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Heme Catabolism & Jaundice

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Heme Catabolism & Jaundice Click to edit Master title style • Edit Master text styles • Second level • Third level Heme• Fourth level catabolism • Fifth level Dr. Marian Maher Lecturer of Biochemistry and Molecular Biology Catabolism of Heme After RBCs reach the end of their life span ( average 120 days), they are phagocytosed by reticulo- endothelial cells of liver, spleen and bone marrow. Hemoglobin is degraded first into globin and heme 1-Oxidation of Heme : a- Heme is degraded by microsomal enzyme; of the reticuloendothelial cells of liver, spleen and bone marrow which requires molecular oxygen and NADPH b- It catalyzes the cleavage of α methenyl bridge between the pyrrole rings I and II to from biliverdin with the release of carbon monoxide CO. c- Iron is liberated from heme d- In mammals, biliverdin is further reduced to bilirubin by NADPH – dependent biliverdin reductase. 2- Transport of Bilirubin: Bilirubin is slightly soluble in plasma. It transported to the liver by non covalent binding to albumin forming hemobilirubin (unconjugated or indirect bilirubin). Liver uptake bilirubin by carrier mediated transport. 3- Uptake of bilirubin by liver: The solubility of bilirubin increase by conjugating with 2 molecules of glucuronic acid to form bilirubin diglucuronide (conjugated or direct bilirubin). This reaction is catalyzed by glucuronosyl transferase enzyme 4- Excretion of conjugated bilirubin: The conjugated bilirubin secreted with the bile into intestine (the unconjugated bilirubin not 90% secreted) where it is hydrolyzed and reduced by By bacteria the gut bacteria to urobilinogen (colorless 10% compound). Most of urobilinogen is oxidized by intestinal bacteria to stercobilin, which give feces the characteristic brown color. Some of urobilinogen is reabsorbed from the gut and enters the portal blood and resecreted to the kidney, where it is converted to urobilin that gives the urine the characteristic yellow color urobilin bacterial oxidation stercobilin Hyperbilirubinemia The normal plasma bilirubin level range from 0.3 – 1 mg/dl . If the serum bilirubin exceeds 1 mg/dl, the condition is called hyperbilirubinemia. If the bilirubin level exceeds 2 mg/dl. Jaundice will occur with yellowish discoloration of sclera, conjunctiva and skin. The sclera is particularly affected because it is rich in elastin, which has a high affinity for bilirubin. Note that: • unconjugated bilirubin can cross the blood-brain barrier into the central nervous system so encephalopathy due to hyperbilirubinemia (kernicterus) thus occurs only with unconjugated bilirubin. • Alternatively, because of its water-solubility, only conjugated bilirubin can appear in urine. ➢ choluric jaundice (choluria is the presence of bile pigment in the urine) occurs only in regurgitation conjugated hyperbilirubinemia, ➢ acholuric jaundice occurs if only the presence of an excess of unconjugated bilirubin. conjugated Type of bil. unconjugated Classification of Hyperbilirubinemia Prehepatic Site of Hepatic defect Posthepatic © Type of Bilirubin Unconjugated Conjugated Neonatal “physiological jaundice” Obstruction of the biliary tree Hemolysis Hepatic damage Gilbert syndrome Crigler-Najjar syndromes types I & II Hepatic damage Site of defect Prehepatic Hepatic Posthepatic Obstruction of the Hemolytic Hepatitis biliary tree Neonatal jaundice” Crigler-Najjar syndromes I & II Gilbert syndrome 1) Hemolytic Jaundice Due to: In neonates Rh incompatibility between maternal and fetal blood. In children and even in adult from enzyme deficiency of G-6-P dehydrogenase or pyruvate kinase or sickle cell anemia. Extensive hemolysis produce bilirubin faster than it can be conjugated UCB levels in the blood become elevated more CB is made and excreted into the bile, the amount of urobilinogen entering the enterohepatic circulation is increased, and urinary urobilin and stercobilin is increased causing jaundice, normal colour of urine and stool 2) Neonatal “Physiological Jaundice” This is transient hyperbilirubinemia due to accelerated rate of destruction of RBCs and to the immature hepatic system of conjugation. Elevated UCB, in excess of the binding capacity of albumin (20–25 mg/dl) Causing diffuse into the basal ganglia, cause toxic encephalopathy (kernicterus) ttt Phototherapy (converts bilirubin to more polar water-soluble isomers) and barbiturates (promoter of bilirubin-metabolism) 3) Hepatocellular Jaundice due to cirrhosis or hepatitis Results in: Damage to liver cells can cause UCB levels in the blood to increase as a result of decreased conjugation Inflammatory oedema of hepatocytes will compress the intracellular canaliculi “mild obstruction” causing increased CB Urobilinogen decreased if micro-obstruction is present Causing the urine consequently darkens, whereas stools may be a pale, clay color 4) Crigler-Najjar syndrome Type I Crigler-Najjar: autosomal recessive disorder due to mutations in the gene encoding bilirubin-UGT (UDP- glucuronyl-transferase)activity in hepatic tissues. serum UCB usually exceeds 20 mg/dL It is characterized by severe congenital jaundice ttt Phototherapy reduces plasma bilirubin levels somewhat, but phenobarbital has no effect. Type II (UDP-glucuronyl-transferase), have some activity and the condition has a more benign course than type I. Serum UCB concentrations usually do not exceed 20 mg/dl,ttt: patients respond to treatment with large doses of phenobarbital 5) Gilbert’s disease due to congenital defect in conjugation by hepatocytes (70– 80% reduction) and 30% of the bilirubin UDP-glucuronosyl transferase activity is retained in Gibert syndrome the condition is harmless. 6) Obstructive Jaundice Due to obstruction of the common bile duct as in Biliary cirrhosis – hepatoma - Gall stones- Cancer head of pancreas “The most severe form of Jaundice, bilirubin > 30 mg/dl”. Preventing passage of CB into the intestine The liver “regurgitates” CB into the blood (hyperbilirubinemia). The CB is eventually excreted in the urine (which darkens upon standing), and is referred to as “urinary bilirubin.” Urinary urobilinogen is absent. Causing GI pain and nausea and produce stools that are a pale, clay color. Prehepatic jaundice Hepatic jaundice Post hepatic Increase bilirubin Defect in conjugation and/or jaundice production excretion of bilirubin in bile Obstruction of bile duct Causes: 1.hemolysis of RBCs as in Hepatic damage 1.Gilbert syndrome biliary stones sickle cell anemia, e.g; 2.Crigler-najjar cancer head of pancreas G6PD deficiency and RH Hepatitis syndrome incompatibility 2.Neonatal jaundice Type of elevated Indirect (unconjugated) Indirect + direct Indirect direct (conjugated) bilirubin Urine 1.Presence of Absent Present Present conjugated bilirubin (acholuric) (choleric) (choluric) (dark colored urine) Normal color 2.urobilinogen increase Decrease Absent Stool 1.Color and consistency Normal Pale clay colored Pale clay colored and 2.stercobilin bulky(steatorrhea) increase decrease absent Blood test: Serum ALT and AST Normal Increased Normal Serum ALP Normal Normal Increased Blood hemoglobin Low …… ………. Reticulocyte count Elevated …….. ……………. Serum bile salts …….. ……. Present (itching) Serum cholestero Normal Normal Increased .
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