《儿科学教学课件》18、infantile hepatitis syndrome（上）.ppt

2014.05,Infantile Hepatitis Syndrome 婴儿肝炎综合征,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Infantile Hepatitis Syndrome,Definition,Infantile Hepatitis Syndrome, IHS A group of symptom complex which includes hepatocellular jaundice, pathologic physical sign of liver and abnormal liver function occurred in infancy. Unknown causes Complicated causes （Europe and America ） neonatal hepatitis,/diseases/hepatitis_neonatal.htm,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,similar characteristics,occurrence in infancy,jaundice,abnormal liver function,Clinical manifestations,nutritional disturbance,hepatomegaly & splenomegaly hepatic architecture abnormality,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Unconjugated hyperbilirubinemia,Conjugated hyperbilirubinemia,Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,similar characteristics,jaundice,abnormal liver function,Clinical manifestations,nutritional disturbance,hepatomegaly & splenomegaly hepatic architecture abnormality,occurrence in infancy,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Clinical manifestations,Hepato-splenomegaly,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,similar characteristics,jaundice,abnormal liver function,Clinical manifestations,nutritional disturbance,hepatomegaly & splenomegaly hepatic architecture abnormality,occurrence in infancy,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Serum bilirubin Serum transaminase Bile acids TP, ALB PT,TB、DB ALT，AST，-GT，AKP,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,similar characteristics,jaundice,hepatomegaly & splenomegaly hepatic architecture abnormality,Clinical manifestations,nutritional disturbance,abnormal liver function,occurrence in infancy,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,患儿，男，2月10天，以“发现皮肤黄染一月余”入院。自生后半月左右发现皮肤发黄，持续至今，无明显进行性加重，亦无减轻及波动，大便色淡黄，小便色如广柑水色。病程中无发热、咳嗽，无腹泻、腹胀，无皮肤出血点，无呕吐拒食。G2P1，产重3.5Kg。母乳及奶粉混合喂养至今。查体：W 5Kg 反应可，哭声大。皮肤巩膜中-重度黄染，心肺听诊正常，腹部饱满，肝肋下3.5cm，剑下4.5cm,质中边钝，脾脏肋下2cm，肛门及四肢正常。 院外肝功结果：TB 206.5mol/L DB 123 mol/L ALT 169U/L AST 233U/L.,举 例,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Metabolic disturbance,Bronchitis or pneumonia,Congenital malformation,Nervous system damage,Eye diseases: cataract,Clinical manifestations,Other Features,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,IHS etiology,Biliary atresia, Cholangiectasis, Caroli disease, etc,metabolic abnormalities of carbohydrate ,amino-acid, protein, lipid, bilirubin, bile acid etc,Hepatic primary infection & hepatic involvement of general infection,infection,Unclear etiology and classifications,Intrahepatic & extrahepatic duct abnormal,Etiology and Clinical manifestations,Other etiologies,Disorders of metabolism,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,IHS etiologic classifi-cation,Biliary atresia, Cholangiectasis, Caroli disease, etc,metabolic abnormalities of carbohydrate ,amino-acid, protein, lipid, bilirubin, bile acid etc,Hepatic primary infection & hepatic involvement of general infection,infection,Unclear etiology and classifications,Intrahepatic & extrahepatic duct abnormal,Etiology and Clinical manifestations,Other etiologies,Disorders of metabolism,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Viral Infection 1. Cytomegalovirus, CMV 2. HAV、HBV、HCV 3. Rubella virus，RV 4. Herps simplex virus, HSV 5. Epstein-Bar virus, EBV 6. Enterovirus,感染因素,Etiology and Clinical manifestations,Infective factors,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,viral Infection bacterial Infection 1. pathogenic bacteria staphylococcus aureus, escherichia coli, Salmonella, staphylococcus epidermidis,streptococcus etc 2. common route of infection umbilical region, respiratory tract, urinary tract etc,感染因素,Etiology and Clinical manifestations,Infective factors,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Omphalitis,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,.viral Infection .bacterial Infection .Other agents infection toxoplasm, treponema pallidum, fungus, HIV,感染因素,Infective factors,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Cytomagalovirus Infection,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Cytomagalovirus Infection,Human Cytomegalovirus （HCMV） double-stranded linear DNA virus - Family: Herpesviridae - Subfamily: - Latency-activation,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Infectious sources infected people Routes of transmission 1. mother-to-child transmission (MTCT) Congenital infection （ infection confirmed in 2 weeks after birth） Perinatal infections （ infection confirmed from 3 to 12 weeks after birth. ） Postnatal infection （ infection confirmed after 12 weeks ） 2. horizontal transmission: close contact , iatrogenic transmission Population susceptibility,Cytomagalovirus Infection,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Tissue tropism of HCMV target cell ：epithelial cell , endothelial cell, fibroblast Different types of HCMV infection active infection latent infection cell transformation partial infection Weaker virulence,Cytomagalovirus Infection,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,cytomegalic inclusion owl eye sign,Cytomagalovirus Infection,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,. Congenital infection 1. In early trimester of pregnancy： dead fetus, dead