Paeds Flashcards
What is Ipratropium (atrovent)?
Muscarinic antagonist used for bronchodilation
Differences in Bronchiolitis and Viral induced Wheeze?
Both present with cough, coryza, wheeze and possibly some respiratory distress
- Brochiolitis does not respond to inhalers, viral wheeze does
- Most children under the age of 1 will have bronchiolitis
- Resp distress in viral induced wheeze is caused by bronchospasm induced by the viral infection. In Bronciolitis it is caused by the large numbers of secretions in the airways.
- Viral induced wheeze will cause resp distress over a short space of time, bronchiolitis will cause a slow progression to respiratory distress
What is a macular rash, a vesicular rash, a papular rash and a pustular rash?
Macular:
- change in colour of the skin - normally associated with erythema
Vesicular:
- Clear fluid filled blisters
Papular:
- Solid raised lesion
Pustular:
- Pus filled blisters
What does coryzal mean?
Cold-like symptoms of the upper respiratory tract snotty inflamamtion of nasal passages
What is brittle bone disease?
Oteogenesis imperfecta
Group of genetic disorders affecting bone development (8 types)
Lack of type 1 collagen production
Types vary from mild symptoms to life threatening ones
What is Duchenne’s muscular dystrophy?
X-linked recessive disorder
Lack of protein dystrophin
Normally present at about 5 years old
Chair at about 8
Life expectancy is avg. 25 years
Progressive muscle weakness, often originally affecting proximal muscles of shoulder and thigh
Also affects smooth muscle - Respiratory failure is ultimate cause of death.
What is tuberous sclerosis?
Genetic condition that causes benign tumours to form, these are usually present from birth.
What is Montelukast?
Leukotriene receptor antagonist for treatment of asthma.
What are CPAP and BiPAP
Both create positive pressure to keep the airways open (usually used when sleeping e.g. in muscular dystrophy)
CPAP is continuous
BiPAP has two pressure settings - one for inhalation and one for exhalation.
What is kawasaki syndrome?
Rare Vasculitis
Range of symptoms, as described by a japanese doctor - it can be sub acute, acute and convalescent. Fever, red palms, strawberry tongue, lymphadenopathy
Treated with aspirin and antibodies
Triad of symptoms in autism?
Lack of:
- Social and emotional communication
- Imagination/flexibility of thought - activities and interests
- Social and emotional interaction
Types of symptoms in autism?
Social interaction:
- Appears unresponsive
- Absent/unusual eye contact and expressions
- Does not spontaneously share interest and enjoyment
- Does not make friends
Social communication:
- Unusual/repetitive language
- Delayed/impoverished language development
- Does not initiate/maintain
Activities & Interests:
- Pre-occupation with narrow interests
- Unusual/repetitive gestures & actions
- Rigid routines
Normal baby weight?
2.7-4.1kg
6-9lbs
Verbal development stages?
1 year - one word
2 years - phrases
3 years - sentences
Causes of clubbing?
Bronchiectasis (CF)
Fibronising alveolitis
cyanotic congenital heart disease
IBD
4 T’s in relation to diabetes?
Tired
Thin
Thirsty
Toilet
Fatigue
Weight loss
Polydipsia
Polyuria
Prevalence of childhood diabetes?
1 in 400 children
Main risk factors of children with diabetes at time of presentation?
Younger children
Fmaily Hx (1st degree relative)
Lower SES
Normal range of fasting blood glucose (for kids)
6.1-7.8
Brief description of DKA?
Essentially it’s a lack of insulin (why it’s type 1) this causes two main things:
- Increased release of glucose production from glycogen - spilling over into the urine, taking Na and K and extra water with it leading to polyuria, polydipsia and dehydration.
- Body uses fat as an alternative source of fuel - to protect the metabolically active brain. This forms ketones in large quantities which lowers the blood pH and induced metabolic acidosis.
What percentage O2 do you use for acutely unwell children?
100% - don’t fuck about.
Complications associated with DKA?
Hyperglycaemia
Hypokalaemia
Cerebral oedema
DKA treatment?
SLOW insulin infusion
Fluid resus - Normal saline w/ K+
Treatment of cerebral oedema?
Sit child up
High conc saline
Intubation
Signs of Cerebral oedema?
Biggest one - headache
Tachycardia/HTN
Also:
- Nausea
- Vomiting
- Reduced consciousness
DO you give child with DKA water?
No - will most likely just vomit it up and this increases risk of aspiration - may even put in NG tube.
What is Alport syndrome?
Genetic condition affecting children, rare. Characterised by glomerulonephritis, end-stage kidney disease and hearing loss. Type IV collagen is affected.
Investigations in children with possible epilepsy?
EEG
Bloods - magnesium
ECG - rule out cardiac cause e.g. long-QT syndrome
Brain imagining
Two types of absence epilepsy?
Childhood absence epilepsy - short fits, grow out if
Juvenile absence epilepsy - longer fits, probably won’t grow out of.
Types of epileptic seizures in childhood/generally?
Generalised:
Absence - transient loss of consciousness, with abrupt onset and termination, normally unaccompanied by motor phenomena apart from some increase in muscle tone and eyelid flickering.
Myoclonic seizures - Repetitive jerking movements of the limbs neck or trunk
Tonic seizures - generalised increase in tone
Tonic-Clonic - classic symptoms of a rigid tonic phase followed by rhythmic jerking movements, may also be accompanied by loss of continence, followed by deep sleep.
Atonic seizures - jerk followed by transient loss of muscle tone.
Focal
- Frontal - motor
- Temporal - auditory, smell or taste
- Occipital - visual
- Parietal - altered sensation (contralateral)
In what type of infection do lymphocytes fall and neutrophils rise?
Bacterial.
What are some of the possible causes of abdo masses in CF children?
Fibrosing colonopathy - right colon (RIF)
Hepatosplenomegaly
Constipation - LIF
Gastrostomy tube.
What is hypospadias?
Urethral orifice is not in the usual position - different grades.
What is rhesus incompatibility and how does it affect the neonate?
Antibodies to rhesus D antigens have developed.
Mother is Rh Negative and baby is positive then the mother may produce antibodies against the babies RhD antigens (on RBCs). Has to be second baby.
How many babies become jaundiced? What causes this around 24hrs?
Roughly 50% of babies are visually jaundiced.
Prehepatic - excessive breakdown of red cells (haemolysis)
Hepatic - abnormality in liver function (neonatal hepatitis)
Posthepatic - absent or bile duct atresia, causes conjugated hyperbilirubinaemia
Why is it important?
Unconjugated bilirubin deposited in the brain (basal ganglia and brainstem nuclei) may cause irreversible neurological damage - kernicterus
Normal physiological jaundice (>24hrs - weeks):
- Marked physiological release of haemoglobin from the breakdown of red cells due to high haemoglobin conc at brith
- Liver does not function as well.
<24hrs may also be a sign of another disorder:
- Haemolytic anaemia
- Infection
- Inborn error of metabolism
- Liver disease
Haemolytic disorders of the newborn?
Rhesus haemolytic disease
- Antibodies to rhesus D antigens have developed. Now mostly treated antenatally - low haemoglobin, hepatosplenomegaly, jaundice. Coombs test is positive.
ABO incompatibility
- Some group O women have IgG anti-A-Haemolysin in their blood, bad for group A infants. Group B infants also at risk. Hepatosplenomegaly is not usually present. Coombs test (detects antibody on red cells) is positive.
G6PD (gluc. 6 dehydrogenase deficiency.
- mainly in non-caucasian backgrounds. Mostly affects males
Spherocytosis
- Rare genetic disorder, Family history is present.
At what time of onset is jaundice worrying?
Before 24hrs - haemolytic disorder or infection.
After 2 weeks - breast milk jaundice, biliary atresia
Signs of respiratory distress in the infant?
Head bobbing Nasal flaring Grunting Recession (4 types) Tachopnoea (>60 RR) Cyanosis
Why do you feed small quantities slowly and early to neonates?
To avoid Necrotising enterocolitis (NEC)
Normal neonatal obs?
HR <170
<60 RR
BP - depends on gestation and age
Sys: ranges from 48-72 roughly
Dia: ranges from 25-50
Mean - above corrected gestation is normal
What is necrotizing enterocolitis?
Bowel becomes inflamed and necrosis occurs. Caused by bowel iscaemia and bacterial infection.
Seen within the first few weeks of life.
