Liver disease in children Q&A

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Averil Tam
Flashcards by Averil Tam, updated more than 1 year ago
Averil Tam
Created by Averil Tam over 6 years ago
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TRUE/FALSE? 1A. The definition of conjugated hyperbilirubinaemia is a direct bilirubin fraction of greater than 30% or >30mmol/l. False. The definition of conjugated hyperbilirubinaemia is a direct bilirubin fraction of greater than 20% or >30mmol/l.
TRUE/FALSE? 1B. A baby is said to have prolonged jaundice if the jaundice persists more than 1 week. False. A baby is said to have prolonged jaundice if the jaundice persist more than 2 weeks.
TRUE/FALSE? 1C. The best test to check for synthetic liver function in a child with abnormal liver function tests is the internal normalised ratio (INR). True
TRUE/FALSE? 1D. Conjugated hyperbilirubinaemia in a neonate is always pathological. True
TRUE/FALSE? 1E. Urinary tract infections can cause unconjugated or conjugated jaundice in a neonate. True
2A. What are some common causes of unconjugated hyperbilirubinemia at day 4 of life? [Excess bilirubin production] Haemolysis (eg. ABO incompatibility or Rh disease, G6PD deficiency, hereditary spherocytosis and thalassaemias), extravasated blood (eg. cephalhaematoma), polycythemia (eg. infant of a diabetic mother), sepsis
2B. What are some common causes of unconjugated hyperbilirubinemia at day 4 of life? [Reduced bilirubin excretion] Hypothyroidism, dehydration, delayed passage of meconium, familial, breast milk jaundice (diagnosis of exclusion) and Crigler-Najjar syndrome (very rare)
3. What is the most important question when faced with a jaundiced infant at 3 weeks of age? A. Type of feeding – breast or formula? B. Is the baby gaining weight appropriately? C. Is the jaundice conjugated or unconjugated? D. Family history of neonatal jaundice? C: Establishing if a jaundiced infant at 3 weeks of age has a conjugated hyperbilirubinaemia is THE most important issue to clarify. The presence of a conjugated hyperbilirubinaemia is pathological and necessitates immediate referral for investigation, particularly for biliary atresia.
4. What are some of the causes of conjugated hyperbilirubinaemia in neonates? Sepsis, metabolic disorders, biliary atresia, choledocal cysts or biliary obstruction from any other cause.
5. A 7 year old girl presents with acute onset of jaundice. What is your approach? [History] History of contact – travel, family, school Past and family history of jaundice, gallstones, liver disease Ingestion of uncooked shell fish (Hepatitis A) Recent injections, surgery, blood products (Hepatitis B, C) Drug ingestion, medication (eg. paracetamol) Recent ingestion of wild mushrooms
5. A 7 year old girl presents with acute onset of jaundice. What is your approach? [Examination] Always check for signs of chronic liver disease: leukonychia, spider naevi, scratch marks, jaundice, bruising, palmar erythema, caput medusae, growth failure Encephalopathy (flapping tremor) Hepatomegaly and/or splenomegaly Slit lamp examination of the eyes (ophthal review) - Kayser Fleischer rings
5. A 7 year old girl presents with acute onset of jaundice. What is your approach? [Investigation] Liver function tests including Internal normalized ratio (synthetic function) Consider the following tests (depending on history and examination): Viral serology (hepatitis A, B, C, EBV, CMV, adenovirus, parovirus, VZV, HSV, HHV6, Enterovirus) Serology for autoimmune hepatitis (anti-nuclear antibody, anti smooth muscle antibody, liver-kidney microsomal antibody and immunoglobulins) Abdominal ultrasound (choledochal cyst) Serum copper and caeruloplasmin levels (Wilson’s disease)
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