HYPERBILIRUBINEMIA JAUNDICE (Icterus)

Presenter: Abdul Mushib Ibrahim MBBS Year 4 UPSM

Definition
Refers to YELLOWISH pigmentation of: SKIN SCLERAE MUCOSA -Due to increased levels of bilirubin in the blood.

VALUES
NORMAL PLASMA Bilirubin: 0.5mg/dl ABNORMAL PLASMA Bilirubin: > 1.5mg/dl or > 35micromoles/L

Classification of Jaundice
Jaundice is classified by 1-Type of Circulating Bilirubin: a)Conjugated b)Unconjugated 2-Site of Problem: a)Pre-Hepatic b)Hepatic c)Post Hepatic/Cholestatic/Obstructive

RBC BREAK DOWN

GLOBIN

HEME

YELLOW DISCOLOURATION OF SKIN

1-Gilberts Syndrome 2-Crigler Najjar Syndrome

biliverdin (green color)

Unconjugatedbilirubinyellow ALBUMIN BLOOD STREAM

Bilary Tree & Cystic Duct-> BILE

Intestinal Bactria

Urobilinogen

LIVER

UDP-glucuronyl transferase

Conjugated Bilirubin stercobilin urobilin

Uncongugated Bilirubin -Is water insoluble. -It does not enter urine. -Bound to plasma Albumin-> travels to Liver->to form congugated Bilirubin. -Results in ACHOLURIC Hyperbilirubinaemia.

Classification of Jaundice
Jaundice is classified by 1-Type of Circulating Bilirubin: a)Unconjugated b)Conjugated 2-Site of Problem: a)Pre-Hepatic b)Hepatic c)Post Hepatic/Cholestatic/Obstructive

Pre-Hepatic-Uncongugated
The pathology is occurring prior to the liver caused by anything which causes an increased rate of breakdown of red blood cells ISOLATED raised Bilirubin levels(Uncongugated)

Hemolytic Jaundice: Genetic diseases, such as: 1-sickle cell anemia 2-spherocytosis 3-thalassemia 4 glucose 6-phosphate dehydrogenase deficiency

Infective Causes: 5-Malaria-In tropical countries 6-Leptospirosis. Congenital Causes: 7-Gilberts Syndrome 8-Cringler Najjar Syndrome
TYPE I TYPE II

Classification of Jaundice
1-Type of Circulating Bilirubin: a)Unconjugated b)Conjugated 2-Site of Problem: a)Pre-Hepatic b)Hepatic c)Post Hepatic/Cholestatic/Obstructive

Hepatic Jaundice-Congugated
DEFINITION Results from the inability of the liver to transport bilirubin across the hepatocyte into the bile duct, occuring as a consequence of parenchymal liver disease.

 Bilirubin transport is impaired because of:

Uptake of Uncongugated Bilirubin into the cells Transport of Congugated Bilirubin into the Canaliculi.

 In Hepatic Jaundice, concentrations of both congugated and Uncongugated Bilirubin increase. CARACTERISTICS OF HEPATIC JAUNDICE
 Increase in Transaminases
AST (Aspartate Transaminase-5-35 iu/L) ALT (Alanine Aminotransferase-5-35iu/L)

NOTE: Increase in other LFTS suggest other specific aetiologies.

 Acute Jaundice in presence of AST > 1000U/L is HIGHLY SUGGESTIVE of: 1. An Infectious Cause
 Hepatitis A, B,C, Alcoholic,  CMV  EBV

2. Hepatic Ischaemia

3. Drugs
     

Paracetamol Overdose Isoniazid, Rifampicin, Pyrazinamide Monoamine Oxidase Inhibitors Sodium Valproate Halothane Statins

Other Causes of Hepatic Jaundice 4. Failure to excrete Congugated Bilirubin

 Dubin Johnson Syndrome Rotor Syndrome

5-Sepsis ,hypoperfusion states 6-Toxins

 Fungi-Amanita Phalloides Carbon Tetrachloride

DIAGNOSING HEPATIC JAUNDICE

Blood Test-LFTS Imaging-essential to identify features suggestive of cirrhosis.
 Irregular liver outline  Splenomegaly Define Patency of Hepatic Arteries, Veins, Portal Vein.