Early signs include feed intolerance and vomiting and bilious aspirates. Abdo becomes distended and stool can be blood stained.
Can induce shock, and can lead to bowel perforation.
Stop feed and give broad spectrum antibiotics, start Parenteral nutrition. Mechanical ventilation and circulatory support may be needed.
Normal ABG ranges?
pH: 7.35-7.45 pCO2: 4.7-6.0 pO2: 11-13 Bicarb: 22-26 BE: -2 to +2
Difference in type 1 and 2 resp failure?
Type 1: low O2 with no Co2 diff
- V/Q mismatch:
- Normal perfusion and reduced ventilation e.g.pulmonary oedema
- Normal ventilation and reduced perfusion e.g. Pulmonary embolus
Type 2: both O2 and CO2 are affected O2 is low, CO2 is high.
- alveolar hypoventilation
Causes of Hyponatraemia?
Fluid status:
- Low volume/Normal volume/High Volume
Low volume:
- D&V
Normal volume, conc. Urine:
- SIADH
Normal volume, dilute urine:
- adrenal insufficiency, Hypothyroidism, too much water
High volume:
- HF
- Liver failure
- Kidney failure
Causes of loose stools in a child?
IBD IBS (less likely) Long term infection Diet - too much fibre Addison's
Symptoms to ask if loose stools is presenting complaint?
Blood/mucus in stool Consistency Colour of poo Weight loss Abdo pain Intolerance to anything Coeliac? Difficult to flush away Smelly
What does fetal calpotectin test for?
Sensitive for IBD, not specific though
Types of spina bifida?
Occulta - most common, vertebrae don’t properly form, doesn’t usually cause issues
Meningocele - meninges push out, will need surgery, outcomes aren’t too bad.
Myelomeningocele - most severe, spinal canal is actually open.
What is Hirschprungs disease?
Congenital megacolon
Part/all of the large intestine has no nerve supply, causes blockage.
Treated surgically by removing that bit of bowel.
What is congenital adrenal hyperplasia?
Collection of autosomal recessive genetic conditions that interfere with the production of steroids from cholesterol by the adrenal glands. Either results in excess or insufficient sex hormones.
Differential diagnoses for widening of the mediastinum?
Lymphoma
Thymus
Vascular anomalies
What is HSP (Henoch-Schonleine-purpura), what are it’s clinical features?
IgA vasculitis
Affects skin, mucous membranes of 3-13yr olds (ish), more common in boys, usually after respiratory infection.
Classic symptomatic triad of
- Purpura, skin rash
- Arthritis
- Abdo pain
Can also get periarticular oedema and glomerulonephritis
Clinical diagnosis based on combination of symptoms.
Hep B problem in children?
In children it can lead to chronic hep b infection (90% of children with hep b)
What is the retic count, in neonates?
The reticulocyte (“retic”) count is the percent of RBCs that contain nucleic acid (also called “reticulated” cells), The retic count is used to assess whether the marrow is producing more RBCs than in the normal steady state.
High in haemolytic disease
Low in congenital anaemia
What is magnesium sulphate used for?
Bronchodilator in children
Prevent eclampsia in pregnancy
What is a pyeloplasty?
Operation to remove a blockage from one of the ureters.
Stent is put in and later removed.
Causes of hyponatraemia in neonates?
Administration of hypotonic fluids during the delivery.
Renal failure
Infection
Gi fluid loss
Adrenal insufficiency
Anatomical terms for a clear fluid filled area superficially on skin?
Vesicle - smallest <5mm
Bulla - >5mm
Anatomical terms for a change in surface colour without elevation or depression?
Small (<5mm) - macula
Larger (>5mm) - patch
Different anatomical terms for raised solid elevation of skin with no visible fluid?
Papule - <5mm
Plaque - larger confluence of papules >1cm
Nodule - similar to a papule (<5mm) but deeper in the dermis.
Tumour - >5mm nodule
What is purpura? What is it called if it’s smaller?
Non-blanching superficial, caused by broken blood vessels measuring 3-10mm
Petechiae is the same but smaller than 3mm.
Causes of jaundice >24hrs?
Physiological jaundice
- normal jaundice, caused by liver not working as well, and high levels of fetal haemoglobin breakdown.
Breast milk jaundice
- unconjugated bilirubin, normal
Dehydration
- in some infants IV fluids are needed. Caused by lack of bilirubin excretion?
Infection
BILIARY ATRESIA
Jaundice metabolism?
Haemoglobin breakdown to form unconjugated bilirubin.
Unconjugated bilirubin is conjugated in the liver
All of this conjugated bilirubin is excreted into the bowel. In the bowel bacteria form urobilinogen (some of this circulates back to the liver and some to the kidneys (where it is excreted in the urine)
Urobilinogen in the bowel is further processed to form stercobilinogen - darker colour in the stool.
Light stools - hepatic blockage (no stercobilinogen in stool)
dark urine/stool - extra urobilinogen in urine - haemolysis
What is neonatal erythema toxicum?
Normal baby rash - will pass,
central yellow plaque with a halo of erythema
What does the TORCH screen include
T- Toxoplasmosis O- Other - syphilis R- Rubella C- CMV H- Herpes
Measles rash and signs?
Koplik spots - appear as white spots in teh mouth before the rash does.
Rash consists of confluenced macula (and sometimes papular) widespread - starts on the head/nech then spreads to rest of body.
Complications from chickenpox?
Ataxia from cerebellitis
Encephalitis
Rheumatitis
GI involvement
What is impetigo? what does it look like?
Superficial skin bacterial infection caused most commonly by Staph A (but also strep & MRSA)
Crusty yellow lesions of face arm and legs - may be painful/itchy
Common causative organisms of abscesses?
Staph and Strep
What is ichthyosis?
Family of rare genetic skin disorders, dry thickened scaly skin.
Linked to metabolic syndromes such as T2DM and PCOS - have to screen.
What is aminophylline?
Methylxanthine, give via infusion - bronchodilator
What is clenil?
Beclomethasone
Principles of the UN statement on the rights of the child?
Right to survival
Right to health and healthcare
Right to develop to the fullest
Right to full participation in family, social and cultural life
Right to freedom from violence
In the UK the 1989 and 2004 children act legislates for these rights
Definition of abuse and neglect?
Maltreatment of a child by inflicting harm or failing to act to prevent harm…
In a family/institutional/community setting…
By those known/not known to them…
by an adult or child
Categories of abuse?
Physical abuse
Sexual abuse
Neglect
Emotional abuse
Definition of physical abuse?
Any physical harm to a child, including when a parent or carer fabricates the symptoms of illness, or causes illness
Definition of sexual abuse?
Forcing or enticing a child to participate in sexual activities, whether they are aware of what is happening or not.
Definition of neglect?
Persistent failure to meet a childs physical or emotional needs, likely to result in the serious impairment of the childs health or development
Definition of emotional abuse?
Persistent emotional maltreatment of a child, such that it impairs the childs emotional development.
Domestic violence for example.
How many 18-24 year olds report some kind of abuse?
11%
Toxic trio that potentially increase the risk of abuse
Domestic abuse
Alcohol/drug misuse
Mental illness
What should you do if you are concerned about a child?
Record full details of presenting incident, your concerns and observations
Listen to child and put them first
Seek advice from a senior
Consider who else might be involved, siblings?
What type of immunity do vaccines produce?
Active immunity
What can vaccines be made up of?
Inactivated or attenuated live organism
Secreted products
Components of cell walls
What is the childhood immunisation schedule?
2 months:
- DTaP
- IPV
- Hib
- PCV
- Men B
- Rotavirus
3 months
- DTaP
- IPV
- Hib
- Rotavirus
4 months
- DTaP
- IPV
- Hib
- PCV
- Men B
12 months
- Hib
- Men C
- MMR
- PCV
- Men B
2-7 years
- Influenza
- 5 - 4 years
- DTap
- IPV
- MMR
Girls 12/13
- HPV
14 Years
- TdIPV
- MenACWY
Aims of herd immunity?
- Reduce sources of infection
- Those who cannot be immunised will still benefit
- May eliminate disease
Contraindications for vaccinating a child?
For inactivated vaccines true anaphylaxis is the only contraindication
Severe allergic reactions, stable neurological conditions, local or general reactions are all not contraindications
Live vaccines
- High dose steroids
- Immunosuppressive treatment
- hypogammaglobulinaemia
- Pregnant
- Had a live vaccine in previous 3 weeks
Which vaccines are inactivated
Pertussis Polio Tetanus Diptheria Pneumococcus
Primary response - IgM then IgG
There’s a primary course then a booster
Which vaccines are live?
