1
Q
What is biliary atresia
A
= a congenital condition where a section of the bile duct is either narrowed or absent.
- There is progressive inflammation & fibrosis of the extrahepatic ducts.
- This leads to cholestasis, liver damage, and liver failure.
2
Q
How does biliary atresia present?
A
History:
- Symptoms may not present until after the first week of life.
- Persistent neonatal jaundice (>14days in term, formula fed, >21days in premature, breast fed)
- Pale stools
- Dark urine
Examination:
- Jaundice
- Findings in >3 month old:
- Hepatosplenomegaly
- Ascites
- Failure to thrive →malabsorption of fats
3
Q
What investigations are done for suspected biliary atresia?
A
Laboratory:
- Newborn blood spot screening → rules of CF
- LFTs → raised conjugated bilirubin, raised GGT
- Physiological jaundice would cause an unconjugated hyperbilirubinaemia
Imaging:
- Ultrasound → first line
- Percutaneous liver biopsy → preferred diagnostic investigation
4
Q
How is biliary atresia managed?
A
Surgical Management:
- Kasai portoenterostomy → removal of the damaged bile ducts & replacement with a loop of intestine to allow bile to flow from the liver to the intestine.
- Not curative, usually the child will need a liver transplant at some point.
- Do within the first 45 days of life.
Liver transplant:
- If surgery unsuccessful, child is failing to thrive, end-stage liver failure
5
Q
How does pyloric stenosis present?
A
Presents within the first few weeks of life.
- Projectile vomiting → non-bilious
- Weight loss/failure to thrive
- Constipation
- Lethargy
- Firm, round mass (feels like an olive) can sometimes be felt in the upper abdomen due to the hypertrophic muscle of the pylorus.
- Visible gastric peristalsis
6
Q
How is pyloric stenosis diagnosed & managed?
A
Diagnosis is made using an abdominal ultrasound.
Laparoscopic pyloromyotomy → an incision is made in the smooth muscle of the pylorus to widen the canal allowing the food to pass from the stomach to the duodenum as normal.
- Excellent prognosis with this.
7
Q
What are the risk factors for GORD in babies?
A
- Premature birth → key
- Parental history of GORD
- Obesity
- Hiatus hernia
- Cerebral palsy
8
Q
How does GORD present in children?
A
If <1yrs old:
- Effortless posseting
- Early onset (within first few weeks)
- Time taken to feed >30mins
- Distressed behaviour during meal times → crying while feeding, refusing to feed
- Hoarseness and/or chronic cough
- Faltering growth
If >1yr old:
- Retrosternal pain
- Epigastric pain
- Nocturnal cough
9
Q
How is GORD managed in children?
A
Supportive:
- Small, frequent meals
- Burping regularly to help milk settle
- Not over-feeding
- Keep baby upright after feeding
- Tilt cot mattress
If more problematic:
- Gaviscon mixed with feeds - can be constipating
- Thickened milk or formula
- PPIs (omeprazole)
10
Q
What are some red flags of constipation in childhood?
A
- Not passing meconium with 48hrs of birth → CF, Hirschsprung’s disease
- Neurological signs of lower limbs → cerebral palsy, spinal cord lesion
- Vomiting → intestinal obstruction, Hirschsprung’s disease
- Ribbon stool → anal stenosis
- Abnormal anus → anal stenosis, IBD, sexual abuse
- Failure to thrive → coeliac disease, hypothyroidism, safeguarding issue
- Abnormal lower back or buttocks → spina bifida, spinal cord lesion, sacral agenesis
- Acute severe abdominal pain & bloating → obstruction or intussusception
11
Q
How is childhood constipation managed?
A
- Treatment of constipation can be a prolonged process, potentially taking months
- Correct any reversible contributing factors → high fibre diet & good hydration important
- Milk is constipating, reduce this & increase water intake.
- Start laxatives → movicol first line, lactulose second line (stool softeners)
- Continue long term, slowly wean off as the child develops a regular bowel habit.
- Faecal impactation may require a disimpaction regimen → high doses of laxatives at first
- Encourage & praise child visiting the toilet → scheduling visits (sit them on the toilet 30mins after a meal), a bowel diary, star charts
12
Q
What is Cows Milk Protein Allergy? What are the two types?
A
= A hypersensitivity to the protein in cow’s milk, which can be IgE or non-IgE mediated.
- IgE mediated → rapid reaction to cow’s milk (occuring within 2hrs of ingestion)
- Non-IgE mediated → reactions occur slowly over several days.
13
Q
How does Cows Milk Protein Allergy present?
A
- Effortless posseting
- Blood in stool
- Abdominal pain
- Diarrhoea
- Urticarial rash (hives)
- Angio-oedema
- Cough or wheeze
- Sneezing
- Watery eyes
- Eczema, dry skin
- Anaphylaxis → only in severe cases.
