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Dr.

Benjamin Sastro, SpPD

University of Pelita Harapan, School of Medicine

JAUNDICE
= icterus Is a yellowish discoloration of tissue resulting from the deposition of bilirubin Sign of liver disease or hemolytic disorder DD/
Carotenoderma The use of quinacrine Excessive exposure to phenol

Bilirubin synthesis

PRODUCTION AND METABOLISM OF BILIRUBIN


Hb breakdown RES (Spleen /liver) Biliverdin + CO + Fe

Bilirubin (water insoluble)

Bind to albumin (unconjugated)

Taken up by hepatocytes Coupled by protein ligandin Conjugated with glucoronic acid

Excreted into bile drains into duodenum

Unconjugated bilirubin (in distal ileum and colon)

Urobilinogen

Faeces (80-90%)

10-20% absorbed

Reexcreted by the liver

Small fraction is excreted in urine

Bilirubin metabolism and excretion

THE EVALUATION OF JAUNDICE


Hyperbilirubinemia may results from
Overproduction of bilirubin Impaired uptake, conjugation or excretion of bilirubin Regurgitation of unconjugated / conjugated bilirubin from damaged hepatocytes or bile

ducts

JAUNDICE: ETIOLOGIC CLASSIFICATION


HEMOLYTIC JAUNDICE or PRE HEPATIC JAUNDICE
HEPATIC OR HEPATOCELLULAR JAUNDICE OBSTRUCTIVE JAUNDICE or POST HEPATIC JAUNDICE

HEMOLYTIC JAUNDICE
Excessive breakdown of Erythrocytes Breakdown of Hemoglobin into Heme and Globin. Heme is converted into Bilirubin. Breakdown of Erythrocytes is called Hemolysis. So this is Hemolytic Jaundice. Since this happens before the Liver, it is called Pre Hepatic Jaundice.

Pre hepatic jaundice


No abnormalities of fat emulsification and absorption. Stools are normal. The blood picture shows anemia. There is reticulocytosis or the presence of extra reticulocytes in the blood. Abnormal erythrocytes are also found: Anisocytosis & Poikilocytosis.

HEPATIC / HEPATOCELLULAR JAUNDICE


Damage caused to the liver or liver cells (Hepatocytes). Damage may be due to:
Infections: Hepatitis A, or B, or instance. Toxins: Alcohol, etc.

Due to damage, the liver cannot process Bilirubin and so its blood level increases.

HEPATIC JAUNDICE
Hepatocytes produce bile. The bile salts in it help in the emulsification and absorption of Fats. In Liver damage, bile is not produced and so fats are not emulsified or absorbed. So the feces in such patients will be:
Bulky: all the fats are unabsorbed Greasy: due to fat content Foul smelling: due to bacterial action

OBSTRUCTIVE JAUNDICE
Also called Post Hepatic Jaundice. The root cause is obstruction to the bile ducts. This causes a block in the flow of bile. Since there is an obstruction, it is called Obstructive Jaundice. Since the cause of the Jaundice is beyond the liver, it is called Post Hepatic Jaundice.

OBSTRUCTIVE JAUNDICE
Feces is greasy & foul smelling. If the obstruction is complete, the stools will be colorless. This is because the stools do not have stercobilin, which gives the characteristic color to normal stools.

INITIAL STEPS
Determine
Predominantly conjugated or unconjugated Other biochemical liver tests are abnormal or not

ISOLATED ELEVATION OF SERUM BILIRUBIN


INDIRECT HYPERBILIRUBINEMIA
Hemolytic disorder
Inherited
Spherocytosis, elliptocytosis G6PD

Aquired
Microangiopathic hemolytic anemias PNH Immune hemolysis

Ineffective erythropoeis
Cobalamin, folate and iron deficiencies

ISOLATED ELEVATION OF SERUM BILIRUBIN


INDIRECT HYPERBILIRUBINEMIA
Drugs
Rifampicin, probenecid, ribavirin

Inherited conditions
Crigler Najjar types I and II Gilberts syndrome

DIRECT HYPERBILIRUBINEMIA
Inherited conditions
Dubin-Johnson syndrome Rotors syndrome

ELEVATION OF SERUM BILIRUBIN WITH OTHER LIVER TEST ABNORMALITIES


Primary hepatocellular process

Intra / extra hepatic cholestasis

MEASUREMENT OF SERUM BILIRUBIN


Van den Bergh reaction
Direct bilirubin
Indirect bilirubin

Normal < 1 mg/dL The presence of scleral icterus indicates a serum bilirubin of at least 3.0 mg/dL