MMR Varicella Influenza Yellow fever BCG
May see mild form of disease
Four main things in a 6 week health check?
Physical exam
Review of development
Give health promotion advice
Opportunity for parent to express concern
What are you checking for in the physical exam in the 6 week baby check?
Congenital heart disease
Developmental dysplasia of the hip
Congenital cataracts
Undescended testes
How would you check for congenital heart disease in teh 6 week check?
Look for cyanosis, ventricular heave, respiratory distress, and tachypnoea; a respiratory rate persistently over 55 is suspicious.
Feel for apex beat and assess whether displaced.
Listen for murmurs. Innocent murmurs are common and are typified by low intensity, localised to a small area of precordium and in the absence of other symptoms or signs. All murmurs should be referred to a specialist for assessment.
How would you check for developmental dysplasia of the hip (in the 6 week check)?
leg-leg length discrepancy
Asymmetry of leg creases
Barlow and Ortolani tests
- Barlow: press both thighs posteriorly looking to see if femoral head dislocates
- Ortolani: looking to see if femoral head pops back in
If abnormality should have USS
Eye exam in the 6 week check?
Examine external eyes (one eye bigger may indicate glaucoma)
Check for normal red reflex
Check for FH, ask if parents have any worries
Any abnormalities should be treated as urgent referral, abnormal red-reflex should be same day
How would you assess the development of the baby in the 6 week check?
Review feeding and weight gain
Check growth chart
Review vision and hearing
Most will be spontaneously smiling, and making a variety of sounds e.g. coos
What health promotion discussions should you have in the 6 week check?
Immunisations
Breast feeding, check tongue tie
SIDS
- No smoking
- Sleep on back (but have tummy time)
- Don’t sleep on same bed/sofa together
- Avoid overheating
- Breast feed
Could ask about maternal depression
Common physical signs of abuse?
Abnormal bruising
Cuts and scratches
Bite marks
Unconscious or fitting (shaken baby)
Subtle signs such as sky away from touch, not want to get changed for PE, afraid to go home.
What would constitute abnormal bruising?
- <1 year (as not walking)
- Disproportionate to reason given
- Multiple sites or clusters
- Look like a hand, stick, tooth, belt
- Non bony part of the body or face e.g. eyes, ears, cheek, back, abdomen
- Ankles or hands
- On neck
- Delay in presentation
Childs behaviour that may suggest sexual abuse?
Anxious about going to a particular place or seeing a particular person.
Sudden mood swings, or aggressiveness
Displays over-sexualised behaviour or inappropriate knowledge for their age
Who is the usual suspect for sexual abuse?
Usually the abuser is a family member or someone known to the child, such as a family friend. For teenagers it is commonly a boyfriend or girlfriend. Child sex abusers can come from any professional, racial or religious background, and can be male or female.
Older children may abuse younger children.
4 areas of early development
Gross motor
Vision and fine motor
Hearing, speech and language
Social, emotional and behavioural
When do you stop correcting for prematurity?
At 2 years post-natal
Rough guide to gross motor development 12 months up until 3 years?
6-8 weeks
- Moves head side to side when prone
- developing head control
6-8 months
- sits briefly
- rolls (both ways)
- starts to crawl
12 months
- walks unsteadily
18 months
- squats to pick things up off the floor
- Runs
- Walks well
2 years
- Kicks ball
- Climbs stairs 2 feet at a time
- 5 years
- Running well
3
- Jumps with both feet
- Stairs 1 foot at a time
4-5
- Stands on one foot
- Throws and catches
Fine motor/vision rough guide from 14 months to 4 years?
6-8 weeks
- grasp reflex
- hands lightly closed
6-8 months
- Palmar grasp
- Object from hand to hand
- Reaches for toys
12 months
- Mature grasp
- Gives objects away
18 months
- Linear scribble
- Tower of three
2 years
- Tower of six
- Circular scribble
- 5
- Tower of eight
3 years
- Circle
- Bridge
4 years
- Square
- Steps after demonstration
- Man with head, legs and trunk
5 years
- Triangle
- Person with 6 body parts
- fork and spoon
Rough guide to communication and hearing 12 months to 3.5 years?
6-8 weeks
- Coos and gurgles
- Watches mums face
- Startled by loud noises
- Cries when hungry or uncomfortable
6-8 months
- Turns to mum
- laughs aloud
12 months
- can say single words, probably two or three in vocab
18 months
- Can point
- 6-10 words, knows two areas of the body
20-24 months
- Simple phrases using two or more words
- 5 - 3 years
- Talks all the time in 3-4 word sentences
4-5 years
- Speech grammatically correct
- Nursery rhymes
- Name and age
Rough guide to social, emotional and behaviour development?
6-8 weeks
- Smiles
- follows people with eyes
6-8 months
- Takes everything to mouth
- Plays with rattle
- Looks at self in mirror
12 months
- Holds spoon, puts to mouth
- waves bye-bye
- Demonstrates affection
18 months
- Symbolic play beginning
- Uses a spoon
24 months
- Dry by day
- Pull some clothes off
- Toddler tantrums
- No awareness of danger
3 years
- Turn taking
- Vivid interactive play with others
- Names friend
4-5 years
- Dresses and undresses
- Complex games
- Comforts others
- independent toileting
What are learning disabilities and learning difficulties?
Learning disabilities is the general condition of arrested development, resulting in the impairment of skills manifest during the developmental period.
Learning difficulties applies to a specific area of difficulty e.g. dyslexia or dyscalculia
Different grades of learning disabilities?
Mild (IQ 50-69)
Moderate (35 - 49)
Severe (20-34)
Profound (<20)
Learning difficulty causes?
Prenatal
- Genetic abnormalities
- Intrauterine abnormalities
- Intrauterine insults
Perinatal
- Prematurity
- Severe asphyxia
- meningitis
Postnatal
- Infection
- Trauma
- Lead poisoning
- Malnutrition
- Neglect
What is neurodisability?
Group of congital or acquired disorders including impairment to the brain or neuromuscular system, affecting: Movement, Cognition, Hearing or vision, communication and emotion/behaviour
General structure to an assessment of development?
History
- Parents concerns
- Is there a barrier to learning?
- Is there a useful diagnosis to be made?
Reports
- Nursery
- School
- physio
- SALT
Observation and informal development
- may be different to testing situation
Formal evaluation
- Schedule of growing skills
- Griffiths (toys)
- Bayley infant development scales
- Hammersmith infant neurological examination
Clinical signs in a neuro-developmental assessment?
Patterns of growth
Dysmorphic features
Neurocutaneous stigmata
CNS abnormalities
- Tone
- Wasting
- reflexes
CVS abnormalities
Visual abnormalities
Hearing abnormalities
- Ask parents
- Check tests were done as newborn
Patterns of mobility
- dexterity
- hand dominance
Causes of abnormal development (broadly)?
Neglect of physical and psychological needs
Ill health
Neurodevelopmental disorder
Causes of abnormal development (broadly)?
Neglect of physical and psychological needs
Ill health
Neurodevelopmental disorder
When does global developmental delay normally present?
Within the first two years
Causes of abnormal motor development?
Central motor
- Cerebal palsy
Congenital myopathy/primary muscle disease
Spina bifida
Global developmental delay
- Syndromes
- Unidentified cause
If a child showed hand dominance or asymmetry of motor skills within the first year is this okay?
No, hand dominance isn’t present until 1-2 years, and this may suggest a underlying hemiplegia
When is the limit age of walking?
When might this be less worrying?
> 18 months
If the child shows the variants of bottom shuffling or commando crawling this may be normal.
What is cerebal palsy?
An abnormality of movement or posture caused by disturbances in the developing fetal or infant brain.
Used up until about 2 years old then would call it acquired brain injury
Often accompanied by disturbances of
- cognition
- communication
- perception
- sensation
- behaviour
- seizure disorder
What are the causes of cerebral palsy
80% antenatal
- Vascular occlusion
- Cortical migration disorders
- Structural maldevelopment
Genetic syndromes
Infection
10% due to hypoxic injuries at delivery
About 10% postnatal
- CNS infection
- Head trauma
- Hypoglycaemia
- Hyperbilirubinaemia
- Hydrocephalus
Early features of cerebral palsy?
