Paediatric Liver Disease Flashcards
Name the components of LFTs
- Bilirubin: total and split
- ALT/AST
- Alkaline Phosphatase
- Gamma GT
Which tests actually test liver function?
- Coagulation: PT/INR and APTT
- Albumin
- Bilirubin
- (Blood glucose)
- (Ammonia)
What are the signs of chronic liver disease in children?
- Growth failure
- Jaundice
- Same signs as adults
How is bilirubin metabolised?
- Erythrocytes are broken down in the reticuloendothelial system in to haem
- The haem is then broken down in biliverdin and then into unconjugated bilirubin
- The bilirubin is then transported to the liver where it is then conjugated and turned into bile
Describe the classification of neonatal jaundice
- Early: <24hrs old (always pathological)
- Intermediate: 24hrs - 2wks
- Prolonged: > 2wks
What is the cause of physiological jaundice?
- Shorter RBC life span in infants
- Relative polycythaemia and immaturity of liver function
- Unconjugated jaundice
- Develops after first day of life
Name some causes of early/intermediate unconjugated infant jaundice
- Sepsis
- Haemolysis (ABO incompatibility, rhesus disease, bruising, red cell membrane defects and red cell enzyme defects)
- Abnormal conjugation (Gilbert’s disease and Crigler-Najiar syndrome)
How can the causes of early/intermediate jaundice be investigated?
- Blood group
- DCT
- Blood film
- G6PD assay
- Genotype and phenotype
What is kernicterus, how does it present and what is it bad?
- Unconjugated bilirubin can cross the blood-brain barrier and deposits in the brain
- It is neurotoxic
- Early signs: encephalopathy, poor feeding, lethargy and seizures
- Late consequences: severe choreoathetoid cerebral palsy, learning difficulties and sensorineural deafness
How can unconjugated jaundice be treated?
- Phototherapy (450nm blue light)
- Exchange transfusion
What are the causes of prolonged infant jaundice?
- Anatomical (biliary obstruction)
- Neonatal hepatitis
- Hypothyroidism
- Breast milk jaundice
What is the most important test in prolonged jaundice and what does it show?
Split bilirubin - shows the amount of conjugated bilirubin
What types of biliary obstruction cause jaundice and how do they present?
- Biliary atresia: conjugated jaundice and pale stools
- Choledochal cyst: conjugated jaundice and pale stools
- Alagille syndrome: intrahepatic cholestasis, dysmorphism and congenital cardiac disease
What are the features of biliary atresia?
- Prolonged, conjugated jaundice
- Pale stools and dark urine
- Progression to liver failure if not identified and treated
How can biliary atresia be treated?
- Kasai portoenterostomy (best if performed within 60 days)
- Liver transplant may be required
