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Clinical Approach to Neonatal Jaundice. Dr. Siddeeg Addow Pediatric Resident Khartoum, Sudan. CONTENTS: INTRODUCTION PATHOPHYSIOLOGY DIFFERENTIAL DIAGNOSIS HISTORY EXAMINATION INVESTIGATION. INTRODUCTION. Bilirubin is the end product of heme degradation
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Clinical Approach to Neonatal Jaundice Dr. Siddeeg Addow Pediatric Resident Khartoum, Sudan
CONTENTS: INTRODUCTION PATHOPHYSIOLOGY DIFFERENTIAL DIAGNOSIS HISTORY EXAMINATION INVESTIGATION
INTRODUCTION • Bilirubin is the end product of heme degradation • Most of the daily production comes from the breakdown of RBCs in the RES • Heme biliverdin bilirubin • Bilirubin is released & bound to serum albumin • Bilirubin is uptake & conjugated with glucuronic acid • Finally conjugated bilirubin is excreted in bile
PATHOPHYSIOLOGY UNCONJUGATED B. CONJUGATED B. • Tightly compounded to s. albumin • Normally very small amount is present as albumin free • Insoluble in water can not be excreted in urine • Toxic • Non toxic • Water soluble • Loosely bound to albumin. Delta fraction
Both conjugated & unconjugated bilirubin may accumulate systemically & deposit in tissues • Normally s. bilirubin level vary b/w 0.3 & 1.2mg/dl. • The rate of systemic bilirubin production is = to the rate of hepatic uptake, conjugation & biliray excretion . • Jaundice becomes evident when the s.bilirubin levels rise above 2.0 to 2.5mg/dl
Levels as high as 30 to 40mg/dl can occur with sever disease • Jaundice occurs when the = b/w bilirubin production &clearance is disturbed by one or more of the following mechanisms: • Excessive production of bilirubin • Reduced hepatic uptake • Impaired conjugation • Decreased hepatocellular excretion • Impaired bile flow
Excessive production of bilirubin • hemolytic anemia's • resorption of blood from internal hemor. • ineffective erythropoiesis
Reduced hepatic uptake: • drugs • some cases of Gilbert syndrome
Impaired bilirubin conjugation: • physiologic jaundice • breast milk jaundice • genetic deficiency of glcuronosyl transferase • decreased expression of glcuronosyl transferase • diffuse hepatocellular diseases
Decrease excretion of conjugated bilirubin: • deficiency in canalicular membrane transport • drug induced canalicular membrane dysfunction • hepatocelluler damage or toxicity
Decreased intrahepatic bile flow : • inflammatory destruction of intrahepatic bile ducts
Extra hepatic biliary obstruction: • gall stone obstruction of biliary tree • extra hepatic biliary atresia • biliary stricture & choledochal cyst • primary sclerosing cholangitis • liver fluke infestation • carcinoma
HISTORY • onset / duration • pain • nausea & vomiting • loss of weight • itching • color of stool • color of urine • past history • ttt &family history
EXAMINATION • color of skin • severity of jaundice • anemia • liver • spleen • gall bladder • ascites
INVESIGATION • CBC • LFT • Prothrombin time • Alfa feto proteins • UG • SG • U/S • ERCP & PTC • Liver biopsy