Abnormal limb/trunk posture and tone
Feeding difficulties
Abnormal gait
Asymmetric hand function
Types of cerebral palsy?
Spastic
- UMN
- Increased limb tone
Dyskinetic
- involuntary, uncontrolled movements
Ataxic
- hypotonia and poor balance
Causes of speech and language abnormalities?
Hearing loss
GDD
Anatomical problem e.g. cleft palate
Environmental deprivation
Normal variant
What type of neuro-developmental deal would downs cause?
Global developmental delay
When does autism present?
2-4 years
Management of autism spectrum disorder?
Applied behavioural analysis is the only intervention really shown to work
What is development co-ordination disorder or dyspraxia?
Disorder of motor planning and/or execution, without any findings on a neurological examination, a disorder if higher cognitive functions it is the inability to execute a planned series of actions.
Difficulties can present as:
- Handwriting
- Dressing (buttons etc)
- Cutting up food
- poorly established laterality
- copying and drawing
- Messy eating
What is dyslexia?
Disorder of reading skills disproportionate to the child’s IQ, need to include vision and hearing assessment.
Causes of in-toeing in children?
Metatarsus varus
- passively correctable
- no treatment required
Medial tibial torsion
- toddlers
- self corrects in 5 years
Persistent anteversion of the femoral neck
- usually self corrects by 8
- if persistent can do femoral osteotomy
Types of muscular dystrophy?
Duchenne
Becker
Congenital
Inheritance pattern of duchenne muscular dystrophy?
X-linked recessive
Brief summary of the pathophysiology of duchenne’s?
Abnormality in coding for the dystrophin protein. Leads to malformation of muscle fibres.
What blood result is high in duchennes muscular dystrophy?
CPK
creatine phosphokinase
When are duchenne’s patients normally first noticed?
5.5 years
Presentation of duchenne’s?
Waddling gait
+/- language delay
Pseudohypertrophy of calves
Clumsier and slower than peers
Progression of duchennes?
Present 5-6 years
In a wheelchair by 10-14
Respiratory failure and associated cardiomyopathy
Life expectancy is late 20s
Management of duchenne’s muscular dystrophy?
Physio
- Appropriate exercises to maintain muscle power and delay the onset of scolios
Contractures, particularly at the ankles should be prevented by passive stretching and wearing of splints
Later CPAP may be required, or NIPPV
Brief differences in becker muscular dystrophy?
Some functional dystrophin is still produced, similar features to duchenne’s, but will progress slower (age of onset is 11 y/o), life expectancy is 40 or normal
Features of congenital muscular dystrophy?
Recessively inherited heterogenous group of disorders.
Normally presents at birth with weakness, hypotonia or contractures
How common is down syndrome in the UK?
1 in 1000 births
Most common form of inheritance for down syndrome?
Non-dysjunction
What is non-dysjunction?
Error at meiosis (sex cell production producing haploid cells.
Chromosome 21 from the parent cell does not divide producing one gamete with two 21’s and one with none.
How can down syndrome otherwise be inherited (not non-dysjunction)?
Translocation
- 21 is joined onto another chromosome (normally 14)
- Can produce carriers
Mosaicism
- Some cells trisomy some not
How is down syndrome screened for antenatally?
Combined test offered at 10-14 weeks
- PAPP-A
- Nuchal translucency scan
- β-hCG
Facial features of down syndrome postnatally? (10)
Round face with flat nasal bridge
Upslanted palpebral fissures
Epicanthic folds
Brushfield spots in iris
Small mouth and protruding tongue
Small, low-set ears
Flat occiput and third fontanelle.
Short neck
Other (non-facial) symptoms of down syndrome?
Single palmar crease
incurved 5th finger
wide gap between Big and 2nd toe
Features of turners syndrome?
Short stature
Webbed neck
Low set ears
Broad chest
Features of edwards syndrome?
Microcephaly
Prominent occiput
Narrow palpebral fissures
Cleft lip/palate
Low set ears
Features of fetal alcohol syndrome?
Smooth philtrum
Thin upper lip
Small palpebral fissures
Single palmar crease
Complications associated with downs?
Cardiovascular complications VSD. ASD PDA Aortic Coarctation MVP Tetralogy of Fallot
Duodenal atresia
Hirschprungs disease
usual age for presentation of diabetes in children?
above 10, between 10-14 yrs normally
Adverse health outcomes for children with diabetes?
Reduced life expectancy
- 20 years in T1DM
- 10 years in T2DM
Increased risk of stroke and MI
Renal failure
Blindness
Stillbirth/miscarriage/congenital malformations
Findings on urine dipstick in childhood type 1 diabetes?
Glycosuria, ketonuria (can be normal if not glycosuria, in diabetes may be indicative of DKA.
Treatment for T1DM in kids?
Fast acting insulin
- novorapid
Slow acting insulin
- Glargine
Mixed
Basal bolus regime
- Long acting at night
- Short acting
Mixed regime
- two injections 10 hours apart
Hypoglycaemia blood glucose in a patient on insulin?
<4mmol/L (<2.7mmol/L in others)
What would you tell a child to look out for in hypoglycaemia?
Sweating
Fatigue
Feeling dizzy
Is it common to be in DKA when presenting for T1DM as a kid?
Yes, 2/3 kids are at presentation.
DKA presentation?
Polyuria Polydipsia Breathless Tired Weight loss N&V Headache Ketotic breath Shock Drowsy Abdo pain Constipation
What type of acid base disturbance would DKA cause?
Metabolic acidosis, (N&V, Dehydration?)
Three stages of paediatric growth?
Infant (0-2)
- rapid growth
- nutrition deoendent
Childhood (2-puberty)
- Steady growth
- Hormone dependent
Pubertal growth
- Rapid linear growth
- Depends on pubertal hormones and GH
- Start of puberty in girls
- End of puberty in boys
When should you refer a child for poor growth?
Short for parents height (mid-parental height)
Height velocity is below that for age
Crossed >2 centiles up or down
Height <0.4th centile
Average age of puberty in girls and boys?
10.5 - girls
14 - boys
What age is precocious puberty in boys and girls?
<8 in girls
<9 in boys
What age is pubertal delay in boys and girls?
> 13 in girls
>15 in boys
What is arrested puberty for boys and girls?
Time to complete puberty is too long
Girls should complete in 4 years
Boys should complete in 6 years
Type of conditions that can cause abnormal growth and development?
Chromosomal abnormalities Malnutrition - poor intake or malabsorption Chronic ill health Iatrogenic Psychological & emotional factors Skeletal abnormalities Syndromes Hormones
How would coeliac disease present in terms of growth and development?
Abdo distention
Discrepancy between parental and child’s height
Drop in weight following weaning
If a kid is fat and tall what’s most likely the diagnosis?
Exogenous obesity
If a kid is short and fat what’s the most likely diagnosis?
Endocrine disorder
How might hypothyroidism present in childhood?
Short stature - thyroxine is important for growth
Might be cold
Dryness of the skin and hair, coarsening of the facial features, constipation and a slow pulse rate all occur in children but tend to be relatively late features.
In terms of growth and development how might turners present?
Pubertal delay and short stature.
Ddx if a girl of 5 y/o started developing pubic hair?
Increased adrenal hormone secretion
Precocious puberty
Premature Adrenarche (normal variant)
Chromosomal abnormalities associated with abnormal growth?
Downs - short
Klinefelters - XXY, boys may grow more quickly
Turners - short/no puberty
Nutritional disorders causing abnormal growth?
GORD - feeding aversion
Coeliac - short
IBD
Pancreatic insufficiency - CF
Most common skeletal abnormality causing poor growth?
Achondroplasia
Genetic syndromes associated with short stature?
Noonans - cardiac and developmental delay
Williams - elfin appearance, warm and bubbly, short stature and cardiac defects
Russel-silver syndrome - poor growth
Hormonal problems resulting in abnormal growth?
Hypothyroidism - short and fat
GH deficiency - short and slightly chubby
Hypopituitarism - short
What can affect GH?
Stress
Poor sleep
Malnutrition
SOL in pituitary
What investigation would you want to do in precocious puberty?
Image pituitaries
If a kid is gillick competent can they refuse consent given on behalf of them?
No - can only give consent
What are you checking for in a newborn infant assessment?
