379
What s the management for JIA?
- NSAIDs and corticosteroid injections
- DMARDs: methotrexate is 1st line if multiple joints or injections of CS
- Immunosuppressants: cyclosporin, azathioprine
- Biological drugs: Etanercept, infliximab, adalimumab
1380
What are the conservative management options for JIA?
- Hydrotherapy
- Physiotherapy
- Occupational therapy
- Psychological support
1381
What are the complications associated with JIA?
- joint deformities
- uveitis
- osteoporosis
- growth and sports restriction
1382
What is Perthes Disease?
Self-limiting hip disorder caused by avascular necrosis of the femoral head.
- Can follow an episode of transient synovitis.
Complex process of stages that can last several years.
Weakened bone of the head of the femur begins to break and fall apart
1383
What are the risk factors and epidemiology of Perthes Disease?
- 4-8 year old boy with delayed skeletal maturity
- MALE (ratio 5:1)
- Caucasian
- low birth weight
- family history
1384
What is the pathogenesis of Perthes Disease?
- Loss of blood supply (avascular necrosis) of the nucleus of the proximal epiphysis
- Abnormal growth of the epiphysis results
- Eventual remodelling of regenerated bone
- symptoms occur with subchondral collapse and fracture
1385
What are the four stages in Perthes Disease?
1) INITIAL/NECROSIS
2) FRAGMENTATION
3) REOSSIFICATION
4) HEALED
1386
What occurs during the initial stage of Perthes disease?
- blood supply to the femoral head is disrupted and bone cells die
- area becomes inflamed
- child may begin to sho signs of the disease, such as a limp or different way of walking
- lasts for several months
1387
What occurs during the fragmentation stage of Perthes disease?
- 1-2 years, the body removes the dead bone and quickly replaces it with initial, softer bone (woven bone)
- bone is in a weaker state
- head of the femur is more likely to break
1388
What occurs during the reossification stage of Perthes disease?
- New, stronger bone develops and begins to take shape in the head of the femur
- Longest stage of the disease
- few years
1389
What occurs during the healed stage of Perthes disease?
- Regrowth is complete an the femoral head has reached its final shape
- How close the shape is to round will depend on: extent of damage in fragmentation phase, and childs age of onset
1390
What is the presentation of Perthes Disease?
- Pain in the hip or knee and causes a limp
- An effusion (from synovitis)
- Change in way child walks/runs
- Pain worsens with activity, gets better with rest
- limited movement of the hip
- ANTALGIC GAIT due to pain
- TRENDELENBERG GAIT seen in late phase
- No history of trauma
- Roll test
1391
What investigations should be done in Perthes disease?
- FBC and ESR
- Early XRAYS
- Technetium bone scan
- MRI
- decrease in size of the nuclear femoral head with patchy density on XRay
- Hip aspiration if a septic joint is suspected
1392
What are the differentials associated with Perthes Disease?
BILATERAL PRESENTATION
- Hypothyroidism
- Multiple epiphyseal dysplasia
- Sickle cell disease
UNILATERAL PRESENTATION
- Septic arthritis
- Sickle cell disease
1393
What is the management for Perthes Disease?
- Refer to orthopaedics
- bracing/traction
- physiotherapy
- surgery in children >6 with >50% femoral head necrosis (PROXIMAL VARUS OSTEOTOMY)
1394
What is a Slipped Capital (upper) Femoral Epiphysis (SCFE)?
Often atraumatic or associated with a minor injury
- Represents a type of instability of the proximal femoral growth plate.
- Common in overweight sedentary teenage boys
1395
What are the four main types of SCFE?
PRE-SLIP: wide epiphyseal line without slippage
ACUTE: slippage occurs suddenly, normally spontaneously
ACUTE ON CHRONIC: slippage occurs acutely where there is existing chronic slip
CHRONIC: steadily progressive slippage
1396
How is SCFE categorized?
STABLE (90%): patient can walk
| UNSTABLE (10%): patient unable to walk
1397
What are the mechanical risk factors of SCFE?
- Local trauma
| - Obesity
1398
What are the inflammatory risk factors of SCFE?
- Untreated septic arthritis
1399
What are the hypothyroidic risk factors of SCFE?
- hypopituitarism
- growth hormone deficiency
- pseudohypoparathyroidism
- vitamin D deficiency
1400
What are some other risk factors for SCFE?
