PAEDIATRICS Flashcards
What is the etiology of anaphylaxis?
- IgE mediated type 1 allergic reaction
- Triggers histamine release from mast cells and eosinophils
- Causes capillary leakage, edema, shock and asphyxia
What are the initial symptoms of anaphylaxis?
- Pruritis (itchy skin)
- erythema (redness)
- urticaria (hives, red raised rash)
- rhinitis
- conjunctivitis
- angiooedema
What are the general symptoms of anaphylaxis?
- Palpitations
- tachycardia
- nausea and vomiting
- abdominal pain
- collapse
- loss of consciousness
What are the airway symptoms of anaphylaxis?
- itching of the palate of external auditory meatus
- dyspnoea
- bronchospasm (wheezing) –> edema and acute stridor
- cyanosis
- circulatory collapse (rare)- reduced CRT hypotension, tachycardia
What is the initial management for anaphylaxis (before ABCDE)
- lay the patient flat
- raise legs with care
What is A in anaphylaxis?
Look for:
- obstruction
- swelling
- signs of allergen
Manage with:
- call for help
- intubation
- high flow o2, 15L through non-rebreathe mask
What is B in anaphylaxis?
Look for signs of respiratory distress:
- tracheal tug
- nasal flaring
- intercostal recession
- headbobbing
What is C in anaphylaxis?
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
What is D in anaphylaxis?
Assess consciousness level
What is E in anaphylaxis?
Assess glucose level and do system review
What is the medical management for anaphylaxis?
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
What are the values in monitoring after anaphylaxis?
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 90mmHgWhat to do if there is no improvement in anaphylaxis symptoms following initial treatment and monitoring?
- repeat fluid challenge
- measure serum mast cell tryptase
- admit patient under paeds team
- educate parents on epi pens and BLS on discharge
What are the causes of cardio-respiratory arrest in children?
- 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
What is the management for cardio-respiratory arrest in children?
- 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
What is the airway recognition in a collapsed child?
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
What is the airway response in a collapsed child?
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
What is the breathing recognition in a collapsed child?
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
What is the breathing response in a collapsed child?
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
What is the circulatory recognition in a collapsed child?
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
What is the circulatory response in a collapsed child?
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
What is the disability recognition in a collapsed child?
- AVPU
- Pupils size and reaction to light
- posture
- blood glucose
- evidence of seizure activity?
What is the disability response in a collapsed child?
- Protect the airway
- Endotracheal tube if GCS <8
- Recovery position if airway not protected
- Give glucose if hypoglycemia
- Treat seizure activity with benzodiazepines
What is the exposure recognition in a collapsed child?
- 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
What is the exposure response in a collapsed child?
- Senior/ expert medical advice?
- Management plan
- Documentation
- Communication (SBARR)
- Organise transfer to HDU/ICU
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
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)
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.
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
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
What are the four main areas for developmental assessment?
- GROSS MOTOR
- FINE MOTOR
- SPEECH AND LANGUAGE
- SOCIAL
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
How is fine motor development assessed?
- Assess dexterity and cognition (important to differentiate between the two)
- Observe use of hands (open or closed grasp)
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
How is social development assessed?
Interaction and everyday skills
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
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
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
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
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
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
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
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
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
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
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
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
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
What are developmental warning signs at any age?
- MATERNAL CONCERN
- DISCORDANCE IN DIFFERENT DEVELOPMENTAL AREAS
- REGRESSION
- PERSISTING PRIMITIVE REFLEXES
What are developmental warning signs at 10 weeks?
No smile
What are developmental warning signs at 6 months?
- Persistent primitive reflexes and squint
- Hand preference
- Little interest in people, toys
What are developmental warning signs at 10-12 months?
- No sitting
- No double syllable babble
- No pincer grip
What are developmental warning signs at 18 months?
- Not walking
- <6 words
- Mouthing and drooling
What are developmental warning signs at 2.5 years?
No 2-3 word sentences
What are developmental warning signs at 4 years?
Unintelligible speech
What is colostrum?
The thin watery milk produced in first few days (high IgG)
What is the Let-Down reflex in breastfeeding?
- Babies suckling stimulates nerve endings in areola
- Neural reflex (message) is passed to pituitary gland via the hypothalamus
- Oxytocin contracts the muscle wall of alveoli to release milk during feeding (posterior pituitary)
- Prolactin stimulates the alveoli to produce breastmilk for future feedings (anterior pituitary)
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
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 (?)
When is breastfeeding contraindicated?
- HIV positive
- TB
- TETRACYCLINES (teeth stain)
- ANTIMETABOLITES
- OPIATES
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
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
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)
What three elements are assessed on centile charts?
- Weight
- Height
- Head circumference
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)
What is the definition of a neonate?
(TERM)- BIRTH - 28 DAYS
(PRETERM)- BIRTH- 44 POST MENSTRUAL WEEKS
What is neonatal jaundice?
Most commonly due to physiological causes and reflects slow conjugation
What are some risk factors for neonatal jaundice?
- PRETERM
- LOW WEIGHT
- FAMILY HISTORY
- MATERNAL DM
- MALE
- EAST ASIAN
What are the causes of physiological neonatal jaundice?
- Immature liver function
- Increased erythrocyte breakdown
- baby is generally well
- Bilirubin levels are <200umol/L
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
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
What are the causes of CONJUGATED neonatal jaundice?
- Neonatal hepatitis
- Bile duct obstruction
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)
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
What particular investigations should be done in early neonatal jaundice?
- SEPSIS, LP, septic screen
- Rhesus status
- Direct Coombs test
- Blood group
- U+Es
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.
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
What are the risk factors for kernicterus?
- high serum bilirubin
- preterm birth
- acidosis
- hypoxia
How is kernicterus treated?
PHOTOTHERAPY
EXCHANGE TRANSFUSION
What is biliary atresia?
