Paediatrics Flashcards
What is biliary atresia
= 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.
How does biliary atresia present?
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
What investigations are done for suspected biliary atresia?
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
How is biliary atresia managed?
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
How does pyloric stenosis present?
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
How is pyloric stenosis diagnosed & managed?
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.
What are the risk factors for GORD in babies?
- Premature birth → key
- Parental history of GORD
- Obesity
- Hiatus hernia
- Cerebral palsy
How does GORD present in children?
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
How is GORD managed in children?
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)
What are some red flags of constipation in childhood?
- 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
How is childhood constipation managed?
- 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
What is Cows Milk Protein Allergy? What are the two types?
= 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.
How does Cows Milk Protein Allergy present?
- 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.
How is Cows Milk Protein Allergy managed?
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.
What age is intussusception most common?
Most common in children aged 6 months - 2 years.