- previous radiation of the pelvis
- chemotherapy
- renal osteodystrophy induced bone dysplasia
1401
What is the general presentation for SCFE?
- pain in the hip, groin, thigh or knee during walking
- Pain is accentuated by running, jumping or pivoting activities
- pre-slip: slight discomfort
1402
What is the presentation of acute SCFE?
- severe pain such that child is unable to walk or stand
- limp: antalgic gait
- external rotation of the leg and trunk shift
- hip motion is limited especially internal rotation and abduction due to pain
1403
What is the presentation of acute-on-chronic SCFE?
- pain
- limp
- altered gait occurring for several months, suddenly becoming very painful
1404
What is the presentation of chronic SCFE?
- mild symptoms with the child able to walk with altered gait
- knee pain
- external rotation of the leg during walking with reduced internal rotation
- mild-to-moderate shortening of the affected leg
- atrophy of the thigh muscle may be noted
1405
What investigations are done for SCFE?
- AP
| - frog-leg lateral XR shows widening of epiphyseal line or displacement of the femoral head
1406
What is the management for SCFE?
- Patient should not be allowed to walk
- Provide analgesia and immediate orthopedic referral if diagnosis is suspected
- Patient should be scheduled for surgery immediately
- Surgical closure of the epiphysis, usually by inserting screws percutaneously
1407
What are the complications associated with SCFE?
- Chondrolysis
| - Avascular necrosis of epiphysis
1408
What is transient synovitis/Irritable hip?
Commonest cause of limp in young children usually affecting boys 2-8 years.
1409
What are the features of transient synovitis?
- often preceded by URTI
- sudden onset of limp
- no pain at rest
- acute onset hip/ and or knee pain- refusal to weight bear
- limited abduction, extension and internal rotation of the hip
- lack of systemic signs of infection
- normal XR and bloods (may have slightly raised ESR)
1410
What is the management for transient synovitis?
- lasts for 2 weeks
- rest and oral analgesia
- observation
- no evidence of long term complications
- important to rule out septic arthritis
SAFTEY NET PARENTS
1411
What is Osgood-Schlatter Disease?
Multiple small avulsion fractures from contraction of the quadriceps muscle at their insertion into proximal tibial apophysis
More common in boys
1412
What is the presentation of Osgood-Schlatter Disease?
- Gradual onset of pain and swelling below the knee
- Relieved by rest
- Tenderness and swelling at the tibial tuberosity
- Pain is provoked by knee extension against resistance or by hyperflexing the knee
1413
What is the management for Osgood-Schlatter Disease?
```
RICE
Rest for 48 hours
Ice 20mins per time, 4-8 times a day
Compression (to reduce swelling)
Elevation (6-10 inches) above the heart
```
simple analgesia
1414
What is the definition of malnutrition?
Being in a nutrient deficiency state of protein, energy or micronutrients. Malnutrition is both a cause and consequence of ill health
1415
What are the causes of malnutrition?
- diets low in protein, energy or specific nutrients
- strict fad or veggie diets
- diseases causing malabsorption (coeliac disease, CF, Crohn's disease, GORD, chronic infection)
- Eating disorders
1416
What is the presentation for malnutrition?
- Bilateral pitting oedema in feet and legs
- Visible severe wasting
- weight for height> 3 SD below the median
1417
How does protein energy malnutrition present?
- Poor weight gain
- Slowed linear growth
- Behavioural changes - irritability, apathy, anxiety, attention deficit.
- Classically apathetic and quiet when lying in bed
- Cry when picked up with a typical monotonous bleat or loud groan
1418
What are the three clinical syndromes associated with malnutrition?
1) MARASMUS
2) KWASHIORKOR
3) NUTRITIONAL DWAFISM
1419
What are the features of marasmus?
- Obvious loss of weight with reduction in muscle mass especially from limb girdles
- SC fat virtually absent
- Thin, atrophic skin lies in folds
- Pinched face has appearance of old man or monkey
- Alopecia and brittle hair
- Sometimes appearance of lanugo hair
- Height is well preserved compared to weight; wasted appearance; muscle atrophy, listless diarrhoea and constipation
1420
What are the features of Kwashiorkor?