- Conjugated hyperbilirubinaemia
- Absence of intra/extrahepatic bile ducts
- Develops over a period of weeks
- Stools become CLAY COLOURED
What are clay colored stools a sign of?
Biliary atresia
How is biliary atresia treated?
if detected within the first 6 wks:
- KASAI PROCEDURE (hepatoporto-enterostomy)
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
What is birth asphyxia/ hypoxic ischemic encephalopathy?
A consequence of intrapartum foetal hypoxia
How is birth asphyxia diagnosed?
- pH <7.05 (acidotic)
- depression of APGAR scores (0-5 at 10mins)
- Delay in establishing spontaneous respiration (>10mins)
How is mild hypoxic ischemic encephalopathy classified?
- irritable
- high pitch cry
- poor feeding
- normal prognosis
How is moderate hypoxic ischemic encephalopathy classified?
- Lethargic
- hypotonic
- poor feeding
- fits
- 40% risk of cerebral palsy
How is severe hypoxic ischemic encephalopathy classified?
- diminished consciousness
- no movement
- multiple seizures
- organ failure
- 90% risk of cerebral palsy
- 30% mortality
What is the management for birth asphyxia/ HIE?
- rapid resuscitation
- avoid cerebral oedema
- treat convulsions
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
TO KNOW: Pigmented naevi
- usually appear ay 2 years of age
- flat or slightly elevated
- very low malignancy risk unless large congenital naevi
TO KNOW: Cafe au lait spots
- uniformly pigmented
- sharply demarcated, macular lesions
- vary greatly in size
- often harmless but associated with neurofibromatosis
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
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)
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.
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
What is a cephalhaematoma?
- Subperiosteal bleeding
- swelling and amount of blood loss is limited by periosteum attached to margins of skull.
What is a possible complication of cephalhaematoma?
It can make jaundice worse as blood is reabsorbed.
What are risk factors for cephalhaematoma?
- forceps delivery
- large baby
- first pregnancy
- difficult and prolonged labours
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
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.
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.
How does hemolytic disease of the newborn present?
- jaundice
- pallor
- hepatosplenomegaly
- hydrops fetalis (accumulation of fluid in 2 or more compartments)
- polyhydramnios
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
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.
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
What are the possible complications of hemolytic disease?
Kernicterus
What is the prevention for hemolytic disease?
Anti- D immunoglobulin should be given to ALL rhesus negative women who have NOT been sensitized.
What is the definition of prematurity?
<37 weeks from the day of last menstruation.
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
What are the complications in prematurity for the brain?
- intraventricular haemorrhage
- post hemorrhagic hydrocephalus
- periventricular leucomalacia
- increased risk of cerebral palsy
What are the complications in prematurity for the respiratory system?
- RDS
- surfactant deficiency
- apnoea and bradycardia
- pneumothorax
- chronic lung disease
What are the complications in prematurity for the cardiovascular system?
- hypotension
- patent ductus arteriosus
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
What are the metabolic complications in prematurity?
- hypoglycemia
- hypocalcaemia
- electrolyte imbalance
- osteopenia of prematurity
What are the complications in prematurity for the blood?
- anaemia of prematurity
- neonatal jaundice
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
What are the GI complications in prematurity?
- Necrotizing enterocolitis
- GOR
- inguinal hernias (with high risk of strangulation)
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
What are the risk factors for prematurity?
- young maternal age
- multiple pregnancy
- infection
- maternal illness
- cervical incompetence
- antepartum haemorrhage
- smoking
- alcohol
- infection
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
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
What can be used to establish cardiac output?
- IV ADRENALINE
- BICARBONATE
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%
What is the value of therapeutic hypothermia?
cool to 33oC for 72 hours
may prevent secondary neuronal damage following moderate/severe asphyxia
What is IUGR?
A condition whereby the baby’s growth slows or stops in the uterus.
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
What are the differentials associated with IUGR?
- PLACENTAL INSUFFICIENCY: head sparing
- CHROMOSOMAL ABNORMALITY: uniform restriction
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.
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
What are the UADS criteria?
- Elevated ratio of femoral length to abdo circumference
- Elevated ratio of head circumference to AC
- Oligohydramnios (low amniotic fluid)
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.
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
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
What can prevent RDS?
ANTENATAL ADMINISTRATION OF CORTICOSTEROID FOR 48 HRS
What are the clinical features of respiratory distress syndrome?
- tachypnoea
- intercostal, subcostal and sternal recession
- cyanosis
- expiratory grunting
What are the investigations for RDS?
- CXR will show:
- AIR BRONCHIOGRAM
- GROUND GLASS APPEARANCE OF LUNG FIELDS
- BELL SHAPED THORAX
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.
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
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.
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.
Which neuromuscular problems are associated with (talipes) club foot?
- cerebral palsy
- spina bifida
- arthrogryposis
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
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
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
What vaccinations are given at 12 weeks?
- Diptheria, Tetanus, Pertussis (DTaP)
- Polio (IPV)
- Haemophilus influenzae type B (Hib)
- Hepatitis B (HepB)
- Rotavirus
What vaccinations are given at 16 weeks?
- Diptheria, Tetanus, Pertussis (DTaP)
- Polio (IPV)
- Haemophilus influenzae type B (Hib)
- Hepatitis B (HepB)
- Pneumococcal
- MenB
What vaccinations are given at one year old?
- Hib and MenC
- Pneumococcal
- MMR (measles, mumps and rubella)
- MenB
What vaccinations are given at eligible pediatric groups?
Influenza (both nostrils)
What vaccinations are given at 3yr4mths or soon after?
- Diphtheria, Tetanus, Pertussis (DTaP)
- Polio (IPV)
- MMR
What vaccination is given to girls aged 12/13 years?
- HPV (2 doses 6-24 months apart)
What vaccination is given to 14 year olds?
