21. Liver Problems Flashcards
What is the function of the liver?
Filtration Clotting factors Enzyme activation Bile production Storage of glycogen, vitamins and minerals
What are the liver damage tests?
Bilirubin (conjugated and unconjugated)
ALT/AST- alanine aminotransferase/aspartate aminotransferase- elevated in hepatocellular damage
Alkaline phosphate — elevated in biliary disease (not used in paeds)
Gamma glutmya transferase (GGT)- elevated in biliary Test
How do you measure liver function?
Coagulation- prothrombin time/ (PT/INR)
Albumin- chronic liver disease
Bilirubin
Blood glucose
Ammonia
What is the most common manifestation of paediatric liver disease
Jaundice- very very common
Incidental finding of abnormal blood test
What are the signs of chronic liver disease in children?
Growth failure Encephalopathy Portal hypertension Spider naevi Ascites Clubbing Rickets
How high is bilirubin to cause jaundice?
> 40-50umol/L
What is beta-karetaenemia?
Or changing of the skin due to lots of vegetables. It will only change skin pigment and not the sclera
Explain the process of unconjugated bilirubin being formed?
Post mature erythrocytes are broken down all over the body into haem. Haem is broken down into biliverdin which is transmitted into reductase
How is conjugated bilirubin excreted?
Unconjugated bilirubin binds to albumin and is transported to the liver. In the liver it is turned into conjugated bilirubin (water soluble)
It then is put into the bile, excreted in the small intestine and excreted in pee or poor
What are the different types of jaundice?
Pre hepatic jaundice- problem with haemolysis, increase in unconjugated bilirubin
Hepatic jaundice- problems with the liver e.g. hepatitis. Mix of conjugated and unconjugated
Post hepatic jaundice- problems with the biliary tree, increase in conjugated bilirubin
What are the types of neonatal jaundice?
Early (<24 hours) always pathological, caused by haemolysis and sepsis
Intermediate (24hrs-2 weeks) Caused by physiology, breast milk, sepsis, heamolysis
Prolonged (>2 weeks) Caused by obstruction, hepatitis, hypothyroidism, breast milk
Why do infants become physiologically jaundiced?
Shorter RBC life span in infants Relative polycythaemia (high RBC) Relative immature liver function
Unconjugated jaundice that occurs after the first day of life
What is kernocterus?
What are the signs?
What are the complications?
Disease of the brain where unconjugated bilirubin crosses the brain barrier and deposits in the brain
Early signs are encephalopathy, poor feeding, lethargy and seizures
Late consequences include cerebral palsy, learning difficulties and deafness
How do you treat kernocterus
Phototherapy- blue light converts bilirubin to water soluble isomer
(Not UV LIGHT)
What are some other causes of early jaundice?
Be specific
Sepsis ABO incompatibility Rhesus disease Bruising G6DP, spherocytosis
How do you investigate early jaundice?
Urine and blood glucose
Coombs Test
GD6P assay
Genotype/phenotype (gilberts)
What are the different causes of older neonatal jaundice
Anatomical (biliary obstruction)- conjugated
Neonatal hepatitis- conjugated
Hypothyroidism- unconjugated
Breast milk jaundice- unconjugated
What is the key message from this lecture?
Conjugated jaundice in children is always abnormal
What is another key message from this lecture?
Always assess stool colour I’m infants with prolonged jaundice
What are the different types of prolonged jaundice (obstructive)
How do Test for it
Biliary atresia- conjugated jaundice pale stools
Investigate With bilirubin USS, biopsy
Choledochal cyst conjugated jaundice, pale stools
Investigate with bilirubin, USS
Alagille sydrome Intrahepatc cholestasis, dysmorphism, congenital cardiac disease. Investigate using genotpying
How does biliary atresia present?
Inflammatory process of bile ducts leading to the destruction of the bile ducts
Prolonged conjugated jaundice
Pale stools, dark urine
Progression to liver failure if not identified and treated
What is the treatment of biliary atresia?
Kasai portentersostomy- needs to be done before 60 days
Early diagnosis is key
If not need liver transplant
Doesn’t always cure, can lead to liver transplant in childhood/ Adult life
Why do you assess children with jaundice early
Prove it’s not biliary atresia as needs treated ASAP
What are some rare causes of prolonged jaundice?
Alpha 1 antitrypsin level Galactosaemia Tyrosineamka Urea cycle defects Haemochromotosis
What are the three things we must remember!
Always ask about stool colour
Test split bilirubin
Conjugated jaundice ALWAYS needs further investigation. Test for biliary atresia until it’s not