- Manifests around 1-2 years with changing hair colour to red, grey or blonde
- Moon faces, swollen abdomen, hepatomegaly, pitting oedema
- Dry, dark skin which splits where stretched over pressure areas to reveal pale areas
- Growth retardation, diarrhoea, apathy and anorexia
1421
What are the features of nutritional dwarfism?
- Patient is small for age
| - Face shape may be affected by size of teeth
1422
What should be included in the assessment of nutritional status?
- Recent weight loss .10% over 3 months?
- Falling across 2 centile lines or below the 3rd centile line
- BMI
- Mid-arm circumference divided by head circumference: MALNUTRITION <0.31
- Upper arm circumference <110mm
1423
What investigations should be done in suspected malnutrition?
- BLOOD GLUCOSE
- FBC +FILM
- URINE MC+S
- STOOL OC+P
- SERUM ALBUMIN
- HIV
- U+Es
- iron, folate, b12
- Pre-albumin, transferrin, retinol-binding protein
- TFTs
- Coeliac serology
- Calcium, phosphate, zinc
- Vitamin levels
1424
What is the management for malnutrition?
- Correct shock, dehydration and electrolytes
- Avoid refeeding syndrome
- Empirical antibiotics for 7 days as commonly infection occurs as well
- Aim for weight gain of 10-15g/day
- Child protection/social services if neglect is suspected
1425
What can result from refeeding syndrome?
- fluid balance abnormalities
- abnormal glucose metabolism
- hypophosphataemia
- hypomagnesaemia
- hypokalaemia
- thiamine deficiency
1426
What is Ricketts?
- Skeleton growth disorder due to inadequate mineralization of bone as it is laid down at the epiphyseal growth plates
1427
What are the common causes of Ricketts?
- MALNUTRITION
- CALCIUM DEFICIENCY
- VITAMIN D DEFICIENCY (from inadequate sun exposure and exclusive breastfeeding from 6-12months)
1428
What is the pathophysiology of Ricketts?
- Deficiency of Ca, Phosphorus or vitamin D, interferes with bone maturation, leading to a build up of osteoid tissue
- Thickening of the metaphysis where active growth is most rapid is seen at the wrists, ankles and costochondral junctions (rickety rosary)
- Toddlers develop bow legs, older children become knock-kneed
1429
What is the presentation of Ricketts?
- Softening of the skull and frontal bossing
- Delayed closure of the fontanelles
- Tender swollen joints
- Enlargement of the ends of ribs
- Bowing of legs (gene varum)
- Delayed walking or waddling gait
- Impaired growth
- Miserable and irritable
- May present with fractures in severe cases
- Dental deformities
- Symptoms of hypocalcaemia
1430
What are the differential diagnoses for Ricketts?
- Osteoporosis
1431
What are the investigations for Ricketts?
- U+Es and bone profile
- LFTs
- Parathyroid hormone
- Hypocalcaemia, hypomagesaemia and hypophosphataemia
- Elevation of plasma PTH typical
- FBC: anaemia suggests possible malabsorption
- Urine microscopy- CKD?
- Vitamin: <25nmol
- Coeliacs/CF causing malabsorption?
1432
What imaging investigations should be done for Ricketts?
- Wrist XR (CHAMPAGNE GLASS WRIST)
- Low bone density on DEXA scanning
- MR scanning helps to evaluate the soft tissues for ligament rupture
- CT: pathological fractures
- Iliac bone biopsy (rare)
1433
When should there be management for Ricketts?
Treat if:
- serum 25-hydroxyvitamin D levels are <25nmol/L
- if levels are 25-50nmol/L: seek advice on preventing of deficiency
- if >50nmol/L: consider other cause
1434
What is the management for Ricketts?
- Refer to paediatrician and dietician if clinical features of Ricketts
- Vitamin D supplements and exposure to sunlight
- - (<1 year old: 5mcg daily)
- - (>1 year old: 10mcg daily)
- Oral calciferol in the form of either ergocalciferol or colecalciferol
- Aged 1-6 months 3000IU/daily for 8-12 weeks
- Aged 6months- 12 years 6000 IU/daily for 8-12 weeks
- Aged 12-18 years 10,000 IU/daily for 8-12 weeks
1435
What are the main forms of genetic testing in children?