- Tetanus, diphtheria (Td)
- Polio (IPV)
- Meningococcal groups (MenACWY)
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
TO KNOW: BCG
- Protects against TB
- Given intradermally
- papule forms and often ulcerates
- heals over 6-8 weeks with a scar
TO KNOW: Tetanus
- In the case of a dirty wound, give tetanus immunoglobulin with booster if last vaccination was >10 years ago
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
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
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
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
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
Haemophilus influenzae B
- Main cause of meningitis before the vaccine was introduced
- Led to severe neurological sequelae e.g. deafness, cerebral palsy and epilepsy.
Pneumococcal disease
- Streptococcus pneumoniae which can produce septicemia, meningitis and pneumonia
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
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
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
Rubella
- Mild illness causing rash and fever
- Harmful to foetus in pregnancy
- Can cause congenital defects such as cataracts, deafness and congenital heart disease
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
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
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.
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)
What are the risk factors for meningitis?
- CSF shunts
- Spinal procedures
- Diabetes
- Crowding
What are the viral causes for meningitis?
- COXSACKIE (most common virus)
- ADENOVIRUS
- EBV
What are the main bacterial causes for meningitis?
- NEISSERIA MENINGITIDES
- STREPTOCOCCUS PNEUMONIA
- HAEMOPHILUS INFUENZA
- MYCOBACTERIUM TUBERCULOSIS
Which bacterial causes most commonly affect neonates?
- Group B strep (50%)
- E. coli (20%)
- Listeria (5-10%)
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)
Which bacterial causes most commonly affect adolescents and adults?
- Neisseria meningitidis
- Strep pneumoniae
What are the other causes of meningitis?
- Disease that is partially treated
- Fungal
- Parasites
- Kawasaki
- Mollaret’s (benign recurrent lymphocytic meningitis- chronic inflammation)
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
What are the clinical features of viral meningitis?
- often preceded by pharyngitis or GI upset
- Infection of mucous membrane to start with
- lymph node involvement
- initial viral illness symptoms
- fever, headache and neck stiffness
- head retraction is a late feature
- often indistinguishable from bacterial but may be milder
- enterovirus, parechiovirus, herpes
What are the clinical features of bacterial meningitis?
- Invasion of nasopharyngeal epithelium
- invades blood stream then meninges
- leaky vessels cerebral edema
- decreased blood flow
- drowsiness and vacant expression
- photophobia
- coma
- reduction in level of consciousness is unique to bacteria
- meningeal cry (high pitched)
- convulsions due to Invasion of nasopharyngeal epithelium
What are the clinical features of meningitis seen in neonates?
- fever no focus
- irritability
- seizures
- poor feeding
- respiratory distress
- coma
What are the clinical features of meningitis seen in infants?
- fever no focus
- nausea and vomiting
- cold peripheries
- lethargy
- unsettled
- refusing food
What are the clinical features of meningitis seen in adolescents?
- unwell
- headache with photophobia
- myalgia
- neck stiffness
- nausea and vomiting
What are clinical signs of raised ICP?
- papilloedema
- altered consciousness
- increased BP
- decreased pulse
- bulging fontanelle
- neck retraction
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
What are some differential diagnoses for meningitis?
- Septicaemia
- Raised ICP
- Menigismus- neck stiffness due to tonsillitis, otitis media, pneumonia, pyelonephritis
What are the appropriate investigations for meningitis?
Distinction between bacterial and viral meningitis can’t be made clinically, so if suspected:
- LUMBAR PUNCTURE
- PCR
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
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
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
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
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
Features of bacterial meningitis?
- PMNs outnumber monocytes
- papilloedema occurs in late disease
- acute onset
- high lactate
- low glucose in CSF
Features of tubercular meningitis?
- Insidious onset
- slight changes in CSF chemistry
- positive tuberculin
- low chloride
Features of fungal meningitis?
- insidious onset
- history of lung infection
- yeast cells in CSF
- slight changes in CSF chemistry
Features of syphilitic meningitis?
- insidious onset
- slight change in CSF chemistry
- positive RPR test
Features of parasitic meningitis?
- acute onset
- slight change in CSF chemistry
- presence of IgM in CSF (Trypanosoma cruz infection= Chagas’ disease, sleeping sickness)
Features of viral meningitis?
- acute onset
- slight change in CSF chemistry
- monocytes outnumber PMNs
Features of subarachnoid hemorrhage?
- red blood cells in CSF
Distinguishing meningitis from meningioma?
- X-ray for tumour presence
Distinguishing meningitis from meningismus?
- there would be a history of non-CNS viral disease (a non-infective state resembling meningitis)
Features of a brain abscess to distinguish from meningitis?
- PMNs may outnumber monocytes
- papilloedema occurs early in disease
- acute or insidious onset
- sterile CSF
What is the management for viral meningitis?
- self limiting, symptomatic treatment
- for HERPES SIMPLEX MENINGOENCEPHALITIS- ACICLOVIR
What is the management for bacterial meningitis in children >3 months?
- IV CEFTRIAXONE for 10-14 DAYS
- DEXAMETHASONE to reduce inflammation
What is the management for bacterial meningitis in infants <3 months?
- IV CEFOTAXIME
- AMOXICILLIN/AMPICILLIN
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
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:
- Frankly purulent CSF
- CSF white blood cell count greater than 1000/microlitre
- raised CSF white blood cell count with protein concentration greater than 1g/L
- bacteria on Gram stain
When should dexamethasone be omitted in meningitis?
- in TB
- MORE THAN 12 HOURS AFTER STARTING ANTIBIOTICS
- CHILDREN UNDER 3 MONTHS
What are the immediate complications of meningitis?
- septic shock
- DIC
- raised ICP
- seizures
- pericardial effusion
- cerebral oedema
- hemolytic anaemia
- subdural effusion
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
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.