Birth weight - centile & age plotted
Posture & movements
Head circumference
Fontanelles
- if tense and no crying need to exclude hydrocephalus
Face
- genetic abnormalities
Pale
- check anaemia
Jaundice
- <24 hrs is abnormal (haemolysis)
Red reflex
- cataracts
- retinoblastoma
Palate
- cleft
RR, auscultate heart
Abdo masses
Femoral pulse
- coarctation of the aorta
Genitalia and anus
- check wee and poo
Muscle tone
Back & spine
Hips
- the two tests (barlow and ortolani)
What is hypoxic ischaemic encephalopathy?
ischaemic injury occurring perinatally at the time of labour or delivery resulting in brain damage.
Common causes of hypoxic ischaemic ancephalopathy/
Excessive/prolonged contractions (hyperstimulation?)
Cord compression (shoulder dystocia & prolapse)
Hypo/hypertension
IUGR/anaemia
Failure to breathe
What causes RDS in neonates?
Surfactant deficiency
Management of neonatal jaundice?
Phototherapy
- blue-green light converts unconjugated bilirubin into a harmless water soluble pigment
Exchange transfusion
- at higher levels of bilirubin
- blood from art line or umbilical vein is removed, and replaced with donor blood.
Signs of respiratory distress syndrome in the newborn?
Same as normal respiratory distress:
- Head bobbing
- Nasal flaring
- Grunting
- Recession (4 types)
- Tachopnoea (>60 RR)
- Cyanosis
Presentation of IVH in the newborn?
Intraventricular haemorrhage
Symptoms:
- Diminished/absent Moro reflex.
- Poor muscle tone.
- Sleepiness.
- Lethargy.
- Apnoea.
Signs:
- The fontanelle may be tense and bulging with severe IVH.
- Neurological depression may progress to coma.
In mild forms there may be no clinical signs, or there may be alternating symptomatic and asymptomatic periods.
Management of IVH in the newborn
Intraventricular haemorrhage
Management is initially mainly supportive and may include the correction of anaemia, acidosis and hypotension. Ventilatory support may also be required for some who deteriorate acutely.
Fluid/volume replacement:
- Packed red blood cells or fresh frozen plasma for anaemia and shock.
- Sodium bicarbonate infusion (carefully) for metabolic acidosis.
What is the APGAR score how do you calculate it?
(named after it’s creator)
A - appearance
P - Pulse (>100 N)
G - Grimace (movements)
A - Activity (Strong/weak)
R - Respiration (cry/no cry)
0 is nothing
1 is something
2 is normal
What is transient tachypnoea of the newborn?
A self-limiting period of respiratory distress most often seen in term babies following pre-labour Caesarean Section. It is thought to be due to delayed absorption of lung fluid and may require oxygen therapy.
What is meconium aspiration?
It is not uncommon for infants to pass meconium before delivery. In rare instances the infant may aspirate meconium stained liquor, this occurs when a hypoxic infant begins to gasp in utero. Pulmonary vasodilatation is delayed and results in Persistent Pulmonary Hypertension which can make an infant very unwell.
Why do we give vit k to babies?
To prevent haemorrhagic disease of the newborn - deficiency in factors II, VII, IX, X1
What is cephalohaematoma in the newborn?
Subperiosteal haematoma (can’t cross suture line) resolves over weeks, may exacerbate jaundice
What is a caput in the newborn?
Oedema and bruising usually associated with a ventousse delivery, resolves in a few days and can cross suture lines
Benefits of breast feeding?
Nutrition, protection from disease, development, allergy, autoimmune, reduced obesity and long term health.
General advice regarding the continuation of breast feeding and the administration of bottle fed milk?
Breast feeding is the gold standard; the World Health Organisation recommends that infants should be exclusively breast fed until 6 months of age
Solids should not be introduced before 4 months
Bottles for formula milk must be sterilised and milk made up according to instructions with boiled water
Causes of prolonged jaundice
Most commonly breast milk jaundice
Although must rule out biliary atresia
- pale stools
- dark urine
Infants at risk of GBS infection?
Premature
If the mother is colonised with Group B strep or has previously had a child with Group B strep infection
There is evidence of maternal infection (pyrexia or elevated CRP)
There is prolonged rupture of membranes
Evidence of fetal distress
Four infections mothers are screened for?
hepatitis B, HIV, syphilis and susceptibility to rubella
When is the blood spot screening performed, what is it testing for?
PKU
Congenital Hypothyroidism
Sickle cell disease
MCADD
CF
Isovaleric acidaemia
Maple syrup urine disease
Homocystinuria
Glutaric Aciduria type 1(GA1)
What type of bilirubin crosses the BBB?
Unconjugated bilirubin, this occurs in kernicterus
What does the ductus arteriosus do antenatally? What is the problem in PDA?
Shunts blood from the pulmonary artery to the aorta.
In PDA this means there is less volume of blood in the aorta
Presentation of PDA in a premature infant?
Classical signs are usually absent
May be systolic murmur in left sternal edge
Bounding peripheral pulses
Hyperactive precordium (can see it moving)
Tachycardia with or without gallop rhythm
Investigations for suspected PDA?
Echocardiogram
Management of PDA in the preterm infant?
Possible NSAIDS
?indomethacin
Conservative
Surgical closure
- may be associated with increased morbidity
Presentation of NEC in the prem baby?
Abdominal distension with increasing gastric aspirates.
Altered stool pattern.
Bloody mucoid stool and bilious vomiting.
Decreased bowel sounds with erythema of the abdomen.
Palpable abdominal mass or ascites.
Associated features are bradycardia, lethargy, shock, apnoea, respiratory distress, temperature instability.
Investigations for NEC?
Bloods - FBC and blood gas, serial CRPs
Abdo XRAY confirms findings/not
Management of NEC?
Nil by mouth
TPN, IV fluids and Abx
surgery inc. bowel follow through can be performed
Most common cause of ambiguous genitalia in newborns?
Congenital adrenal hyperplasia
Diagnosis of hirschsprungs disease?
Rectal biopsy
Presentation of hirschsprungs disease?
Delayed passage of meconium (> 2 days after birth), abdominal distension and bilious vomiting. May have distended abdomen, and bowel movements not working otherwise - visible waves of peristalsis.
Causative organism in scarlet fever?
Group A haemolytic streptococci
How do left to right shunts commonly present?
Breathless
How to right to left shunts commonly present?
Blue
inheritance pattern of haemophilia?
X-linked recessive
Causes of left to right shunts?
VSD
PDA
ASD
Causes of right to left shunts?
Tetralogy of fallot
Transposition of the great arteries
Innocent murmurs?
The S’s
aSymptomatic patient
Soft blowing murmur
Systolic only
left Sternal edge
ASD presentation?
None (common)
Recurrent chest infections / wheeze
Arrhythmias
Ejection systolic murmur on upper left sternal edge
Management of ASD?
Correction by 3-5 years of age in ASDs that are significant enough to cause right ventricle dilatation
Presentation of a VSD?
Small:
- asymptomatic
- Loud pansystolic murmur left lower sternal edge
CXR, ECG will be normal
Echo will show defect
Large:
- HF, breathlessness, recurrent chest infections
Tachypnoea, tachycardia, hepatomegaly
Active precordium
Pansystolic murmur
CXR
- cardiomegaly
- Enlarged pulmonary arteries
- Pulmonary oedema
ECG
- Biventricular hypertrophy
Echo
- shows defect
VSD management?
Small may manage conservatively
First line is increased calorific density of feeding – orally if they can tolerate it, and if they can’t manage oral feeds can supplement with NG feeds.
Don’t give fluids – more volume, more work – exacerbate the problem
Manage HF:
- Diuretics
- Captopril ACEI
Digoxin (but not really)
What is a PDA (in a term infant)?
When the PDA has not closed 1 month after EDD
PDA clinical features?
Continuous murmur beneath the left clavicle
Wider pulse pressure - collapsing or bounding pulse
Large PDA may cause HF & pulmonary hypertension
CXR and ECG normally normal
Management of PDA >1month
Close it with coil or occlusion device
Four features of tetralogy of fallot?
Large VSD
Overriding aorta
Subpulmonary stenosis
RV Hypertrophy
Presentation of tetralogy of fallot?
severe cyanosis
hypercyanotic spells
squatting on exercise
Clubbing
Loud ejection systolic
Management of tetralogy of fallot?
Neonates
- prostin to keep ductus arteriosus open
Surgically
- shunt
- RVOT stent (more common now)
if less severe can perfom surgery 3-6 months
in attack:
- On shoulder with calming
- Otherwise give O2 and morphine +/- intravenous propranolol.