1. KAROTYPING
2. FISH (fluorescent in situ hybridization)
3. DNA ANALYSIS
4. GUTHRIE TESTS
5. DIAGNOSTIC TESTING
6. CARRIER TESTING
7. PRENATAL TESTING
8. PREIMPLANTATION TESTING
9. PREDICTIVE TESTING
1436
What does the Guthrie test test for?
- Sickle cell disease
- Cystic fibrosis
- Congenital hypothyroidism
- Inherited metabolic diseases
1437
What are the features of autosomal dominant inheritance?
- Parents affected
- Females and males affected
- female and male transmission
- 50% recurrence risk
- high new mutation rates
- variable penetrance in some conditions
1438
What are some examples of autosomal dominant conditions?
- ACHONDROPLASIA
- HUNTINGTONS DISEASE
- MARFAN SYNDROME
- TUBEROUS SCLEROSIS
- MYOTONIC DYSTROPHY
- VON WILLEBRAND DISEASE
1439
What are the features of autosomal recessive inheritance?
- Parents not affected
- Parent heterozygote carriers
- 1 in 4 risk of condition if carrier parents
- 2 in 3 risk of unaffected sibling being carrier
- more common in consanguinity
1440
What are some examples of autosomal recessive conditions?
- CF
- PKU
- Sickle cell disease
- Thalassaemia
- CAH
1441
What are the features of X linked recessive inheritance?
- Affects males
- Carrier female usually unaffected
- 50% of boys of carrier mother affected
- 50% of daughters of carrier mothers are carriers
- 100% of daughters of affected men are carriers
- No transmission of father to son
- New mutations are uncommon
1442
What are some examples of X linked recessive conditions?
- Fragile X
- DMD
- Haemophilia A/B
- G6PD deficiency
- Red green colour blindness
1443
What is Downs Syndrome?
Commonest congenital anomaly associated with developmental delay, where the underlying abnormality os trisomy of chromosome 21. This is usually of maternal origin and the incidence increases with maternal age.
1444
What are the three types of Down's syndrome?
1) NON-DYSJUNCTION at meiosis (95%)
2) ROBERTSONIAN TRANSLOCATION (4%)
3) MOSAICISM (1%)
1445
What are the features of non-dysfunction down's syndrome?
- Occurs at meiosis
- extra maternal chromosome
- karyotype: 47XX+21 OR 47XY+21
- Increased incidence of trisomy 21 secondary to non-dysjunction with increasing maternal age (>35), independent of paternal age
1446
What are the features of Robertsonian translocation?
- Chromosome 21 usually translocated onto chromosome 14
1447
What are the features of mosaicism?
- Some cells normal, some trisomy 21
| - This is due to non-dysjunction during mitosis after fertilization- usually less severely affected
1448
What is the general presentation of down's syndrome?
- Hyperflexibility
- Muscular hypotonia
- Transient myelopysplasia (increase in the number of circulating blasts that have acquired mutations) of the newborn
1449
What are the head dysmorphic features associated with Down's syndrome?
- Brachycephaly
- Oblique palpebral fissures
- Epicanthic folds
- Ring of iris speckles- Brushfield's spots
- Ears set low, folded or stentotic meatus
- Flat nasal bridge
1450
What are the mouth features of Down's syndrome?
- Protruding tongue (small narrow palate)
| - High arched palate
1451
What are the neck features of Down's syndrome?
- Loose skin on nape of neck
1452
What are the hand features of Down's syndrome?
- Single palmar crease
- Shot little finger
- in-curved little finger
- short broad hands
1453
What are the feet features of Down's syndrome?
- Large sandal gap
1454
What medical problems are associated with Down's syndrome?
- Congenital heart defect (1/3)- AVSD
- Duodenal atresia (1/3)- DOUBLE BUBBLE* appearance on XRAY
* two air filled bubbles are seen in the abdomen, representing two discontinguous loops of bowel in a proximal, or high small bowel obstruction
1455
What are the investigations for Down's syndrome?
- Antenatal screening
- Confirmation of diagnosis: prenatal examination of fetal cells from amniocentesis or chorionic villus sampling.
- Postnatal chromosomal analysis
- Screening for complications
1456
What is the conservative management for Down's syndrome?
- MDT approach: parental education and support, genetic counselling, IQ testing with appropriate educational input
- Can often be in mainstream school with extra input
- Regular reviews will be needed to assess feeding, bowel, behavior, vision, hearing and any other specific condition
1457
What is the medical management for Down's syndrome?