 Liver Biopsy-to define the cause of Hepatic Jaundice.

Classification of Jaundice
1-Type of Circulating Bilirubin: a)Unconjugated b)Conjugated 2-Site of Problem: a)Pre-Hepatic b)Hepatic c)Post Hepatic/Cholestatic/Obstructive

POST HEPATIC-Obstructive (Cholestatic) Jaundice

Caused by: -Failure of hepatocytes to initiate bile flow. -Obstruction of Bile flow in bile ducts

Disease States
Obstructive Jaundice extrahepatic cholestasis
Choledocholithiasis (CBD stone) Cancer (peri-ampullary or cholangio CA) Strictures after invasive procedures Acute and chronic pancreatitis Primary sclerosing cholangitis (PSC) Parasitic infections
Ascaris lumbricoides, liver flukes

 Drug induced Cholestatsis

Flucloxacillin Augmentin Nitrofurantonin Steroids (Pill) Sulfonylureas  Prochlorperazine  Chlorpromazine.

 Congugated Bilirubin Dark Urine But less Congugated Bilirubin enters the gut thus feaces is pale. When severe associated with pruritis RXrelief of obstruction.

Cholestatic Jaundice is CHARACTERISED by: INCREASED: ALP -Alkaline Phosphatase-30-150 GGT(-g- Glutamyl transpeptidase U/S is indicated to determine mechnical obstruction & Dilatation of bilary tree.

EVALUATION

Initial Evaluation: History

Jaundice, pale stool, tea-colored urine Fever/chills, RUQ pain (cholangitis)
Could lead to life-threatening septic shock

Reasons to have hepatitis or cirrhosis?

Alcohol, Viral, risk factors for viral hepatitis

Exposure to toxins or offending drugs Inherited disorders or hemolytic conditions Recent blood transfusions or blood loss? Is patient septic? Recent gallbladder surgery? (CBD injury)

Initial Evaluation: Physical Exam

Signs of end stage liver disease (cirrhosis)
Ascites, splenomegaly, spider angiomata, and gynecomastia

Jaundice evident first underneath the tongue, also evident in sclerae or skin Courvoisiers sign = painless, but palpable or distended gallbladder on exam
Could indicate malignant obstruction (e.g Pancretic Cancer) Unlikely to be caused by gallstone obstruction.

Screening Labs
URINE TEST -Bilirubin is absent in pre-hepatic cause. -Urobilinogen is absent in obstrcutive cause. HAEMATOLOGY -FBC -Clotting

BIOCHEMISTRY -U&E LFT (Bilirubin,ALT,AST, ALK PHOS, GGT, Total Protein, Albumin) Alk Phos moreso than AST/ALT implies cholestasis (intrahepatic vs obstruction)
Alk Phos also seen in sarcoid, TB, bone In this case, GGT is specific for biliary origin

Predominant AST/ALT implies intrinsic hepatocellular disease

AST/ALT ratio > 2 in alcoholic hepatitis

albumin or INR c/w advanced liver dz

Imaging for Obstructive Jaundice

RUQ Ultrasound
See stones, CBD diameter->6mm obstruction.

ERCP
Direct visualization of biliary tree/panc ducts Procedure of choice for choledocholithiasis Diagnostic AND- therapeutic

Endoscopic Ultrasound CT scan

Identify both type & level of obstruction If abdominal malignancy is suspected

Treatment
If Medical, then treat the etiology If Obstructive Jaundice:
Ascending cholangitis
For cholangitis: IVF, IV Antibiotics, Decompression

Stones (remove stones vs stent vs drainage)

Done via ERCP or open (surgery)

Benign stricture (stent vs drainage catheter) Cancer (Stent vs drainage +/- resect the CA)

THE END
REFERANCE -DAVIDSONS-PRINCIPLES & PRACTICE OF MEDICINE -OXFORD HAND BOOK OF CLINICAL MEDICINE -WIKIPEDIA