14
Q
How is Cows Milk Protein Allergy managed?
A
Avoiding cow’s milk should fully resolve symptoms, but it will take several weeks to do this washout:
- Breast feeding → mothers should avoid dairy products
- Formula → replace with hydrolysed formulas (protein is broken down)
Most children will outgrow CMPA by 3 years old, often earlier.
Every 6 months, children can be tried on the first step of the milk ladder, and then slowly progress up the ladder until they develop symptoms.
15
Q
What age is intussusception most common?
A
Most common in children aged 6 months - 2 years.
16
Q
What causes intussusception?
A
- No clear cause in most cases
- May be associated with preceding/concurrent viral infection
- Pathological lead points → an abnormal area of bowel which is caught & pulled by peristalsis.
- Meckel diverticulum
- Intestinal polyps
- Cystic fibrosis
- Henoch-Schonlein Purpura
17
Q
How does intussception present?
A
Typical triad of symptoms:
- Severe, colicky abdominal pain → intermittent, episodes where the child is inconsolable & draws their knees up to their chest
- Vomiting → becomes bilious in later stages when bowel obstruction occurs
- Redcurrent jelly stool → a late feature that occurs when ischaemic mucosal tissue is sloughed off & excreted in the stool mixed with blood & mucus.
Other symptoms:
- Pale, lethargic, unwell child → worsens as dehydration worsens.
- RUQ mass on palpation, sausage-shaped
18
Q
What investigation is gold-standard in suspected intussusception, and what does it show?
A
Abdominal USS → gold standard
- Shows a target sign
19
Q
How is intussusception managed?
A
Acute Management:
- Prompt fluid resuscitation → failure to do so is one of the main causes of mortality
- Analgesia
- Insertion of NG tube to decompress the stomach
- NBM
Definitive Management:
- Non-Surgical → therapeutic enemas are first line
- Contrast, air, or water are pumped into the colon through a foley catheter to force the folded bowel out of the bowel & into normal position.
- Contraindicated if there is evidence of shock, perforation, or peritonitis.
- Surgical
- Where enemas are unsuccessful or contraindicated, to manually reduce the intussusception.
- If the bowel become gangrenous or the bowel is perforated, then surgical resection is required.
20
Q
How does appendicitis present?
A
- Abdominal pain
- Central initially, moves down to the RIF within the first 24hrs & remains localised here.
- Tenderness at McBurney’s point (1/3 of distance from ASIS to umbilicus)
- Rebound tenderness in RIF → increased pain when suddenly releasing the pressure of deep palpation.
- Indicate peritonitis
- Nausea & vomiting
- Low-grade fever
- Anorexia
- Rovsing’s sign → palpation of the LIF causes pain in the RIF
21
Q
If not detected on the heel-prick test, how may a child present with cystic fibrosis?
A
- Meconium ileus - not passing meconium within 24hrs, abdominal distention, vomiting
- Recurrent LRTIs, chronic cough, thick sputum production
- Failure to thrive
- Pancreatitis
- Steatorrhea
- Abdominal pain & bloating
22
Q
What is the gold standard investigation for cystic fibrosis?
A
Sweat test -> will show a raised chloride concentration
23
Q
What respiratory management is given to people with CF?
A
- Chest physiotherapy - to help clear mucus
- Antibiotics
- Prophylactic (flucloxacillin) & treatment
- Oral, nebulised, intravenous
- Mucolytic drugs
- DNAse - breaks down the DNA in neutrophils which makes the mucus less viscous
- Hypertonic saline - helps to rehydrate the mucus - makes it easier to clear.
- Vaccinations - pneumococcal, influenza, varicella
- Lung transplant - not many are done
24
Q
What GI management is given to people with CF?
A
- High calorie diet
- For malabsorption, increased respiratory effort, coughing, and infections
- Creon tablets
- To digest fats in patients with exocrine insufficiency
- Liver transplant - if failure
25
How does CF affect the respiratory system? What are the clinical complications of this?
- Thickened secretions reduce mucociliary clearance from bronchi & increase salt concentration leads to impaired bacterial defences.
- Bacterial colonisation & lung inflammation increases.
- Causes a repeated cycle of infections causing sticky mucous & vice-versa → leads to bronchiectasis.
- Thickened secretions in sinuses lead to recurrent infection & subsequent nasal polyps due to chronic inflammation.
- Respiratory complications:
- Recurrent infections
- Bronchiectasis
- Respiratory failure
- Pneumothorax
- Haemoptysis
- Nasal polyps
- Chronic sinusitis
- Allergic bronchopulmonary aspergillosis (occasionally seen in asthma, but mostly a CF complication)
26
How does CF affect the male fertility system?