- Antibiotics in recurrent respiratory infections, thyroid hormone for hypothyroidism
1458
What is the surgical management for Down's syndrome?
- Correction of the congenital heart defects, esophageal/duodenal atresia
1459
What are the complications associated with Down's syndrome?
- Congenital heart defects
- Duodenal/oesophageal atresia
- Secondary otitis media
- Hearing impairment
- Strabismus, cataracts and myopia
- Hypothyroidism
- Atlantoaxial instability
- Leukaemia
- Early onset Alzheimers
- Delayed motor milestones
- Moderate to severe learning difficulties
- Small stature
- Increased susceptibility to infections
- Epilepsy
1460
What is strabismus (Squint)?
Defect leading to one eye varying in direction of gaze.
| - Can lead to amblyopia in childhood if left untreated, which is diminished acuity of central vision.
1461
How can squints be classified?
- CONGENITAL or ACQUIRED
- RIGHT, LEFT or ALTERNATING
- PERMANENT OR INTERMITTENT
- MANIFEST or LATENT
- CONCOMITANT (non-paralytic) or INCOMITANT (paralytic)
- PRIMARY, SECONDARY OR CONSECUTIVE
- SITUATIONAL
1462
What are the three main types of squint?
1) ESOTROPIA/CONVERGENT
- inward turning squint
- caused by accommodation and often seen in patients with hyperopic refractive error
2) EXOTROPIA/DIVERGENT
- outward squint
- begins intermittently with tiredness and progresses
- cause is convergence insufficiency that responds well to orthoptic exercises
- struggle with reading as one eye deviates outwards
3) HYPER/HYPOTROPIA
- deviated vertically
- far less common
- develops after childhood and incomitant
1463
What are the risk factors for non-paralytic squint?
- Family history of strabismus or ambylopia
- Prematurity
- Neonatal jaundice
- Encephalitis
- Meningitis
- Cerebral palsy
- Craniofacial abnormalities
- Down's and Turner's syndrome
- Developmental delay
- Fetal alcohol syndrome
- Febrile illness
1464
What are the causes of non-paralytic squint?
- Anisometropia: dissimilar refractive errors in both eyes causes the relative blurring of retinal images.
- Opacification of the cornea or lens (cataract) can blur images
- Abnormalities of the retina: causes incorrect transmission of images
1465
What are the clinical features of non-paralytic squint?
- parental concern
- intermittently close one eye- especially when out in sunlight
- Motor skills may be reduced
- Compensatory head tilt or chin lift
1466
What are the causes of paralytic squint?
- Acquired through damage to the extra ocular muscles or their nerves.
- III, IV, VI cranial nerves are involved. Myopathies, unlike neuropathies- are often bilateral
1467
What are the three main methods of examination for squint?
1) Gross inspection
2) Light reflex tests
3) Cover tests
1468
What is Hirschberg's test?
Rough estimate of the degree of strabismus.
- hold a pen torch arm's length away from patient and shine in from of their eyes
- ask patient to look at light
- observe where the reflection of the pen torch lies with respect to the cornea
- if it lies in the INNER MARGIN of the pupil- outward deviation
- if it lies at the OUTER MARIGN- inward deviation
1469
Wha is the cover/uncover test?
- An object to focus on is held in front of the patient
- One eye is completely occluded for several seconds and the uncovered eye is observed for movement as it focuses on the object
- This eye is then covered and the other eye is observed for movement
- Movement of the eye outward suggests esotropia and vice versa for exotropia
1470
What is the alternate cover test?
Similar tot he cover test but:
| - occluder is rapidly switched from one eye to the other
1471
What are the investigations for squint?
- Orthoptic assessment
- Assessment of motility, accommodation, fixation, binocularity, stereopsis and refraction
- If the visual acuity is subnormal, investigation is needed
1472
What is the management for non-paralytic squint?
- glasses to correct refractive error
- Correction of amblyopia
- - patching the child's good eye
- Occasionally, cycloplegic drops may also be used in the good eye
1473
What is the management for paralytic squint?
- identify and treat the underlying causes
- correction of residual squint/double vision, usually with prisms in glasses
- surgical correction is rarely indicated
1474
What is autism spectrum disorder?
