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
Causes liver failure >2y
Seronegative hepatitis Paracetamol OD Mitochondrial disease Wilson disease Autoimmune hepatitis
Causes chronic liver disease
Post viral hepatitis B/C Autoimmune hepatitis Sclerosing cholangitis Drug induced liver disease Cystic fibrosis Wilson disease Fibropolycystic liver disease Non-alcoholic fatty liver disease Alpha1-antitrypsin deficiency
Features Viral hepatitis
Nausea Vomiting Abdominal pain Lethargy Jaundice Large tender liver Splenomegaly Raised transaminases Normal coagulation
Features Hepatitis A
Mild illness 2-4w
Self limiting prolonged cholestatic hepatitis and fuliminant hepatitis
Mx Hepatitis A
IgM antibody Dx
No treatment
Vaccination of close contacts within 2w of onset
Human normal immunoglobulin in those at high risk
Transmission of hepatitis B
Perinatal from mother to baby
Horizontal spread within families
Inoculation with infected blood via needles, dialysis etc.
Sexual transmission
Outcome for infants with HBV
Usually asymptomatic
90% become chronic carriers
Outcomes for older children with HBV
Usually asymptomatic
Majority resolve spontaneously
1-2% develop acute liver failure
5-10% chronic carriers
Dx Hepatitis B
HBV antigens and antibodies
IgM core antibodies in acute infection
HBV surface antigen ongoing infectivity
Cx chronic hepatitis
Cirrhosis
Hepatocellular carcinoma
Mx chronic HBV
Poor efficacy
Interferon or Pegylated interferon successful in 30-50%
Antiviral .e.g. Lamivudine, adefovir effective in 25%
Prevention hepatitis B
Antenatal maternal screening
Surface antigen positive: babes receive vaccination from birth
E Antigen positive: hepatitis B immunoglobulin
Check antibody response to vaccination at 12m
-further vaccination required in 5%
Rest of family vaccinated
Features Hepatitis C
IVDU
Vertical transmission greater in HIV co-infection
Majority become chronic carriers: cirrhosis and hepatocellular carcinoma risk
Mx hepatitis C
Pegylated interferon and ribavirin is curative
Treatment >3y old: may resolve with viral infections
Features hepatitis D
Cannot replicate without HBV
Causes acute exacerbation of chronic HBV
Cirrhosis in chronic infection
Features Hepatitis E
Mild self limiting illness in most
Associated with water, infected pork, blood transfusion
During pregnancy: fulminant hepatic failure with high mortality
Features autoimmune hepatitis and sclerosing cholangitis
Presents 7-10y
More common in girls
Acute hepatitis, fulminant hepatitis, or chronic liver disease
Autoimmune features: skin rash, arthritis, haemolytic anaemia, nephritis
Association with IBD, coeliac, other autoimmune disease
Dx Autoimmune hepatitis and sclerosing cholangitis
Elevated total protein Hyperglammaglobulinaemia IgG >20g/L Positive autoantibodies Low serum complement C4 Typical histology
Mx Autoimmune hepatitis and sclerosing cholangitis
Prednisone
Azathioprine
Urosodeoxycholic acid- sclerosing cholangitis
Liver disease in CF
Second most common cause of death
Most common abnormality steatosis
Other cx; progressive biliary fibrosis from thick tenacious bile, cirrhosis, portal hypertension
Histology: fatty liver, focal biliary fibrosis, focal modular cirrhosis
Mx Liver disease in CF
Supportive therapy Endoscopic treatement of varices Nutritional therapy Ursodeoxycholic acid Liver transplantation/ heart-lung transplantation
Features Wilson disease
AR mutation in caeruloplasmin
Defective excretion of copper in the bile
Accumulation of copper in brain, liver, kidney, cornea
Presents >3y
Presentation Wilson disease
Young children: liver disease Older children: neuropsychiatric disease (extrapyrimidal signs) Renal tubular dysfunction Vitamin D resistant rickets Haemolytic anaemia Kayser-fleischer rings develop >7y
Ix Wilson disease
Low serum caeruloplasmin and copper
Increased urinary copper excretion
-further increased by cheating agent penicillamie
Dx hepatic copper on biopsy or gene mutation
Mx Wilson disease
Penicillamine or trientine -promote urinary copper excretion -reduce hepatic and CNS copper Zinc: reduces copper absorption Pyridoxine: to prevent peripheral neuropathy
Features Fibroplastic Liver Disease
Inherited condition affecting biliary tree development
Liver cystic disease
Fibrosis
Renal disease
Features congenital hepatic fibrosis
Children >2y Hepatosplenomegaly Abdominal distension Portal hypertension Normal LFTs Coexisting cystic renal disease: hypertension and renal dysfunction Histology: bands of hepatic fibrosis
Cx congenital hepatic fibrosis
Portal hypertension
Varices
Cholangitis
Features non-alcoholic fatty liver disease
Fatty deposits and inflammation, fibrosis, cirrhosis and liver failure
Associated with metabolic syndrome and obesity
Usually asymptomatic
RUQ pain, lethargy
Ix Non-alcoholic fatty liver disease
Echogenic liver on US
Mildly elevated transaminases
Biopsy: marked steatosis +/- inflammation and fibrosis
Cx chronic liver disease
Nutrition: fat malabsorption (long chain), protein malnutrition (high liver catabolism), anorexia (NG feed) Fat soluble vitamin (oral or IM) Pruritus Encephalopathy Cirrhosis Portal hypertension Oesophageal varices Ascites Spontaneous bacterial peritonitis Renal failure
Indications for liver transplant
Severe malnutrition unresponsive to therapy
Cx refractory management: bleeding, varices, ascites
Poor quality of life
Failure of growth and development
Post transplanation Cx Liver
Primary non function Hepatic artery thrombosis Biliary leaks and strictures Rejection Sepsis Death occurs within first 3m 1y survival ~ 90% 20y survival >80%
Mx Pruritis in liver disease
Loose cotton clothing Avoid overheating Cut nails Emollients Phenobarbital: stimulate bile flow Cholestyramine: absorb bile salts Ursodeoxycholic acid: solubise bile Rifampicin: enzyme inducer
Fat soluble vitamins defiencies
K: bleeding
A: retinal changes and night blindness
E: peripheral neuropathy, haemolysis, ataxia
D: Rickets and fractures
Features Cirrhosis
Extensive fibrosis with regenerative nodules
2’ to hepatocellular disease/chronic bile duct obstruction
Diminished hepatic function
Portal hypertension
Splenomegaly, varices, ascites
Risk of hepatocellular carcinoma
Signs of cirrhosis
Jaundice Palmar erythema Plantar erythema Telangiectasia Spider navaei Malnutrition Hypotonia Dilated abdominal veins Splenomegaly Shrunken and impalpable liver
Screening in cirrhosis
Cause of chronic liver disease
Upper GI endoscopy: varices and erosive gastritis
Abdominal US: liver, spleen, varices
Biopsy
Ix decompensated cirrhosis
Elevated aminotransferases and alkaline phosphatase
Decreased plasma albumin
Prolonged PT
Mx oesophageal varices
Upper GI endoscopy
Blood transfusion and H2 antagonist in acute bleeds
Octreotide infusion, vasopressin analogues, endoscopic band ligation and sclerotherapy in persistent bleeds
Portocaval shunting
Stent placement between hepatic and portal vein
