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1、,*,單擊此處編輯母版標題樣式,單擊此處編輯母版文本樣式,第二級,第三級,第四級,第五級,單擊此處編輯母版標題樣式,單擊此處編輯母版文本樣式,第二級,第三級,第四級,第五級,*,Jaundice(,黃疸,),Department of Gastroenterology,First Affiliated Hospital of Chongqing Medical University,XIAOHONG TAO(,陶小紅,),Jaundice or icterus:,What is jaundice?,Clinically it is the yellow,discoloration,of the
2、,Sclera,(,鞏膜,),Skin,Mucous membrane,due to deposition of bile pigment,(,bilirubin,膽紅素,),Serum total bilirubin(normal 1.7-17.1 umol/lit),Conjugated bilirubin:0-3.42mol/l,,,Unconjugated bilirubin:1.7-13.68mol/l.,1.DEFINITION,2.BILIRUBIN METABNISM,Heme Degradation,HEME,BILIVERDIN,O,2,Fe,+3,NADPH,NADP,+
3、,(opens the porphyrin ring),BILIRUBIN,NADPH,NADP,+,BILIRUBIN diglucuronide,BILE,The Fate of Bilirubin,Alb=albuminB=bilirubinGST=glutathione-S-transferase,UDPGA=uridine diphosphoglucuronic acid;CB=conjugated bilirubin,UGT1A1=UDP-glucuronosyltransferase 1A1,MRP2=Multi-drug Resistance Protein 2,Adapted
4、 from Harrisons 15th Ed.“Principles of Internal Medicine,2001.,MRP2,B,+GST,CB,Plasma,Hepatic Cell,Bile,Alb,B,Alb,?,:GST,B,sER,B,+,UDPGA,UGT1A1,Bilirubin Excretion,Intestines,Liver,B,CB,CB,B,Urobilinogen,B-glucoronidase,bacteria,bacteria,Bile,Enterohepatic circulation,Kidney,Urobilin,ox,Urobilinogen,
5、Urobilin,Stercobilin,Stercobilingogen,feces,Urine,ox,Liver,Albunin,Heme,Ferroheme,transferase,Bilirubin,Conjugated bilirubin,Kidney,Urobilin,Stercobilinogen,Urobilinogen,Intestines,Albumin,Bilirubin,reductase,Heme,Globin,Hemoglobin,oxidase,Biliverdin,Marrow,Infantility Erythrocyte,Ferroheme Enzyme,1
6、5%20%,Bilirubin,|,Albumin,Erythrocyte,80%85%,UDP,UDPGA,Bilirubin glucuronolactone,urobilinogen,Enterohepatic circulation,Y/Z protein,cathepsin,Erythrocyte,Biochemical change of Normal bilirubin metabolism,3.CLASSFICATION,1.Depending on Etiology,Hemolytic Jaundice,溶血性黃疸,Hepatocellular Jaundice肝細胞性黃疸,
7、Cholestatic Jaundice膽汁淤積性黃疸,Congenital jaundice,先天性黃疸,2.Depending on bilirubin,Unconjungated bilirubin increased jaundice,Conjungated bilirubin increased jaundice,Etiology,1.Congenital hemolytic anemia(,thalassemia(,海洋性貧血,),hereditary spherocytosis(,遺傳性球形紅細胞增多癥,).,2.P,osteriority acquired hemolytic
8、anemia(autoimmunity hemolytic anemia,hemolytic disease of newborn,posttransfusion hemolytic,Favism(,蠶豆病,),.,Mechanism,A large number of erythrocyte destroyed rapidly,Anemia,hypoxia and toxity of erythocyte metabolism products,3.1.Hemolytic Jaundice,Hemolytic Jaundice mechanism,Clinical Manifestation
9、,Mild jaundice,light lemon,no skin itch(,癢,).,Acute hemolysis:fever,chill,headache,vomit,backache,anemia,hemoglobinuria(,血紅蛋白尿,),acute renal failure.,Chronic hemolysis:anemia and splenomegaly(,脾大,).,Laboratory Examination,1.Serum TB,UCB,CB normal.,2.UCBintestinal CBfaecal color deepen.,3.Intestinal
10、Urobilinogen(,尿膽原,)urinary Urobilinogen.,4.Acute hemolytis,occult blood test(+).,5.Blood test:anemia,reticulocyte(,網(wǎng)織紅細胞,),erythrocyte proliferation.,3.2.Hepatocellular Jaundice,Etiology,Hepatocyte damage.,Mechanism,Impair hepatocyte uptakeing,conjugating and excreting bilirubin,UCB,CB reflux into b
11、lood,serum CBjaundice.,Hepatocellular Jaundice mechanism,Clinical Manifestation,Skin and mucosa:light to deep yellow,mild skin itch,(,癢,),Tired,loss of appetite,hemorrhagic tendency,ascites,coma.,Primary disease manifestation,Laboratory Examination,CB,UCB,Icteric hepatitis:CB UCB,Urine:CB(+),urobili
12、nogen,Blood test:liver damaged,3.3.Cholestatic Jaundice,Etiology,Obstruction in liver:virus hepatitis,drug caused,drug hepatitis,primary biliary cirrhosis.,Obstruction of Extrahepatic bile duct:narrow,obstruction,stone,inflammtory,tumor.,Mechanism,Obstruction causes small bile duct and bile capillar
13、y broken,conjugated bilirubin reflux to blood.,Failure of hepatocyte excreting CB,bilirubin deposit and bile thrombus(,膽栓,)formation.,Cholestatic Jaundice mechanism,Clinical Manifestation,Skin dark yellow,yellow green.,Skin itch,(,癢,),bradycardia(,心動過緩,),dark yellow of urine,faecal light yellow or c
14、lay color.,Courvoisiers sign.,Laboratory Examination,Serum CB,Urine bilirubin(+),Urobilinogen,stercobilin(,糞膽原,)or absence,Serum alkaline phosphatase and Cholesterol(,膽固醇,),A.Hemolytic anemia,excess,hemolysis,unconjugated bilirubin,(in blood),conjugated bilirubin,(released to bile duct),UB,Urobilino
15、gen,B.Hepatitis,unconjugated bilirubin,(in blood),conjugated bilirubin,(in blood),UB,Urobilinogen,C.Biliary duct stone,unconjugated bilirubin,(in blood),conjugated bilirubin,(in blood),UB,Urobilinogen,5.Genetic Disorders of Bilirubin Metabolism,Condition,Defect,Bilirubin,Clinical,Findings,Crigler-Na
16、jjar syndrome,severely defective,UDP-glucuronyl transferase,Unconjugated bilirubin,Profound jaundice,Gilberts syndrome,reduced activity of,UDP-glucuronyl transferase,Unconjugated bilirubin,Very mild jaundice during illnesses,Dubin-Johnson syndrome,abnormal transport of conjugated bilirubin into the biliary system,Conjugated bilirubin,Moderate jaundice,Rotors syndrome,deficiency of uptaking UCB,and excreting CB,CB,jaundice.,Crigler-Najjar syndrome,Gilberts syndrome,Three kinds of jaundice laborot