- Inability to relate to others
- Spectrum of difficulty from severe learning disability to Aspergers where there is normal intelligence/
- More common in boys
- Chromosome 7q is very important
1475
What is the triad of impairment in autism spectrum disorder?
1) SOCIAL INTERACTION
2) IMAGINATIVE THOUGHT
3) COMMUNICATION
1476
What is the presentation of autism spectrum disorder?
Usually manifests between 2 and 4 years when language and social skills normally rapidly expand
- SPEECH DELAY is common first concern
- LACK OF EYE CONTACT
- LACK OF SOCIAL SMILE, IMITATION, RESPONSE TO NAME
- LACK OF INTEREST IN OTHERS
- LACK OF EMOTIONAL EXPRESSION
- FEW DIRECTED VOCALISATION
- ABSENCE OF JOINT ATTENTION SKILLS (pointing to show, referencing others/events)
- FEW REQUESTING BEHAVIOURS
- FEW SOCIAL GESTURES
- REDUCED PRETEND PLAY
- REGRESSION
1477
What are the speech and language disorders associated with autism spectrum?
- delayed development may severe
- formal pedantic language
- monotonous voice
- impaired comprehension with over-literal interpretation of speech
- echoes questions, repeats instructions, refers to self as you
1478
What behavioral problems are associated with autism disorder?
- imposition of routines with ritualistic and repetitive behaviour on self and others
- violent temper tantrums if disrupted
- unusual stereotypical movements such as hand flapping and tiptoe gait
- concrete play
- poverty of imagination in play and general activities
- peculiar interests and repetitive adherence
- restriction in behaviour repertoire
1479
What are the co-morbidities associated with autism spectrum disorder?
- general learning and attention difficulties
| - seizures
1480
What investigations can be done for autism spectrum disorder?
- Paediatric neurologists, developmental and behavioural pediatricians, child psychiatrists
- Examination of physical status particular attention to neurological and dysmorphic features
- Karyotyping and fragile X DNA analysis
- Hearing and sight examination
- Investigations to rule out recognised causes of ASD
1481
When is diagnosis of ASD made?
when an individual shows 6 or more symptoms across the THREE CORE AREAS:
- Communication
- Social impairment
- Impairment of interest, activities and/or behaviors
1482
What are the available screening questionnaires for autism?
- CHAT (CHecklist for Autism in Toddlers)
- Pervasive developmental disorder screening test (PDDST)
- Screening Tool for Autism in Two year olds (STAT)
- Social Communication Questionnaire (SCQ)
1483
What is the management of autism spectrum disorder?
- TEACCH method
- visual augmentation
- SALT
- social skill group
- occupational therapy
- supportive groups for parents
1484
What is the medical management for autism spectrum disorder?
If significant aggression: RISPERIDONE
| ADHD: METHYPHENIDATE
1485
What is the definition of childhood blindness?
- best corrected visual acuity of less than 3/60 in the better eye or a field of vision less than 10 degrees
1486
What are the causes of childhood blindness?
- Vitamin A deficiency
- Measles
- Opthalmia neonatorum
- infective corneal ulcers
1487
What is the primary prevention for vitamin A deficient blindness?
- Vitamin A supplementation
| - Nutrition education
1488
What is the primary prevention for blindness caused by measles?
IMMUNIZATION
1489
What is the primary prevention for blindness caused by ophthalmia neonatorum?
- cleaning eyes of newborn at birth
| - povidone iodine prophylaxis
1490
What is the definition of deafness?
- Partial or complete loss of hearing
1491
How is hearing loss classified (decibels hearing loss)?
MILD: 25-39 dB HL: cannot hear whispers
MODERATE: 40-69 dB HL: cannot hear conversational speech
SEVERE: 70-94 dB HL: cannot hear shouting
PROFOUND: >95 dB HL: cannot hear sounds that would be painful for a person with normal hearing
1492
What are the two types of hearing loss?
1) CONDUCTIVE HEARING LOSS (almost all glue ear)
| 2) SENSORINEURAL HEARING LOSS
1493
What are the causes of hearing loss?
1) GENETIC: Turner's, Kleinfelters
2) INTRAUTERINE: TORCH, HIV, maternal drugs/alcohol.toxins, streptomycin
3) PERINATAL: prematurity, low birth weight, birth asphyxia, severe hyperbilirubinaemia, sepsis
4) POSTNATAL: childhood infections, meningitis, encephalitis
5) UNKNOWN (20-30%)
1494
What is the presentation of hearing loss?
