This document defines jaundice and discusses its various causes and mechanisms. Jaundice is caused by an increased bilirubin level in body fluids and can be due to increased production from hemolysis, impaired excretion from conditions like liver disease, or obstruction of bile flow from the liver or gallbladder. The causes, signs, investigations, and approach to evaluating a patient with jaundice are described.

1. JAUNDICE By Dr. Osman Bukhari

3. Definition Yellow discoloration of the sclera, mucus membranes & skin from increased serum bilirubin concentration in the body fluids, detectable when serum level is above 3mg/100ml Mechanism 1-Increased production in haemolysis 2-Imparied excretion a) Congenital non-haemolytic hyper bilirubinaemia

4. b) Hepatocellular jaundice : acute or chronic parenchymal liver disease c) Cholestasis Haemolytic jaundice 1-Due to increased RBC destruction 2-Jaundice is mild & urine is dark. 3-Anaemia 4-Spleenomegaly +_ 5-Increased uncongugated bilirubin in the blood

5. 6-Increased urobilinogen in urine in the absence of bile pigments 7-Reticulocytosis 8-Decreased haptoglobin 9-Peripheral blood macrocytosis , polychromasia & red cells abnormality (spherocytosis & sickles ) 10-Bone marrow erythrocytosis with megaloblastic changes from folate deficiency 11-Decreased RBC survival

6. . 12-Increased serum L.D.H 13-Normal L.F.T Congenital non haemolytic hyper bilirubinaemia Inherited disorders due to either defective bilirubin uptake, conjugation or excretion 1-Gilberts (unconjugated hyper bilirubinaemia) -Autosomal dominant -Deficient glucuronyl transferase & defective bilirubin uptake from plasma leading to Unconjugated hyper bilirubinaemia

7. -Commonest variety affecting youngs -Mild jaundice which may follow recovery from viral hepatitis or precipitated by infection, fatigue or fasting -May be asymptomatic or dyspeptic symptoms -Normal life spam -No evidence of haemolysis -No treatment but occasionally Phenobarbitone

8. 2- Crigler – Najjar (unconjugated) -Rare -Type I is A.R in neonates with absent glucuronyl transferase with rapid death from kernictorus -Type 2 is A D in neonates with reduce glucuronyl transferase activity leading to severe jaundice & patient can survive to adult life with Phenobarbitone, photothearapy & liver transplantation

9. 3- Dubin – Johnson (conjugated) -A.R -Defective canalicular excretion of bilirubin -Any age -Jaundice is mild -Liver histology shows black pigmentation -Normal life span

10. 4- Rotors (conjugated) -AD -Similar to Dubin – Johnson but no pigmentation in Liver histology

11. - Hepatocellular jaundice 1-Impaired bilirubin transport to the bile at any point between uptake of unconjugated bilirubin into hepatocytes & transport of conjugated bilirubin into the canaliculae 2-Cholestasis may occur from inflammatory aedema 3-Both conjugated & unconjugated hyper bilirubinanemia 4-Jaundice is variable

12. 5-Urine is dark 6-Increased Amino transferases Cholestatic jaundice (obstruction) 1-Intra hepatic : due to failure of hepatocytes to generate bile flow or obstruction of bile flow in the bile ducts within the liver e.g. PBC, Drugs, Viral hepatitis, pregnancy, primary Sclerosing cholengitis, idiopathic 2-Extra hepatic : due obstruction of bile flow in extra hepatic bile ducts -Choledocholithiasis

15. -Carcinoma (Ampullary, Pancreatic, Cholangio carcinoma & secondaries in the porta hepatis -Benign tumours -Cystic fibrosis -parasitic infections -Traumatic biliary stricture -Choledocal cyst -Pancreatitis 3-Clinical features due to Cholestasis include:

16. -Early features: jaundice, dark urine, pale stool & pruritis -Late features: Xanthama & Xanthelasma, malabsorption (steatorhoea, weight loss, bleeding tendency & Osteomalacia) 4-Clinical features due to cholangitis include fever, Rigors, pain & hepatic abscess 5-Investigations: -High ALP, G.GT, & prolong P.T -Mainly conjugated bilirubin

17. -U/S, ERCP, CT & P.T.C, Serology & liver biopsy Underlying cause of Cholestatic jaundice related to clinical features 1-Static or progressive jaundice = Carcinoma 2-Fluccuating jaundice = stone, stricture, Pancreatitis or Choledocal cyst 3-Abdominal pain = stone, Pancreatitis or Choledocal cyst 4-Cholangitis = stone, stricture or choledocal cyst

18. 5-Abdominal scar = stone , stricture 6-Irregular hard Hepatomegly = hepatic carcinoma 7-Palpable G.B =Carcinoma below cystic duct e.g. Ca head of pancreas 8-Abdominal mass = Ca, Pancreatitis (cyst) or Choledochal cyst 9-Occult blood in stools = Ampullary tumour

19. Work out a case of jaundice Or Abnormal liver function tests 1-Young patient : likely to have viral hepatitis. Ask about history of drugs, alcohol & sexual behaviour 2-Elderly patient with weight loss :- Ca 3-Country of origin (HBV) + travel (HAV) 4-Abdomial pain: biliary obstruction orhepatitis 5-Duration of illness: short history with prodrome suggest HAV as a recent outbreak of jaundice

20. 6-I.V. drugs abuse, tattooing, blood transfusion or plasma products (HBV,HCV) 7-Male homosexuality or female prostitution: HBV 8-Alcohol consumption 9-History of drug ingestion in the previous three months 10-Recent anaesth: Halothane 11-Recent history of biliary surgery or Ca 12-F.H: Gilbert, Wilson's 13-Pruritus: Cholestasis

21. 14-Fever & chills: Cholangitis or liver abscess 15-Smooth tender Hepatomegaly: hepatitis & extra hepatic obstruction 16-Nodular hepatomegaly: malignancy 17-Spleenomegaly: PHT 18-Acitis: Cirrhosis or PHT or Ca 19-Palpable G.B: Ca head of pancreas 20-Generalised L.N: Lymphoma 21-Viral marker for high risk groups

22. 22-U/S to exclude extra hepatic Cholestasis ـــــ dilated ducts ـــــ level of obstruction ـــــ cause of obstruction 23-Liver biochemistry 24-CBC & Retics count 25-Low serum alb. & prolonged PT: chronic liver disease 26-Leucocytosis suggest bacterial infection e.g. Cholangitis or hepatic abscess

23. 27-lucopenia with viral hepatitis 28-Abnormal lymphocyte: inf. Mono. 29-Serolgy & alphafoetoprotien 30-ERCP & PTC 31-FNAC 32-Fine needle biopsy: more sensitive 33-Generally -Young patient ـــــ viral markers -If negative ـــــ U/S -If both U/S & viral markers are negative ــــ serology

24. -U/S ـــــ obstruction ـــــ ERCP & PTC -U/S ـــــ Focal lesion ـــــ FNAC or biopsy -U/S ـــــ abnormal liver parenchyma ـــــ liver biopsy -U/S ـــــ normal ـــــ viral markers -U/S ـــــ portal or venous obstruction ـــــ Angiography