Liver Flashcards
Features hepatic dysfunction
Encephalopathy Jaundice Epistaxis Cholestasis (pale stools, dark urine, deficiency fat soluble vit) Ascites Hypotonia Peripheral neuropathy Rickets (Vit D deficiency) Varices with portal hypertension Spider Naevi Muscle wasting (malnutrition) Bruising and petechia Splenomegaly with portal hypertension Hepatorenal failure Palmar erythema Clubbing Loss of fat stores (malnutrition)
Physiological jaundice
Common
90% resolve by 2 w
- 3w if preterm
Feature Bile Duct Atresia
Prolonged neonatal jaundice Progressive fibrosis and obliteration of the biliary tree Chronic liver failure and death within 2y Mild Jaundice Pale stools Faltering growth Hepatomegaly Splenomegaly
Ix Biliary atresia
Raised conjugated bilirubin
Abnormal LFTs
Fasting abdo US: contracted or absent gall bladder
Dx by ERCP: failure to outline normal biliary tree
Liver biopsy: neonatal hepatitis w extrahepatic biliary obstructive features
Mx Biliary Atresia
Palliative surgery:
-Kasai hepatoportoenterostomy (jejenum anastamoses with porta hepatis)
-fibrotic ducts bypassed to allow biliary drainage
Early surgery increases success rate
Fat soluble vitamin supplementation
Liver transplant
Cx Biliary Atresia
Cholangitis
Cirrhosis
Malnutrition
Causes of prolonged neonatal jaundice
Breast milk jaundice Infection (esp UTI) Haemolytic anaemia Hypothyroidism High GI obstruction Critter-Najjar syndrome Biliary Atresia Choledochal cysts Neonatal hepatitis syndrome Intrahepatic biliary hypoplasia (alagille syndrome)
Causes of neonatal hepatitis syndrome
Congenital infection Inborn errors of metabolism alpha1-Antitripsin deficiency Galactosaemia Tryosinaemia type 1 Errors of bile acid synthesis Progressive familial intrahepatic cholestasis Cystic fibrosis Intestinal failure associated liver disease (parental nutrition)
Features Alagille syndrome
AD inheritance
Characteristic triangular face
Skeletal abnormality .e.g. Butterfly vertebrae
Congenital heart disease: peripheral pulmonary stenosis
Renal tubular disorders
Eye defects
Profoundly choleostatic with pruritis and faltering growth
Mx Alagille Syndrome
Confirmed by genetic test Nutrition and fat soluble vitamins Pruritis difficult to manage Liver transplant Death by cardiac cause
Features Progressive familial intrahepatic cholestasis
AR disorder affecting bile salt transport Jaundice Intense pruritis Faltering growth Rickets Diarrhoea Hearing loss Gallstones
Mx Progressive familial intrahepatic cholestasis
Dx by gene mutation in bile salt transporter genes
Nutritional support and fat soluble vitamins
Liver transplant due to progression of fibrosis
Pruritis difficult to manage
Features alpha1-Antitypsin deficiency
AR disorder (protease inhibitor PiZZ chromosome 14)
Protein accumulation within hepatocytes
Liver disease in infants and children
Lung disease in adulthood
Presentation alpha1-Antitrypsin deficiency
Prolonged neonatal jaundice Bleeding due to Vit K defieincy Hepatomegaly Splenomegaly Cirrhosis Portal hypertension
Mx alpha1-Antitrypsin Deifiency
Dx on enzyme level in plasma and protein phenotype
50% have good prognosis
Liver transplant
Avoid smoking: risk of pulmonary disease
Features Galactosaeia
Rare Poor feeding Vomiting Jaundice Hepatomegaly Symptomatic when fed milk Untreated leads to cataracts and developmental delay Late features: ovarian failure, learning difficulty
Galactosaemia fatal course
Usually caused by Gram negative sepsis
Shock
Haemorrhage
DIC
Ix Galactosaemia
Detecting galactose in urine
Enzyme galactose-1-phosphate-uridyl transferase in rbc
Can be masked by recent blood transfusion
Mx Galactosaemia
Galactose free diet prevent liver disease progression
Features Fulimant Hepatitis
Development of hepatic necrosis with subsequent loss of liver function \+/- liver encephalopathy Due to infection or metabolic conditions Jaundice Coagulopathy Hypoglycaemia Electrolyte disturbance
Early signs hepatic encephalopathy
Alternate periods of irritability and confusion with drowsiness
Aggressive and difficult behaviour
Cx fulminant hepatitis
Cerebral oedema
Haemorrhage
Sepsis
Pancreatisis
Dx fulminant hepatitis
Bilirubin normal in early stages Transaminases elevated 10-100x normal level Alkaline phosphatase increased Abnormal coagulation Plasma ammonia increased EEG shows hepatic encephalopathy CT cerebral oedema
Mx fulminant hepatitis
Maintain blood glucose >4mmol/L: IV dextrose
Broad spectrum Abx : sepsis prevention
Antifungals
IV vitamin K
H2 blocking/PPIs: prevent GI haemorrhage
Fluid restriction and mannitol in cerebral oedema
Poor prognostic factors fulminant hepatitis
Shrinking liver Rising bilirubin Falling transaminases Worsening coagulopathy Progression to coma -Without liver transplant ~ 70% in coma will die
Causes of acute liver failure <2y
Infection .e.g. Herpes simplex Metabolic disease Seronegative hepatitis Drug induced Neonatal haemochromatosis