- Congenital/ perinatally profound sensorineural hearing loss shows at 6-9 months
- Lesser degrees may present with:
- minor speech impediments
- language delay
- behaviour problems
- problems at school
1495
Wha are the red flag symptoms associated with hearing loss?
- acute onset
| - unilateral sensorineural deafness
1496
What investigations should be done in suspected hearing loss?
- auditory brainstem response
- otoacoustic emissions and audiometry
- tympanometry
- Rinnes Webers
- MRI/CT scanning
- Karyotyping
1497
What is the management for hearing loss in children?
- Family support
- Grommets for glue ear
- Auditory-oral approaches
- hearing aids to amplify sound
- radio aids
- cochlear implants
- lip reading/speech reading
- BSL
- finger spelling
1498
What is the definition of significant developmental delay?
performance 2 or more SD below the mean
1499
How is the extent of delay classified?
MILD: functional age is <33% below chronological age
MODERATE: functional age is 34-66% below chronological age
SEVERE: functional age is >66% below chronological age
1500
what are the PRENATAL causes of developmental delay?
- Genetic: chromosome/DNA disorders
- Vascular: occlusions, haemmorhage
- Metabolic: hypothyroidism, PKU
- Teratogenic: alcohol/drug abuse
- Congenital infection: TORCH
- Neurocutaneous syndromes: tuberous sclerosis
1501
What are the PERINATAL causes of developmental delay?
- Extreme prematurity: IVH
- Birth asphyxia: HIE
- Metabolic: Symptomatic hypoglycemia, hyperbilirubinaemia
1502
What are the POSTNATAL causes of developmental delay?
- Infection: meningitis, encephalitis
- Anoxia: suffocation, near drowning
- Trauma: head injury
- Metabolic: hypoglycaemia, inborn errors of metabolism
- Vascular: stroke
1503
What are the red flag symptoms of developmental delay?
- by 3-4 months does not respond to loud noises or babble
- by 7 months no responding to sound
- by 12 months- not using single words like mama
- by 2 yers cannot speak 15 words, does not use two word phrases
1504
What is Fragile X?
Most common cause of learning disability in boys
| - Full expansion in the CCG triplet in the FRAXA gene on chromosome Xq27.3
1505
What is the presentation for Fragile X?
- borad forehead
- elongated face
- strabismus
- large prominent ars
- highly arched palate
- hyperextensible joints
- pectus excavatum
- mitral valve prolapse
- enlarged testicles
- hypotonia
- flat feet
1506
What is the management for Fragile X?
- SALT
- Special needs education and behavioural therapy
- ADHD: dextrofetamine and methylphenidate
- aripiprazole for mood stabilization and anticonvulsants for seizures
- genetic counseling
1507
What are the main types of abuse?
- Physical abuse
- Emotional abuse
- Sexual abuse
- Neglect
- Fabricated or induced illness (FII)
1508
What is the screening timeline in the UK?
1) MATERNAL BLOOD TEST (0-10wks)
2) DOWN SYNDROME (11-13wks)
3) CONGENITAL ANOMALY SCREEN (18-20wks)
4) NEWBORN HEARING SCREEN (birth)
5) NEWBORN PHYSICAL EXAMINATION (72hrs and 6 wks)
6) NEWBORN BLOOD SCREEN (day 5-8)
7) SCHOOL SCREENING (at entry, obesity at age 11)
1509
What are the four Cs of consent in adolescents?
THE 4 C's
- Comprehend
- Consider
- Choose
- Consequences
1510
MAINTENANCE FLUIDS
100ml/kg: 1st 10kg
50ml/kg: 2nd 10kg
20ml/kg: every additional kg
0.9% SALINE + 5% DEXTROSE
1511
BOLUS FLUID
20ml/kg
0.9% SALINE
REMEMBER TO CHECK MAXIMUM
1512
FLUID DEFICIT
% DEHYDRATION X WEIGHT(KG) X 10
1513
What is the general rule for analgesia?
1. FIRST STEP- mild pain
- <3 months= paracetamol
- >3 months= paracetamol and ibuprofen
2. SECOND STEP- moderate to severe pain
- Morphine
- Regular intervals- breakthrough doses