What are AVSDs most commonly associated with?
Trisomy 21
Common genetic abnormality associated with fallots?
22q 11 deletion
What is coarctation of the aorta?
Narrowing of the aortic arch
Clinical findings in coarctation?
Hypertension in upper vasculature - arm
Treatment of coarctation?
Prostin to maintain ductus arteriosus and supply lower half of body
Then surgery in first few weeks to correct
If narrowing is less, may present late
- Notching on CXR
- Absent femorals
- HTN upper body
Management in transposition of arteries/
Prostin to keep ductus arteriosus open
Sugical operation called balloon atrial septostomy - shunt in atrium
Later (2 weeks) do arterial switch proceadure
What Is HLH in children how do they present and how do you manange?
Hypoplastic left heart - very rare
Pesent dueing antenatal screening usually or at birth will be very sick with no peripheral pulses
Need to do norwood proceadure
What is aortic stenosis in newborns?
Aortic valve leaflets are fused together can present with other abnormalities such as coarctation of the aorta
How does aortic stenosis in newborns present?
Asymptomatic murmur, ejection systolic (upper right sternal edge)
Carotid thrill
Ejection click
Management of aortic valve stenosis?
Monitor early to assess when to intervene then balloon valvotomy, less complicated when older.
If problems such as intolerance to exercise may have to do younger, will probably eventually need to replace aortic valve
Interval symptoms in asthma?
Night time cough
Wheeze on exertion
Diagnosis of asthma in children, 3 cardinal symptoms?
Breathlessness
Wheeze
Cough
What might asthma show on spirometry?
15% improvement on reversibility
Treatment of asthma?
Preventers
- ICS
- LABA
- Leukotriene receptor antagonist
Relievers
- SABA
- Ipratropium bromide
Start with SABA,
then ICS
then LABA/Leukotriene
then more steroid
Genetic abnormality in CF and inheritance pattern?
ABnormality in the CFTR chloride channel on chromosome 7 causing thick mucus secretions
1 in 20 people are carriers
Autosomal recessive
How does CF present?
20% at birth with bowel obstruction (meconium ileus)
Older
- cough
- recurrent infection
- fatty light stool (pancreatic insufficiency)
- failure to thrive
CF management?
Physiotherapy (at least twice a day)
High calorie diet
Pancreatic enzyme supplements
Psychological input
Bronchiolitis management?
Supportive, O2 therapy,
High flow
CPAP
Ventilation
NG, IV fluids if needed
Bronchiolitis presentation?
Couple of days of coryzal illness (longer than VIW)
Irritability, feeding difficulty
Cough, wheeze (expiratory) , crackles
Croup (upper airway obstruction) presentation?
Stridor
Barking cough
Hoarse voice
<38.5
don’t look toxic
Persistent upper airway obstruction (with stridor) most likely caused by what?
Laryngomalacia
What does laryngomalacia look like?
Omega shaped deformity of the epiglottis
most common cause of croup?
Parainfluenza virus
Croup management?
Don’t look at throat
Keep child calm
Oxygen and hydration
Dexamethasone steroid orally
Nebulised budesonide
Nebulised adrenaline
Intubate
Croup management?
Don’t look at throat
Keep child calm
Oxygen and hydration
Dexamethasone steroid orally
Nebulised budesonide
Nebulised adrenaline
Intubate
How can you (maybe) differentiate between viral and bacterial pneumonia?
Viral:
- more common in infants
- fever <38.5
Bacterial
- Fever >38.5
- Any age
- Resp rate >50
Presentation of pneumonia in children?
Fever
Tachopneoa
Cough
O/E:
- Dullness
- Reduced air entry
- Bronchial breathing
Investigations and management of kids with pneumonia?
Investigations:
- sputum analysis
- bloods
- blood culture
- CXR
- URINE PNEUMOCOCCAL ANTIGEN
Tx
- Oxygen
- Physio if collapse (of lung)
- Abx
If empyema then chest drain and urokinase enzyme
What is whooping cough, how does it present, how do you treat?
Highly contagious infection caused by pertussis
Week of coryzal symptoms then spasmodic cough with inspiratory whoop
Viral induced wheeze in children treatment?
Bronchodilators and steroids as well as supportive care.
Recurrent cough Ddx in children?
Series of respiratory tract infections
Asthma
CF
Reflux
Foreign body
TB
Habit
How many Obstructive sleep apnoea present in children?
Loud snoring
Witnessed apnoeas (stopped breathing)
Restless and disturbed sleep
Can cause daytime sleepiness leading to learning and behavioural problems
What is obstructive sleep apnoea normally caused by?
Adenotonsillar hypertrophy
Key investigation for sleep apnoea on children?
overnight pulse oximetry, however normal oximetry doesn’t exclude the condition and may have to do polysomnography
General Ddx for vomiting?
Reflux
Feeding issues
Infection
- Gastro/elsewhere
Intolerances
Obstruction
Metabolism
CAH
Renal failure
Appendicitis
Raised ICP
Coeliac disease
Migraine
Anorexia
Pregnancy
Testicular torsion
When does reflux (GORD) normally resolve in children?
By 12 months, normally by 6 months.
Complications of reflux in children?
Failure to thrive
Oesophagitis
- haemetemesis
- Iron deficiency anaemia
Pulmonary aspiration
Apparent life-threatening events
What is colic in kids?
Colic is a frequent crying in a baby who appears to be otherwise healthy and well fed. It occurs in 1 in 5 babies and affects male and female infants equally
Usually begins within the first few weeks of life resolving by 4-6 months. Crying tends to be worst in the late afternoon or evening and usually lasts for several hours.
During episodes the baby’s face may become flushed, and they may clench their fists, draw their knees up to their tummy, or arch their back.
Linked to reflux and migraines.
What causes reflux in babies?
Liquid diet
Immature lower oesophageal sphincter
Predominantly lying down
Short distance from mouth to stomach
Reflux management?
Conservative (depends on severity)
- Don’t worry
- Sit up
- Thicken feed.
Medical:
- Ranitidine (5HT anatag)
- PPI
How does cows milk protein allergy present?
Atopic eczema
Gastro-Oesophageal Reflux Disease
Chronic Gastro-intestinal symptoms, including chronic constipation
How would pyloric stenosis present?
Around 3 weeks
Worsening vomits with feeds, progressing to projectile vomiting, non-bilious with undigested milk.
Mallory-Weiss tear may lead to blood in vomit.
Weight loss, dehydration, reduced urine output and even shock. Infants may become jaundiced; resolves with treatment.
Hypochloraemic Metabolic Alkalosis
Persistent vomiting leads to a loss of hydrogen and chloride ions, leading to a Hypochloraemic Metabolic Alkalosis. There is increased reabsorption of Bicarbonate in the kidneys whilst passive Na reabsorption is hindered by chloride loss; active reabsorption is done at the expense of Potassium ions which, along with vomiting losses, leads to Hypokalaemia (this may initially be concealed due to a potassium shift from extracellular fluids).
Pyloric stenosis management?
Initial management includes placing the child nil by mouth and correcting the dehydration and electrolyte imbalance. The child can then go to surgery for a Pyloromyotomy - a laparoscopic procedure where the thickened muscle is cut to allow widening of the stomach outlet. The infant is usually able to feed within 6 hours and makes a full recovery with no ill effects.
Test for cows milk protein allergy?
- Take it away for 3-4 weeks
- AND
- GIVE IT BACK!!!!!!!!!!!
Overflow constipation management?
Movicol first line with escalating regime
Then add stimulant laxative
Add lactulose if hard stools
continue treatment for a few weeks after regular habit established and taper down slowly
What do you need to check with constipation?
Leg weakness/delay gross motor development (spinal abnormality)
Constipation from birth
Faltering growth
What investigations are available for coeliac disease, when would you perform them?
TTG and total IgA are first line serological tests. MUST be done whilst the patient is eating gluten.
Definitive diagnosis is a biopsy - needs to be done whilst on gluten - shows subtotal villous atrophy
Features of crohns disease?
Diarrhoea
Abdo pain
Oral ulceration
Rectal bleeding
Anal tags, fissure and fistulae
Weight loss
Poor growth and delayed puberty
Features of ulcerative colitis?