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
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.
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
What are the early clinical signs of meningococcal septicemia?
- leg pain
- cold peripheries
- mottling
- dyspnoea
What are the late clinical signs of meningococcal septicemia?
- confusion
- seizures
- coma
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)
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.
In our of hospital management of meningococcal disease, where should the antibiotics be administered
- IM in children (quads)
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.
Is meningococcal disease notifiable?
YES
Inform Public Health England
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
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
When is ceftriaxone contraindicated in the treatment of meningococcal infection?
- premature babies
- babies with jaundice
- hypoalbuminaemia
- acidosis
- if calcium containing infusions are being administered
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.)
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
What are the early complications associated with meningococcal disease?
- DIC
- AKI
- adrenal haemorrhage
- circulatory collapse
- seizures
- raised ICP
- hydrocephalus
- cerebral venous thrombosis
What are the late complications associated with meningococcal disease?
- deafness
- skin scarring
- renal failure
- limb amputations
- gait problems
- cognitive deficit
- incontinence
- developmental delay
What is the prognosis/ follow up?
- 15% have long term neurological complications including deafness and developmental impairments.
- Offer a formal audiological assessment ASAP
- inform the GP, health visitor and school nurse about meningitis/meningococcal septicaemia to monitor to late onset complications.
- Children with recurrent episodes should be assessed by a specialist in infectious disease or immunology
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
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
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
Which conditions in the acutely unwell patient can present with purpura?
- Acute bacterial sepsis including invasive meningococcal disease (RAPID ONSET)
- Acute leukemia (ACUTE-SUBACUTE ONSET)
Which conditions in all other child patients can present with purpura?
- Non-accidental injury (VARIABLE ONSET)
- Idiopathic thrombocytopaenic purpura (ACUTE ONSET)
- Drugs (co-trimoxazole, quinine, carbamazepine, valproate) (VARIABLE ONSET)
- Congenital bleeding disorders (hemophilia and VW disease) (CHRONIC HISTORY)
- Acquired hemophilia (ACUTE-SUBACUTE ONSET)
- Non-leukaemic bone marrow failure (SUBACUTE)
- Vitamin deficiency. (SUBACUTE)
- Raised SVC pressure (ACUTE)
- Vasculitis (e.g. SLE, HSP, viral infection) (SUBACUTE-CHRONIC)
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
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.
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
What key flags in new purpura can indicate certain causes?
- A short acute illness = suspicion of sepsis
- Recent viral illness or immunization in a well child= ITP, HSP, general vasculitic viral rash
- Rash is often on the lower limbs and in crying/vomiting children around the head and neck = thrombocytopaenia
- Bruising on the trunk, ears, face which cannot be adequately explained = non accidental injury/ severe congenital bleeding disorder
- New drug has recently been started = drug related purpura
- History of recent blood transfusion = post-transfusion purpura
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
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.
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.
What is amyloid purpura?
Typical “pinch purpura” because of its appearance on the cheeks
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.
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
What is chicken pox?
Varicella Zoster virus infection with outbreaks during the winter/spring, mostly found in under 5s
It is usually self limiting
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
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
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
What is the pattern of rash development in chicken pox?
- PAPULES
- VESICLES
- PUSTULES (fever)
- SCABS
How is diagnosis of chicken pox made?
CLINICAL DIAGNOSIS
- but PCR can be used.
What is the management for chicken pox?
- Encourage fluid intake
- Paracetamol
- Antihistamines and emollients
- High dose steroids in the immunocompromised, but it new lesions after 8 days, give IV ACYCLOVIR
- In >12y.o., pregnancy or close contacts, give:
- -> 5-7 days oral acyclovir, 800mg 5 daily
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
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
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
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
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.
What are the three categories of conjunctivitis?
- Neonatal conjunctivitis
- Infective conjunctivitis
- Allergic conjunctivitis
What is neonatal conjunctivitis?
- Any conjunctivitis in the first 28 days of life
- Contamination from maternal GU tract.
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.
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
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.
What are the causes of infective conjunctivitis?
- Staphylococcus. aureus
- Staphylococcus. epidermidis
- Strep. pneumoniae
- Gonococcal
- Chlamydial
- Adenovirus
- HSV
- HZ
How is infective conjunctivitis treated?
- Stop contact lens wearing
- Chloramphenicol
- Fusidic acid
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
How is allergic conjunctivitis treated?
- Topical antihistamines
- Topical mast cell stabilizers (e.g. sodium cromoglicate)
How does conjunctivitis present?
- Discomfort
- Thick discharge
- Mild photophobia
- ‘Red eye’- uniforms engorgement of all the conjunctival blood vessels
- Yellow-white mucopurulent discharge and bilateral if bacterial
- Eyes difficult to open in the morning- glued together by discharge
- Pain (minimal)
- Vision is usually normal- smearing on waking is common
- Visual acuity- mild temporary blur secondary to discharge which can be wiped away
What should be considered in a history of conjunctivitis?
- Ask about contact lens use
- Time course- onset, duration (in chronic cases consider STDs)
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
What are the differentials associated with conjunctivitis?
- Blepharitis
- Uveitis
- Acute glaucoma
- Keratitis
- Scleritis
- Episcleritis
- Orbital cellulitis
- Ocular HSV
What are the complications associated with conjunctivitis?
- corneal ulceration
- otitis media
- pneumonia
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.
What are the causes of orbital cellulitis?
- extension of infection from periorbital structures, and from face, lacrimal sac and dental infection
- Extension of preseptal cellulitis, particularly in young children
- Direct inoculation of the orbit from trauma
- Haematogenous spread from distant bacteraemia
What organisms are most responsible for orbital cellulitis?
- Staphylococcus. aureus
- Strep. pneumoniae
- Strep. pyogenes
- Haemophilus influenzae
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
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).
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.
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
What is preseptal cellulitis?