History that should be taken from patients presenting with jaundice


Duration of jaundice Previous attacks of jaundice Pain Chills, fever, systemic symptoms Itching Exposure to drugs ( Physician prescribed/herbal/vitamin/anabolic steroid ) Biliary surgery Anorexia, weight loss Colour of urine and stool History of injections or blood transfusions Occupation

HISTORY
Recent travel history Exposure to people with jaundice Alcohol consumption Accompanying symptoms
Arthralgia, myalgia, rash, anorexia, weight loss, abdominal pain, fever, pruritus, changes in the urine and stool

Examination of patients with jaundice


Depth of jaundice Scratch marks Signs of chronic liver disease: Palmar erythema Clubbing White nails Dupuytren's contracture Gynaecomastia Liver: Size Shape Surface Enlargement of gall bladder Splenomegaly Abdominal mass Colour of urine and stools

PHYSICAL EXAMINATION
Assess patients nutritional status Muscle wasting Stigmata of CLD
Spider naevi, liver palm, gynecomastia, caput medusae, Dupuytrens contracture, parotid gland enlargement, testicular athropy

Jugular vein distention Right pleural effusion Size and consistency of the liver Spleen enlargement Ascites Murphys sign

LABORATORY TESTS
Enzyme for hepatic necrosis
ALT, AST

Enzyme for cholestatic process


AP, GGT

To assess liver function


Albumin, PT

HEPATOCELLULAR CONDITIONS THAT MAY PRODUCE JAUNDICE


Viral hepatitis
Hep A,B,C,D and E, EB virus, Cytomegalovirus, Herpes simplex

Alcohol Drugs toxicity


Predictable, dose dependent, eg acetaminophen Unpredictable, idiosyncratic, eg isoniazid

Environmental toxins
Vinyl chloride, Jamaica bush tea, wild mushrooms

Wilsons disease Autoimmune hepatitis

CHOLESTATIC CONDITIONS THAT MAY PRODUCE JAUNDICE


INTRAHEPATIC
Viral hepatitis Alcoholic hepatitis Drugs toxicity Primary biliary cirrhosis Primary sclerosing cholangitis Vanishing bile duct syndrome Inherited

CHOLESTATIC CONDITIONS THAT MAY PRODUCE JAUNDICE


INTRAHEPATIC
Cholestasis of pregnancy Total parenteral nutrition Non hepatobiliary sepsis Benign postoperative cholestasis Paraneoplastic syndrome Venoocclusive disease Graft versus host disease

CHOLESTATIC CONDITIONS THAT MAY PRODUCE JAUNDICE


EXTRAHEPATIC
Malignant

Cholangiocarcinoma Pancreatic cancer Gall bladder cancer Ampullary cancer Malignant involvement of the porta hepatis lymph nodes
Choledocholithiasis Primary sclerosing cholangitis Chronic pancreatitis AIDS cholangiopathy

Benign

Summary points
An isolated raised serum bilirubin concentration is usually due to Gilbert's syndrome, which is confirmed by normal liver enzyme activities and full blood count Jaundice with dark urine, pale stools, and raised alkaline phosphatase and glutamyl transferase activity suggests an obstructive cause, which is confirmed by presence of dilated bile ducts on ultrasonography Jaundice in patients with low serum albumin concentration suggests chronic liver disease Patients with high concentrations of bilirubin ( > 100 mol/l) or signs of sepsis require emergency specialist referral Imaging of the bile ducts for obstructive jaundice is increasingly performed by magnetic resonance cholangiopancreatography, with endoscopy becoming reserved for therapeutic interventions

Conjugation and secretion of bilirubin

Dupuytrens contracture

A 67 year old woman, who has previously been healthy, undergoes emergency surgery for a ruptured abdominal aortic aneurysm. Intraoperatively she requires 8 units of PRC to maintain her BP and Ht. Following surgery she is hemodinamically stable. On the third post operative day she appears jaundiced, but abdominal exam is unremarkable and she is afebrile. Total serum bilirubin concentration at this time is 8.3 mg/dl (direct 6.3). Serum AP level is 110 units and serum AST level is 51 U/ml. The most likely explanation for the woman jaundice is A. a stone in the common bile duct B. halothane hepatitis C. post transfusion hepatitis D. acute hepatic infarct E. benign intrahepatic cholestasis

Case