Biliary Disease

Dr Emma Wall Lecturer in Medicine MUST

Introduction
Bilirubin metabolism Jaundice and diseases of bilirubin metabolism History and investigations of Jaundice Gall bladder and bile metabolism Non surgical causes of obstructive jaundice Diseases of the gall bladde Clinical Cases

Bilirubin metabolism 1
Formed from breakdown of haemoglobin in bone marrow and spleen Circulates as UNCONJUGATED bilirubin (UCB) Becomes conjugated in the hepatocytes by glucuronic acid CONJUGATED bilirubin (CB) secreted in bile into the gall bladder and into the gut

Bilirubin metabolism 2
CB some reabsorbed by enterohepatic circulation CB some converted by gut bacteria to urobilinogen then reabsorbed and excreted by kidneys Urobilinogen also converted to stercobilinogen and excreted in faeces colours them brown.

Metabolism of Bilirubin

Diseases of Bilirubin
Pre-hepatic Raised UCB failure of breakdown or uptake
Hepatic hepatocellular jaundice CB

Post hepatic raised CB usually blockage or failure of excretion of bilirubin

Diseases of Bilirubin pre hepatic Jaundice

Physiology Excess bilirubin production Through haemolysis or failure of uptake / conjugation Raised unconjugated bilirubin in blood UCB is water insoluble so no bilirubin in urine Causes Physiological = neontal, dyserythropoesis Congenital = Gilbert syndrome, glucuronyl transferase deficiency Haemolysis = malaria, sepsis, drugs, inherited e.g. g-6PD deficiency

Hepatocellular Jaundice
Hepatocyte damage with some cholestasis Conjugated bilirubinaemia Causes: Viral hepatitis, drugs, alcoholic hepatitis, cirrhosis, metastases, haemachromatosis, sepsis, Wilsons disease, Budd-chiari syndrome, Inherited failure of excretion of bilirubin DubinJohnson syndrome

Drug induced Jaundice

Hepatitis Paracetamol Anti-TB Statins Valproate Halothane Mono-amine oxidase inhibitors Cholestasis Antibiotics, esp penicillin/cloxacillin Steroids Oral contraceptives Chlorpromazine Procholperazine Suphonylureas

Post hepatic Jaundice

Blockage of bile drainage into gall bladder/gut Conjugated bilirubin overspills into blood deep jaundice Raised water soluble CB in blood dark urine Reduced Bilirubin in gut to make urobilinogen and stercobilinogen so pale faeces

Causes of post hepatic Jaundice

Gall stones Pancreatic cancer Cholangiocarcinoma Lymph nodes at the porta hepatis Sclerosing cholangitis Primary Biliary cirrhosis Biliary atresia

Investigation of Jaundice 1
HISTORY: drugs, alcohol, pale stools, dark urine, sexual contacts family history, blood transfuions EXAMINATION: Scratch marks, signs of chronic liver disease, masses in the abdomen, ascites, signs of auto-immune disease

Investigation of Jaundice 2
Hb and Blood film: - ?haemolysis LFTs:Bilirubin with ALT/Alk P ? Obstructive or hepatitic. Albumin ?chronicity Urine dip: bilirubin is + in obstructive/cholestatic. in haemolysis. Urobilinogen is absent in obstructive/cholestatic. Virology: Hep B and C, HIV USS to look for dilated ducts and masses