RECTAL BLEEDING
PASSAGE OF MUCUS
DIARRHOEA
URGENCY
ABDOMINAL PAIN
POOR GROWTH &DELAYED
PUBERTY
Manifestations of IBD outside the bowel?
JOINTS: Arthritis can occur, leading to painful swollen
joints e.g.knees hips ,ankles.
SKIN : “Erythema Nodosum”- red painful lumps on the
lower legs ,shins and occasionally the arms.
EYES : “Iritis” - inflammation of the iris. “Episcleritis”-
inflammation of the eyeball.
LIVER : Inflammation and scarring of the liver and bile
ducts.
Difference pathophysiologically between Crohns and UC?
UC:
- Affects colon only
- Diffuse progression from rectum upwards
- Mucosal involvement
- Histology – crypt abscesses / pseudopoyps
Crohns
- Can affect anywhere along the GI tract – pan enteric
- Skip lesion
- Transmural involvement
- Histology – granulomas/ cobble stone appearances
Confirmation of IBD diagnosis?
Upper and lower colonoscopy with biopsy.
Big risk in UC?
Toxic megacolon
Toxic megacolon management?
- IV fluids and NBM
- IV antibiotics
- IV steroids
- Blood transfusion if necessary
- Urgent surgical review (N.B. emergency colectomy has a mortality of 10%, but perforation has a mortality of 33%)
What is toxic megacolon?
Toxic megacolon is an acute form of colonic distension.
It is characterized by a very dilated colon (megacolon), accompanied by abdominal distension (bloating), and sometimes fever, abdominal pain, or shock.
What is toddler’s diarrhoea?
Toddler’s diarrhoea typically occurs in the second year of life and is associated with undigested food such as peas and carrots in the stools. The child is well and growing normally. It is thought to relate to a rapid intestinal transit time. It resolves by the age of 4 years.
What is intussusception?
Describes the invagination of the proximal bowel into the distal segment - commonly ileum into caecum at the ileocaecal valve
When does intussusception usually occur?
3 months to 2 years
Complications in intussusception?
Venous obstruction causing engorgement, bleeding and bowel perforation
Presentation of intussusception?
Paroxysmal severe colicky pain and pallor
May refuse feeds, vomit - may be bilious
Redcurrent jelly stool
Sausage shaped mass in abdomen
Management of intussusception?
IV fluid resus
Rectal air insufflation - by a radiologist
Surgically if the air fails
Abdominal migraine presentation?
Periodic pain, can be in the midline assoc. with vomiting and facial pallor
Headache is normally present too, but not necessarily
Gastroenteritis pathogens?
Most common - rotavirus (up to 60%)
Others:
- adenovirus
- norovirus
- coronavirus
- astrovirus
Bacteria are less common, may have blood in the stool
- Campylobacter jejuni
- Shigella and salmonella - blood and pus
- Cholera and e. coli - profuse rapidly dehydrating
groups of children at risk of dehydration with respect to gastroenteritis?
<6 months or born with low birth weight
> 6 diarrhoeal stools in prev. 24 hrs
> 3 vomits in previous 24 hours
Not tolerating fluids
Malnourished
Signs of dehydration in children?
Decreased conscious level
Sunken fontanelle
Dry mucous membranes
Sunken eyes
Tckypnoea
prolonged cap refill
Tchycardia
Pale skin
Hypotension
Weight loss
Cold extremities
Reduced tissue turgor
Management of gastroenteritis?
May get stool sample if in shock
Hypernatraemic dehydration is hard to manage:
- Oral rehydration solution
IV fluids (if shock) - need to measure plasma sodium, and if hypernatraemic then slow infusion, over 48 hours.
Antibiotics if there is sepsis
Gastroenteritis presentation?
D & V, may be evidence of infection such as contact with an infected person or travel abroad.
What amount of ORS do you give in management of dehydration?
50 ml/kg over 4 hours
How do you correct fluid deficit (in IV fluids) in kids - not maintenance?
100ml/kg if shocked
50ml/kg if not shocked
0.9% saline or normal saline with 5% dex
most common pathogens causing UTIs in children?
E coli - almost all
Klebsiella
Proteus species
Risk factors for UTIs?
Constipation
- incomplete voiding, urinary stasis
Voiding dysfuntion
- Sit on heel in girls, pinch penis in boys, so that they don’t have to go to the loo, may not empty properly urinary stasis again.
Anatomic anomoly
- PUJ (Pelviureteric junction obstruction)
- others
Neuropathic
- spina bifida
1 year fairly common - not potty trained, need basic hygiene advice perhaps
> 6 months more common in girls
<6 month more common in boys
Presentation of UTI?
Preverbal (<3 months)
- fever
- vomiting
- lethargy
- irritability
- poor feeding
- tenderness
> 3 months (if verbal )
- dysuria
- frequency
- abdo/loin tenderness
- dysfunctional voiding
Management of UTI?
Really ill - pyelonephritis
- Analgesic
- fluids
- IV abx
Well child
- Abx
- ?analgesia
Types of enuresis
Primary nocturnal enuresis
Daytime enuresis (normally with night)
Secondary enuresis
What is daytime enuresis?
lack of bladder control during the day in a child over 3-55 years
What can cause daytime enuresis?
Lack of attention to bladder sensation, normal or developmental or psychogenic
Detrusor instability
Bladder neck weakness
Neuropathic bladder
UTI
Constipation
Ectopic ureter
Management (inc. investigations in daytime enuresis?
Examination to rule out neuropathic bladder
Urine sample for microscopy to exclude UTI
USS of bladder
urodynamics
Can use star reward charts, bladder training and pelvic floor exercises if neuropathic bladder or anomaly excluded
What is secondary enuresis?
Loss of previously achieved urinary continence
Causes of secondary enuresis?
Emotional upset (most common)
UTI
Polyuria from diabetes or CKD
management of secondary enuresis (inc. management)?
Test urine sample for infection, glycosuria and proteinuria
USS of renal tract
Assess urinary osmolality of early morning sample to assess urinary concentrating ability
Causes of proteinuria?
Orthostatic proteinuria
- when standing up, will resolve
Glomerular abnormalities
Increased glomerular filtration pressure
Reduced renal mass
HTN
Tubular proteinuria
Clinical features of nephrotic syndrome?
Protein in urine so low plasma albumin resulting in oedema (periorbital, scrotal, vulval, leg and ankle)
May have ascites
May have breathing difficulties resulting in breathlessness
Complications of nephrotic syndrome?
infection
- peritonitis
- cellulitis
Hypovolaemia
VTE - PE
Acute renal failure
protein depletion
Most common cause of nephrotic syndrome in kids
Minimal change glomerulonephritis. Epithelial cell foot processes fuse
What is steroid sensitive nephrotic syndrome? What are the features that suggest this?
When the nephrotic syndrome is treatable with steroids, normally:
Between 1-10years No macroscopic haematuria Normal BP Normal complement Normal renal function
Management of nephrotic syndrome
Normally try steroids
Try to use steroid sparing agents as many will relapse and steroid over use can cause all kinds of shit
Prevent complications:
Maintain hydration
- Don’t restrict fluids (unless must)
- IV albumin
- Caution with diuretics
Minimise oedema
- Low salt
Prevent infection
- Imms
If they have lots of relapses then will need steroids long term
If the urine is brown or red what is the haematuria likely caused by?
Brown - glomerular
Red - LUTI
Most common cause of haematuria?
UTI
Causes of acute nephritis in children?
Post infective (strep) - doesn't last long, prognosis is good
Vasculitis e.g. HSP
IgA nephropathy
Features of acute nephritis in children?
Decreased urine output and volume overload
Hypertension
Oedema
Haematuria and proteinuria
Management of acute nephritis in children?
Manage fluid and electrolyte balance, diuretics when necessary.
Definition of HTN in kids?
Blood pressure above the 95th centile for height, age and sex
Presentation of HTN in kids?
Vomiting, headaches facial palsy, retinopathy, convulsions or proteinuria.
Causes of hypertension in kids?
Renal
Polycystic kidneys, tumours, parenchymal disease
Coarctation of the aorta
Catecholamine excess
- Phaeochromocytoma
Endocrine
- CAH
- Cushings
- Hyperthyroidism
Essential HTN
Meningococcal septicaemia most likely causative organism?
Neisseria meningitidis - gram negative
Presentation of bacterial meningitis and of meningococcal septicaemia?