Infection anterior to the orbital septum
What are the causes of preseptal cellulitis?
- A result of local skin trauma
- Due to spread from local infection (e.g. sinuses)
- Spread from distant infections such as from the upper respiratory tract
What organisms are most responsible for preseptal cellulitis?
- Staphylococcus. aureus
- Staphylococcus epidermidis
- Streptococci
- Anaerobes
How does preseptal cellulitis present?
- Acute onset of swelling, redness, warmth and tenderness of the eyelid
- Fever, malaise, irritability in children
- Ptosis
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).
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
What are the possible complications of preseptal cellulitis?
- Progression of infection into orbital cellulitis
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
How are food allergies classified?
- IMMUNOLOGICAL REACTIONS- both IgE (acute, often rapid onset) and non IgE mediated (delayed and non-acute reactions).
- NON-IMMUNOLOGICAL REACTIONS
What is the epidemiology of food allergy?
Though to affect 5 in 100 children
2 in 100 develop cow’s milk protein allergy
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?
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
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
What are the presentations common to both type of response to food allergy?
- Pruritis
- Erythema
- diarrhoea
- abdominal pain
Which are the food most commonly involved in food allergies?
- milk
- eggs
- fish and seafood
- peanuts
- sesame
- tree nuts
- soy beans
- wheat
- kiwi fruit
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
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
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
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
What is infectious mononucleosis?
ALSO KNOWN AS GLANDULAR FEVER
What is infectious mononucleosis caused by?
Epstein Barr Virus
If tonsillitis is present with infective mononucleosis, what are some differentials for this disease?
- Streptococcal infection
- Diptheria
- Leukaemia
- Lymphoma
- Toxoplasmosis and CMV
- Hepatitis
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
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
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.
What is Kawasaki disease?
An idiopathic and self limiting disease
Autoimmune mediated systemic vasculitis that affects small and medium sized arteries.
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)
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
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
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
What are some other signs of Kawasaki disease?
- CARDIOVASCULAR- pancarditis, aortic or mortal competence, tachycardia
- GI- hydrops of the gallbladder, jaundice, hepatomegaly, diarrhoea
- BLOOD- mild anaemia
- RENAL- sterile pyuria, mild proteinuria
- CNS- aseptic meningitis
- MSK- arthritis, arthralgia
- Others- anterior uveitis, BCG site, inflammation
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
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
What are the complications associated with Kawasaki disease?
- Coronary artery aneurysms
- Sudden cardiac death
- Pericarditis/myocarditis
- Valvular disease
- Cardiac dysrhythmia
- Heart failure
- Acute arthritis
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
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
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
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
What are the complications for measles?
- Bronchopneumonia
- Giant cell pneumonitis
- Cervical adenitis
- Otitis media
- Encephalitis
- Vitamin A deficiency and blindless
- Lymphopenia
- Miscarriage, low birth weight, preterm birth
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
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
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
What are the investigations for rubella?
- Serological and PCR testing
- FBC- low WBC, increased lymphocytes and thrombocytopenia
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
What are the complications in rubella?
- Rubella encephalopathy may occur 6 days after the rash
- Arthritis and arthralgia
- Thrombocytopenia
- Guillain- Barré syndrome/neuritis
- Panencephalitis
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
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
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
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
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
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
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
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
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
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
What is the main cause of childhood anaemia?
- Iron deficiency (due to rapid growth and diet)
Where is sickle cell disease more likely found?
Central African origin
Where is beta thalassemia more likely found?
- Mediterranean
- Middle Eastern
- Southeast Asian
What are the three main ways in which anaemia can be classified?
- Reduced red blood cell/ haemoglobin production
- Increased red blood cell destruction (haemolysis)
- Blood loss e.g. GI blood loss and heavy menstruation in girls
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)
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
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
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
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
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
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
What is the prescription of sodium ferrate in treating childhood anaemia?
5-11 years old:
- SODIUM FEREDETATE 190mg/5ml THREE TIMES DAILY
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
How long does it take for iron levels to return to normal?
- A month or two.
Brain tumors:
- 2nd commonest site for tumours in childhood
- 75% are infratentorial or midline
- The most common are ASTROCYTOMAS
- Most are idiopathic
What are the five main classifications of brain tumours in children?
- Gliomas
- PNETs (primitive neuroectodermal tumours)
- Congenital
- Pineal tumours
- Benign tumours
What are examples of gliomas found in children?
- Astrocytic tumour
- Oligodendroglioma
- Ependyoma
- Mixed glioma
- Ganglioma
- Choroid plexus tumour
What are examples of PNETs found in children?
- Medullo blastoma
- Pineoblastoma
What are examples of congenital tuners found in children?
- Teratoma
- Craniopharyngioma
What are examples of pineal tumours found in children?
- Germinoma
- Embryonal cell carcinoma
- Choriocarcinoma
- Pineocytoma
- Pineoblasotma
What are examples of benign tumours found in children but more common in adults?
- Meningioma
- Acoustic neuroma
- Pituitary tumour
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
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
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
What is the radiotherapy management for brain tumours in children?
- Low doses to localized areas
- Gamma knife and interstitial seeds
What is the chemotherapy management for brain tumours in children?
- Various combinations, but normally VINCRISTINE
- ETOPOSIDE, CYCLOPHOSPHAMIDE and 5-FLUOROURACIL are also used
What are the complications of brain tumours in children?
- Intellectual decline
- Growth hormone deficiency
- Secondary brain tumour if irradiated
- Cavernomas
What is the prognosis for brain tumours in children?
- Surgical mortality for craniotomy is 1%
- 1/3 of all cancer deaths
What is hemophilia A?
A bleeding disorder caused by deficiency of FACTOR VIII.
Type A is 5x more common than type B
What is hemophilia B?
A bleeding disorder caused by deficiency of FACTOR IX. It is LESS SEVERE and rarer than type A.