- Thick secretions during development lead to Congenital Bilateral Absence of the Vas Deferens
- Sperm is produced, but cannot reach the semen, so fertility treatments can support people with CF having children.
27
How is DKA managed in children?
- Correct dehydration
- Correct evenly over 48hrs to reduce the risk of cerebral oedema
- Only give a fluid bolus if severe
- Add potassium & monitor closely - due to prior lack of insulin (which normally drives K+ into cells), treatment causes the risk of hypokalaemia.
- Fixed-rate insulin transfusion
- Add IV dextrose once blood glucose falls below 14mmol/L
- Antibiotics if underlying infection
28
What is the criteria for a diagnosis of DKA?
To diagnose DKA you require:
- ***Hyperglycaemia*** (blood glucose > 11 mmol/l)
- ***Ketosis*** (blood ketones > 3 mmol/l)
- ***Acidosis*** (pH < 7.3)
29
What fluids are given in paediatrics for maintenance, replacement, and resuscitation?
Maintenance & replacement -> 0.9% NaCl + 4% glucose
Resus -> 0.9% NaCl
30
How is the volume of maintenance fluids in children determined?
Based on the child's weight.
First 10kg weight - 100ml/kg/day
Second 10kg - 50ml/kg/d
For every kg above 20kg - 20ml/k/day
31
How is a fluid deficit & total fluid requirement calculated in paediatric replacement fluid prescribing?
- Clinical signs of dehydration are only detectable when the patient is 2.5-5% dehydrated.
- Therefore if the child has symptoms/signs of dehydration, but no red flags → 5% dehydrated
- If any red flags of dehydration or child is clinically shocked → 10% dehydration. If shocked, treat as below.
- Calculating a fluid deficit → % dehydration x weight (kg) x 10
- Total fluid requirement → maintenance fluids + fluid deficit
32
What volume of resuscitation fluid is given in paediatrics if the patient is shocked?
A standard bolus of 10mL/kg over 10 minutes.
33
What is Henoch-Schonlein Purpura & how does it present?
= an IgA vasculitis that presents with a purpuric rash affecting the lower limbs & buttocks in children.
- Inflammation occurs due to IgA deposits in the blood vessels of affected organs → skin, kidneys, GI tract.
**History:**
- Purpuric rash
- Due to inflammation & leaking of blood from small blood vessels under the skin, forming purpura.
- Typically purple & palpable under the skin.
- Typically start on the legs & spread to buttocks → can also affect the trunk & arms.
- Abdominal pain
- Associated with nausea & vomiting
- Bloody diarrhoea may be seen
- Joint pain
- Hips, knees & ankles → can be associated with swelling
- Kidney involvement
- Frothy urine
- Haematuria
- Fever → low-grade
- Preceding upper respiratory tract infection/gastroenteritis
34
How is Henoch-Schonlein Purpura managed?
Most children with HSP have a complete spontaneous recovery within weeks to months, but may require supportive care:
- Analgesia → paracetamol
- Rest & good hydration
- Prednisolone → for severe pain
Follow up for renal involvement & blood pressure is important
35
How is croup managed?
Croup is a self-limiting illness. Management aims to reduce severity & avoid the need for intubation.
- Calm reassurance
- Mild Croup → oral dexamethasone as a single dose, send home with safety netting
- Moderate croup → oral dex, period of observation, safety netting
- Severe croup → nebulised adrenaline (act immediately, allowing steroids to take action), oxygen if hypoxic, oral/IV dex, monitoring
36
What is Kawasaki disease? When should a diagnosis be considered?
= acute, self-limiting systemic, medium-sized vessel vasculitis which typically affects children under 5yrs old.
A diagnosis of KD should be considered in any child <5 years old, with a persistent high fever (39) for more than 5 days.
37
How does Kawasaki Disease present?
A diagnosis of KD should be considered in any child <5 years old, with a persistent high fever (39) for more than 5 days.
**Other features:**
- Widespread erythematous maculopapular rash
- Desquamation (skin peeling) on the palms & soles
- Strawberry tongue
- Cracked lips
- Cervical lymphadenopathy
- Bilateral conjunctivitis → no exudate
**Disease Course:**
- Acute phase → 1-2 weeks, child is more unwell with rash/fever/lymphadenopathy
- Subacute phase → 2-4 weeks, acute symptoms settle, desquamation/arthralgia/coronary artery aneurysms
- Convalescent phase → 4-8 weeks, recovery
38
How is Kawasaki Disease managed?
- admit. Early treatment aims to prevent coronary artery aneurysms.
- High dose aspirin until apyrexic for 48hrs, then low dose for 6 weeks
- Infusion of IV immunoglobulin for first 10 days
- Serial echos to monitor for aneurysms