Different conditions, bacterial meningitis, does often present with sepsis though:
Bacterial meningitis
- Fever
- Headache
- Stiff neck, bulging fontanelle, photophobia
- Altered mental state
- Non-blanching rash
Shock
Seizures, focal neurological signs
Meningococcal septicaemia without meningitis probably wouldn’t have the classic stiff neck/photophobia/bulging fontanelle
Red flags of SOL in kids?
Visual field deficits
Cranial nerve abnormalities
Headache worse on lying down, wakes kid up associated with morning nausea or vomiting
Abnormal gait Torticolitis (tilting of the head) Growth failure papilloedema (late) Cranial bruits Personality change
Three main divisions of headache in kids?
Primary
- Migraine
- Tension
- Cluster
- Cough/exertional
Secondary
- Due to SOL or raised ICP
Trigeminal or other neuralgia
What is the most common migraine in kids?
Migraine without aura (90%)
Features of migraine without aura in kids? How does migraine with aura differ?
1-72hrs
Commonly bilateral, may be unilateral though
Pulsatile, normally over temporal or frontal area
Photophobia/phonophobia
GI symptoms such as nausea, vomiting, abdo pain
Aggravated by physical activity
Aura obviously also has aura, this may be visual sensory or motor and positive or negative symptoms
Management of headaches (primary)?
Rescue
- Analgesia (paracetamol and NSAIDS)
- Antiemetics
- 5HT agonists
Prophylaxis
- Pizofen (5HT antagonists)
- Beta-blockers
- Sodium channel blockers
Psychosocial support
Most likely pathogen to cause encephalitis in children?
Herpes simplex - it is usually viral
Presentation of encephalitis?
Most patients with viral encephalitis present with the symptoms of meningitis (fever, headache, neck stiffness, vomiting) followed by altered consciousness, convulsions, and sometimes focal neurological signs, signs of raised intracranial pressure, or psychiatric symptoms. There may be an association with a history of infection elsewhere in the body.
BDZ in status epilepticus in children?
Rectal diazepam
How do you calculate maintenance fluids in kids?
first 10kg - 1 Litre (100ml/kg)
10-20kg - 500ml (50ml/kg)
20-80kg 20ml per kg
What is neurofibromatosis type 1 and type 2?
Genetic disorder causing lesions to the skin, nervous system and skeleton
Type 1
- More common
- Genetic inheritance - autosomal dominant
- cafe au lait spots
- increased risk of developing benign and malignant tumours
Type 2
- less common
- presents in adolescence
- Central CNS tumours e.g. vestibular schwannoma
Diagnostic criteria for NF1 diagnosis?
Need two or more of these:
Six or more cafe au lait spots >5mm (>15mm after puberty)
Axillary freckles
> 1 neurofibroma
optic glioma
Lisch nodule (Iris haemotoma)
Sphenoid dysplasia
First degree realtive
What is tuberous sclerosis?
genetic disorder resulting in the growth of benign tunours around the body, may have neurocutaneous and systemic lesions
What is haemophilia, what are the types, inheritance pattern and features?
Haemopilia A and B, X-linked recessive disorders
In A there is factor 8 deficiency and is more common
B is factor 9 deficiency, less common
2/3rd of patients have family history, 1/3rd don’t as can be a de novo mutation.
Can range from mild to severe deficiency:
- severe would have recurrent spontaneous bleeding into joints and muscles - would lead to bad arthritis
normally present <1 year, can lead to intraventricular haemorrhage in neonates and bleeding episodes (into joints and stuff when starting to walk)
Management of Haemophilia?
Replace the lost factor (8 in A, 9 in B), prophylactic therapy normally begins at 2-3 years.
What is ITP?
Immune thrombocytopenic purpura
- lack of circulating platelets leading to bleeding
Normally occurs in children following viral infection or immunisation and is self-limiting in 6-8 weeks.
Can cause bleeding and purpura
Management of sickle cell disease?
Full imms and antibiotic prophylaxis (due to increased risk of infection) throughout childhood.
Supportive in acute event
Hydroxyurea may predict in children who are struggling
Clinical manifestations of Sickle cell disease?
Anaemia, chronic and acute
Infection
Painful crisis
Priaprism - needs to be treated promptly
Splenomegaly
Other long term issues
Main causes of iron deficiency in children?
Main:
- Inadequate intake (may be because still breast fed >6 months)
- Malabsorption
- Blood loss
Other disorders such as:
- Sickle cell
- Thalassaemias
- G6PD deficiency, spherocitosis
- Haemophilias
Most common cancer in children? how o you differentiate ALL and AML
ALL
AML and ALL are diferentiated morphologically
Leukaemia presentation, features and management?
2-5 years, normally insidious over a few weeks, but can be v rapid.
General:
Malaise, anorexia
Bone marrow infiltration
- Anaemia - pallor, lethargy
- Netropaenia - infection
- thrombocytopaenia - bruising, petechiae
- bone pain
hepatosplenomegaly, lymphadenopathy
Other:
- CNS - headache. vomiting
Testicular enlargement
Types of lymphoma in childhood?
Hodkin and non-hodgkin
hodgkin is painless lymphadenopathy, non is mediastinal mass, prognosis is good with combined chemotherapy
What is CVID?
B cell issue
umbrella diagnosis in that it encompasses a group of genetic disorders which result primarily in hypogammaglobulinaemia or failure of antibody production
Presents with recurrent infection
What is anaphylaxis and how do you manage it?
Severe rapid onset immunological reaction that threatens ABC
Normally IgE mediated reaction to food allergy, asthma is a. risk factor.
ABCDE approach
High flow O2
Adrenaline IM:
>12 500mg (0.5ml of 1 in 1000)
6-12 0.3ml
<6 0.15ml
Fluid and steroids
Orbital cellutitis and periorbital cellulitis differences and management?
Periorbital cellulitis is not in the obit yet, it presents with erythema, tenderness and oedema of the eyelid, it may follow local trauma. Needs prompt Iv antibiotics to prevent it spreading to the orbit and becoming orbital cellulitis.
Orbital cellulitis is an emergency, there is proptosis, painful and limited ocular movement and reduced visual acuity. It may be complicated by meningitis, cavernous sinus thrombosis or abscess formation.
Chickenpox presentation and management?
Varicella zoster infection, normally happens by 5 years
Pyrexia
Headache and malaise
Vesicles for 3-5 days - papule, vesicle, pustule and crust
Can be very itchy, less so in younger kids
Not a problem in younger kids but much more of a problem in older adults if they haven’t had it. Just general supportive management, can use antihistamine and paracetamol
What is slapped cheek disease? Presentation and management?
Fith disease or parovirus infection or erythema infectiosum.
Coryzal symptoms at first
then free from symptoms for 7-10 days
Then slapped cheek rash
About 1-4 days after the facial rash appears, an erythematous macular rash develops on the extremities, mainly on the extensor surfaces. disappears after 3-21 days.
What is hand foot and mouth disease, how does it present and how is it managed?
Viral illness which commonly causes lesions involving the mouth, hands and feet.
Can affect other areas such as genitalia and buttocks.
Coxsackievirus A16 (CA16) and enterovirus 71 (EV71)
Prodrome such as low grade fever, malaise and loss of apetite
Lesions begin in mouth, macular that progress to ulcers, then skin lesions
Self-limiting
Management is supportive, assure it is not foot and mouth disease in animals. Paracetamol, soft diet and analgesics.
May rarely have complications such as meningitis or encephalitis, infection from open sores, cardiorespiratory failure.
What is JIA?
Juvenile idiopathic arthritis (JIA) is defined as joint inflammation presenting in children under the age of 16 years and persisting for at least six weeks, with other causes excluded.
Lots of different types, can cause fever, is not infective in origin.
What is malrotation and volvulus?
A volvulus is a complete twisting of a loop of intestine around its mesenteric attachment site. This can occur at various locations of the gastrointestinal (GI) tract, including the stomach, small intestine, caecum, transverse colon and sigmoid colon.
Midgut malrotation refers to twisting of the entire midgut about the axis of the superior mesenteric artery (SMA).
Bilious vomiting is key, malrotation may lead to volvulus, which is pretty bad
Volvulus may present with abdo mass, distention and pain - urgent care needed, surgical correction.
Appendicitis presentation?
RIF (may start periumbilical) pain, worse on movement is key, nausea vomiting and anorexia.
Rebound tenderness
What causes roseola infantum?
Human Herpes virus 6
Most likely cause of nephrotic syndrome in children/young adults?
Minimal change disease