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.
What is the presentation of hemophilia A in severe disease?
- Neonatal bleeding
- Neonatal intracranial haemorrhage
- History of spontaneous bleeding into joints
- Spontaneous haemarthroses
- Haematuria
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
What is the presentation of hemophilia A in moderate disease?
- Bleeding following venepuncture
What is the presentation of hemophilia A in mild disease?
- Only bleed after major trauma/ surgery
What is the presentation of hemophilia B in severe disease?
- Spontaneous hemorrhages and haemarthrosis.
- Ventouse delivery may produce an enormous haematoma.
What is the presentation of hemophilia B in moderate disease?
- Suffer hemorrhage from minor trauma or surgery
- some times spontaneous haemarthrosis.
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
What are the physical signs of hemophilia B?
- Painful and swollen joints
- Bleeding into CNS –> neuro signs
- pallor
- dyspnoea
- tachycardia
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)
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
What is the management for acute episodes of bleeding in haemophilia?
- ABCDE
- Fresh Frozen Plasma (FFP), containing the deficient factor should be transfused
- Desmopressin
- Tranexamic acid (which binds to plasminogen- reduces the conversion of plasminogen to plasmin which breaks down fibrin).
What are the main complications of haemophilia?
- JOINT DISEASE
- Haemorrhage and associated complications (dependent on site)
How does platelet deficiency (vWF deficiency present)?
- Petechial haemorrhage
- Ecchymoses
How does clotting factor deficiency present?
- haematomas
- haemarthroses
What is Henoch Schönlein Purpura (HSP)?
An IgA mediated autoimmune hypersensitivity vasculitis, most common in
- 4-6 year olds
- females
What are the 4 organs affected in HSP?
- Skin
- Joints
- Gut
- Kidneys
What are the risk factors for HSP?
INFECTIONS
- Group A strep
- Myoplasma
- EBV
VACCINATIONS
ENVIRONMENTAL
- Allergens
- Pesticides
- Cold
- Insect bites
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%
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
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)
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
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
What is Hodgkin’s lymphoma?
Malignant proliferation of lymphocytes
- Affects males more than females
How is Hodgkin’s lymphoma classified?
FOUR SUBTYPES
- Nodular sclerosing HL (most common)
- Lymphocyte- rich HL
- Mixed cellularity HL
- Lymphocyte- depleted HL
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.
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
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%
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
What is the management for Hodgkin’s lymphoma?
RADIOTHERAPY : IA and IIA
CHEMOTHERAPY: for more advanced stages
What is the standard regime for chemotherapy treatment in Hodgkin’s lymphoma?
ABVD
- Adriamycin
- Bleomycin
- Vinblastine
- Dacarbazine
What are the complications of radiotherapy use in Hodgkin’s lymphoma?
- May increase solid tumor risk
- Lung fibrosis
- Hypothyroidism
- IHD
What are the complications chemotherapy use in Hodgkin’s lymphoma?
- Myelosuppression
- non-Hodgkin’s
- alopecia
- AML
- nausea
- infection
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
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
What is the pathogenesis of Non-Hodgkin’s lymphoma
- Malignant clonal expansion of lymphocytes which occurs at different stages of lymphocyte development
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
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%
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
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
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.
What are the four different types of leukemia?
- Acute Lymphoblastic Leukaema- ALL (78%)
- Acute Myeloid Leukaemia- AML (15%)
- Chronic Myeloid Leukemia- CML
- Chronic Lymphocytic Leukaemia- CLL
What are the risk factors for leukaemia?
- Caucasian
- Boys
- Influenza exposure
- Downs
- Blooms
- Ataxia telangiectasia
- Fanconi’s
- Radiation
What is the general presentation of leukaemia?
- Anaemia
- Thrombocytopaenia
- hepatosplenomegaly
- lymphadenopathy
- frequent illness
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
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
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
What is the general management for leukemia?
- Individual and family support groups
- No routine immunisations during therapy and for 6 months afterwards.
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.
What is the management for AML?
- Intensive chemotherapy
- Intense marrow suppression until haematopoietic recovery occurs
What is the management for CML?
- Myeloablative haematopoietic stem cell transplantation from fully matched related and unrelated donors
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
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
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
What is the prognosis for ALL?
- Overall cure rate of 80%
- Best for children aged 1-10 years
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.
What is the classification of sickle cell anaemia?
- HbSS- SICKLE CELL ANAEMIA: homozygote for the beta S globin with usually a severe or moderately severe phenotype
- HbS- BETA THALASSAEMIA: severe double heterozygote for HbS and beta thalassemia, and almost clinically indistinguishable from sickle cell anaemia
- HbSC DISEASE: double heterozygote for HbS and HbC with intermediate clinical severity.
- HbS- BETA+ THALASSAEMIA: mild to moderate severity, but variable in different ethnic groups
- HbS- HEREDITARY PERSISTENCE OF FETAL Hb: symptom free
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
How is sickle cell trait investigated?
The only abnormality in laboratory tests is presence of haemoglobin AS on electrophoresis.
How should a person with sickle cell trait be managed?
- adequate hydration
- avoidance of of fluid loss and severe heat to prevent complications.
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
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
What are the different forms of sickle cell crises?
- VASO-OCCLUSIVE CRISES
- APLASTIC CRISES
- SEQUESTRATION CRISES
- ACUTE CHEST SYNDROME
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)
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
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
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
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.
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
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
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
How is infection in sickle cell disease managed?
- Oral penicillin prophylaxis should be started at diagnosis
- Routine childhood vaccination including pneumococcal.
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
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
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.
How is acute chest syndrome treated in sickle cell crises?
- CPAP and exchange transfusion
- Antibiotics- macrolide with intravenous cephalosporin
How is priapism in sickle cell crises, treated?
- Hydration and analgesia
- Adrenaline
- Etilefrine
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
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
What is the pathogenesis for neuroblastoma?
- Chromosomal and molecular abnormalities
What is the oncogene associated with neuroblastoma?
MYCN
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
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
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)
What are the complications of neuroblastoma at presentation?
- Cord compression
- Severe HTN
- Renal insufficiency
What are complications of neuroblastoma due to chemotherapy and surgery?
- Myelosuppression and immunosuppression
- Hearing loss
- Tumour lysis syndrome- hyperkalaemia, hyperuricaemia, hyperphosphataemia
- Intussusception
- Haemmorhage.
What is A-thalassaemia?
Mutation of the alpha globing gene, (4 different genes)
What is B- thalassemia?
Beta globin gene defects (2 different genes)
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.
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
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
What is the management for thalassemia?
- Blood transfusion (target is Hb>95)
- Iron chelation: desferrioxamine
- Regular reviews
- Haemoglobinopathy cards
- Family support
What are the complications associated with thalassemia?
- Iron overload –> endocrine dysfunction
- Transfusion risks
- Osteoporosis
- Gall stone and gout
- Hepatocellular carcinoma
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)
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)
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
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
What is the management for Wilms tumour?
- Nephrectomy followed by chemotherapy
- Radiotherapy to the flank in stage III
How many stages of Wilms tumour are there?
Five
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
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)
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
What is Stage IV Wilms tumour?
- Haematogenous metastases- beyond stage III i.e. to the lung, liver, bone, or brain
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.
What is a venous hum?
- Blowing continuous murmur in systole and diastole
- Heard below the clavicles
- Disappears on lying down
What is a pulmonary flow murmur?
- Brief high pitched murmur at SECOND LEFT INTERCOSTAL SPACE
- Best heard when child is lying down
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
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
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
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
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
Which pathologies present at the UPPER LEFT STERNAL BORDER?
- Pulmonary stenosis
- Atrial septal defect
- Innocent pulmonary flow murmur
- Tetralogy of Fallot
- Coarctation of the aorta
- Aortic stenosis
- Patent ductus arteriosus with pulmonary HTN
Which pathologies present at the UPPER RIGHT STERNAL BORDER?
- Aortic stenosis
- Supraventricular aortic stenosis
- Subaortic stenosis
Which pathologies present at the LOWER LEFT STERNAL BORDER?
- Ventricular septal defect
- Still’s murmur
- Hypertrophic obstructive cardiomyopathy
- Tricuspid regurgitation
Which pathologies present at the APICAL AREA?
- Mitral regurgitation
- Mitral valve prolapse
- Hypertrophic obstructive cardiomyopathy
- Vibratory innocent murmur
Which pathologies produce SYSTOLIC MURMURS?
- VSD
- TR
- MR
- PS
- AS
- ASD
- PDA
Which pathologies produce DIASTOLIC MURMURS?
- AR
- PR
- MS
Which pathologies produce CONTINOUS MURMURS?
- PDA
- Venous hum
- Arteriovenous malformations
How should heart murmurs be analyzed?
- INTENSITY (Grade 1-6)
- TIMING (Systolic or diastolic)
- LOCATION
- TRANSMISSION
- QUALITY (musical, vibratory, blowing, harsh etc)
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
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
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)
What are the 6 signs to indicate that a systolic murmur is likely to be pathological?
- Pansystolic murmur
- Harsh murmur
- Abnormal heart sounds
- Early/mid-diastolic click
- > Grade 3 murmur
- Heard over upper left sternal border
What are examples of innocent murmurs?
- STILL’S MURMUR
- PULMONARY FLOW MURMUR
- VENOUS HUM
- CAROTID BRUIT
- PERIPHERAL PULMONARY STENOSIS
What are the features of Still’s murmur?
- Mid left sternal border
- mid systolic
- grade 2-3
- twanging string
- musical
- vibratory sound
Differential: VSD
What are the features of pulmonary flow murmur?
- Upper left sternal border
- Mid-systolic
- Grade 1-3
- Grating
Differential: PS, ASD
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
What are the features of carotid bruit?
- Supraclavicular area
- ejection systolic
- grade 2-3
Differential: AS
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
What are the congenital heart diseases that present with a murmur?
- Pulmonary valve stenosis
- Atrial septal defect
What are the congenital heart diseases that present with cyanosis?
- Tetralogy of fallot
- Transposition of the great arteries
What are the congenital heart diseases that present with heart failure?
- Ventricular septal defect
- Atrioventricular septal defect
- Patent ductus arteriosus
What are the congenital heart diseases that present with shock?
- Coarctation of aorta
- Aortic valve stenosis
What is tetralogy of fallot?
The most common CYANOTIC congenital heart disease
Commonly seen in Downs syndrome and 22q11 microdeletion (Digeorge’s)
What are the four anatomical abnormalities associated with tetralogy of fallot?
- VSD
- OVERRIDING AORTA
- PULMONARY STENOSIS
- RIGHT VENTRICULAR HYPERTROPHY
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
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
What is the benefit of knee-to-chest/ squatting in tetralogy of fallot?
- Calms the infant
- Reduces systemic venous return
- Increases systemic vascular resistance
What are the best investigations for Tetralogy of fallot?
ECHOCARDIOGRAM is most comprehensive
ECG and CXR are also helpful
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
What are the complications associated with tetralogy of fallot?
- Cerebral thrombosis
- Brain abscess
- Bacterial endocarditis
- CCF
- Sustained VT and aortic root dilation
- Sudden death
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
What are the 5 types of atrial septal defect?
- Patent foramen ovale
- Secundum ASD
- Primum ASD
- Sinus venosus defect
- Coronary sinus defect
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
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
What is primum ASD?
Also known as partial AVSD and is associated with the presence of a common atrioventricular orifice.
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.
What is coronary sinus defect?
Tissue separating coronary sinus from the left atrium, allowing a shunt through the defect and coronary sinus orifice
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
What are the investigations in ASD?
ECG will show tall p waves and right axis deviation
Echocardiography
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
How is VSD classified?
Based on location of the lesion in the septum?
What are the types of VSD?
- Perimembranous: inlet, trabecular and infundibular
- Muscular
- Acquired
- Inlet/AV canal
- Subarterial infundibular
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
What are the investigations for VSD?
ECG will show biventricular and left atrial hypertrophy
Echocardiography
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
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
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
What are the risk factors associated with PDA?
- Prematurity
- asphyxia
- rubella infection
- genetic syndrome
- vampiric acid during pregnancy
- high altitude births
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)
What investigations are done in PDA?
- Echocardiogram
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
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
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
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
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
What are the complications associated with coarctation of the aorta?
- Recoarctation after repair
- CCF
- Aortic dissection, thoracic aortic artery aneurym and cerebral aneurysm rupture
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
What are the maternal risk factors for TGA?
- rubella infection
- FAS
- aged >40 years old
- DM
What are the three subtypes of TGA?
- TGA with intact ventricular septum
- TGA with VSD
- TGA with VSD and pulmonary stenosis
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
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
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
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.
How is asthma in children described?
Chronic inflammatory disease of airway leading to obstruction and:
- Wheeze
- Cough
- Dyspnoea
- Chest tightness
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
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
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
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
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
What investigations should be done with a low probability of asthma?
- Detailed investigations and referral especially when alternative diagnosis seems likely
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
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
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
What is the general management for childhood asthma?
- Allergen avoidance
- Smoking cessation of caregivers
- Education of family
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
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
What are the side effects of long term steroid use in childhood asthma?
- Growth stunting
- Immunosuppression
- Thrush
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)
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
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
What are the 4 steps to take if a child is having an acute asthma attack?
- Help them sit up straight and keep calm
- Help them take one puff of their reliever inhaler (usually blue)
- Call 999 if: symptoms get worse with inhaler
- Repeat step 2 if ambulance takes longer than 15 mins
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
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
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
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
What constitutes a near fatal asthma attack?
- Raised PaCO2 and/or requiring mechanical ventilation with raised inflation pressures
What investigations should be done in acute asthma attacks?
- PEFR (use best of three readings)
- O2 Saturations
- CXR and ABG
What is the management for an acute asthma attack?
ABCDE
Transferred to hospital ASAP
- SpO2 <94%, then high flow oxygen via NRBM
- Salbutamol (inhaler/ 2.5-5mg nebulizer)
- Steroid therapy (oral pred [20mg 2-5yo], [30-40mg >5])
- IV Salbutamol bolus (15ug/kg)
- Nebulised ipratropium bromide
- IV aminophylline with severe bronchospasm
- IV magnesium sulphate
- HDU/ICU admission
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.
What are the pathogens responsible for pneumonia in neonates?
- Female genital tract
- Group B strep,
- E.coli
- Gram negative bacilli
- Chlamydia trachomatis
What are the pathogens responsible for pneumonia in infants-preschool?
BACTERIAL (60%)
- Steptococcus pnuemoniae
- Staphylococcus aureus
- H. influenza
VIRAL (40%)
- Parainfluenza
- Influenza
- Adenovirus
- RSV
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
What are the pathogens responsible for aspiration pneumonia in children?
- Enteric gram negative bacteria
- Steptococcus pnuemoniae
- Staphylococcus aureus
What are the pathogens responsible for pneumonia in the non-immunized?
- Haemophilus influenza
- Bordatella pertussis
- Measles
What are the pathogens responsible for pneumonia in the immunocompromised?
BACTERIAL
- Pneumocystis carinii
- Tuberculosis
VIRAL
- CMV
- VZV
- HZV
- Measles
- Adenoviruses
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
What is the presentation for pneumonia?
- Fever
- Tachycardia
- Tachypnoea
- Cough
- Sputum
- Vomiting post coughing
- Poor feeding
- Diarrhoea
- Preceding URTI
What are the consolidation signs associated with pneumonia?
- Decreased breath sounds
- Dullness to percussion
- Increases tactile/vocal fremitus
- Bronchial breathing
- Coarse crepitations
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
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
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
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
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.
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
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.
Which antibiotics should be prescribed in pnuemonia?
- ORAL AMOXICILLIN OR ERYTHROMYCIN
- IV CEFUROXIME AND/OR ERYTHROMYCIN (in severe)
- METRONIDAZOLE (aspiration)
What should be done in respiratory failure from pneumonia?
- CPAP
- BiPAP
- PICU transfer
Which immunisations should be given to protect from pneumonia?
- Hib
- Pneumoncoccal
- Influenza
What are the complications associated with pneumonia in children?
- Pleural effusion
- Empyema
- Lung abscess
- Septic shock
- Acute respiratory distress syndrome
- Acute respiratory failure
What is bronchiolitis
- Lower respiratory tract infection in infants
- 2-6 months old.
- Viral infection of the bronchioles
Which virus is most likely to cause bronchiolitis?
RESPIRATORY SYNCYTIAL VIRUS
- hMPV
- adenovirus
- parainfluenza
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
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
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
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
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
When should you consider referral for bronchiolitis?
- Respiratory rate >60
- Difficult with breastfeeding or inadequate oral fluid intake
- Clinical dehydration
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.
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
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.
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%
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.
When are vaccines against RSV given for babies?
- Premature babies
- lung, heart or neurological disease
- immunocompromised
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
Which pathogen is most responsible for croup?
Parainfluenza virus
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
How is croup scored?
WESTLEY SCORING SYSTEM
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
What do the Westley scores for croup indicate?
0-3= MILD CROUP
4-6 = MODERATE CROUP
> 6 = SEVERE CROUP
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
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
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)