birth, or abortion 2. In mid or late trimester of pregnancy: （1）various malformations （2）multi-system involvement （3）more sequelae , poor prognosis,Cytomagalovirus Infection,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,congenital cytomegalovirus infection,Cytomagalovirus Infection,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Cytomagalovirus Infection,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,.Congenital infection .Perinatal infections and postnatal infection hepatitis ： jaundice（DB）、hepatosplenomegaly、 liver function damage (mild or moderate ) interstitial pneumonia ： prolonged courses , several months less sequelae hepatic cirrhosis can happened,Cytomagalovirus Infection,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Examination of virology 1. Evidences of CMV active infection CMV-IgM in serum positive The titer of CMV-IgG increased over 4 times Cytomegalic inclusion is found in tissue CMV-mRNA positive in body fluid or tissue CMV is isolated in body fluid or tissue.,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Examination of virology. Evidences of CMV active infection 2.HBV (1) HBV: serologic marker of HBV (2) HBV-DNA 3.Other viruses anti-HAV-IgM; anti-HCV HSV-IgM,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Examination of virology. Bacteriologic tests blood culture , urine culture Other pathogens tests ATAB Treponema pallidum antigens/antibodies,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Biliary atresia, Cholangiectasis, Caroli disease, etc,metabolic abnormalities of carbohydrate ,amino-acid, protein, lipid, bilirubin, bile acid etc,Hepatic primary infection & hepatic involvement of general infection,infection,Unclear etiology and classifications,Intrahepatic & extrahepatic duct abnormal,Etiology and Clinical manifestations,Other etiologies,Disorders of metabolism,IHS etiology,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,1. Biliary atresia 2. Congenital cholangiectasis classification： Intrahepatic & extrahepatic etiology：congenital infection congenital anomaly bile ducts 3.Caroli disease 4. Alagille syndrome,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,similar clinical feature in abnormal bile duct relevance disease： pale stool，dark urine Conjunctive hyperbilirubinemia increased AKP, -GT level,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,1. Biliary atresia 2. Congenital cholangiectasis classification： Intrahepatic & extrahepatic etiology：congenital infection congenital anomaly bile ducts 3.Caroli disease 4. Alagille syndrome,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Caroli disease,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,1. Biliary atresia 2. Congenital cholangiectasis classification： Intrahepatic & extrahepatic etiology：congenital infection congenital anomaly bile ducts 3.Caroli disease 4. Alagille syndrome,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Alagille syndrome,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Imaging investigations B mode ultrasound Magnetic Resonance Cholangiopancreatography (MRCP) X-Ray Radioisotope scanning (Technetium-99 ) Endoscopic Retrograde Cholangiopancreatography ERCP Liver biopsy,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,radioisotope scanning,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Imaging investigations B mode ultrasound MRCP X-Ray Radioisotope scanning (Technetium-99 ) Endoscopic Retrograde Cholangiopancreatography ERCP Liver biopsy,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,ERCP,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Imaging investigations Radioisotope scanning (Technetium-99 ) Endoscopic Retrograde Cholangiopancreatography ERCP Liver biopsy,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Biliary obstruction,Alagille syndrome,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Biliary atresia, Cholangiectasis, Caroli disease, etc,metabolic abnormalities of carbohydrate ,amino-acid, protein, lipid, bilirubin, bile acid etc,Hepatic primary infection & hepatic involvement of general infection,Disorders of metabolism,infection,Unclear etiology and classifications,Intrahepatic & extrahepatic duct abnormal,Etiology and Clinical manifestations,Other etiologies,IHS etiology,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,1. Disorders of carbohydrate metabolism eg：Galactosemia : galactose-1-phosphate uridyltransferase Fructose intolerance Glycogenosis Mucopolysaccharidosis 2. Disorders of amino acid metabolism eg：Tyrosinemia Citrin deficiency （NICCD） Citrullinemia (type) 3. Disorders of lipid metabolism eg：Niemann-Pick disease Gaucher，s disease,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,4. Disorders of bilirubin metabolism Gilbert syndrome Crigler-Najjar syndrome Rotor syndrome Dubin Johnson syndrome 5. Disorders of bile acid metabolism Progressive Familial Intrahepatic Cholestasis, PFIC 6. Other disorders of metabolism Alpha-1 antitrypsin deficiency Wilson disease Hemochromatosis,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,progressive familial intrahepatic cholestasis, PFIC,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,4. Disorders of bilirubin metabolism Gilbert syndrome Crigler-Najjar syndrome Rotor syndrome Dubin Johnson syndrome 5. Disorders of bile acid metabolism Progressive Familial Intrahepatic Cholestasis, PFIC 6. Other disorders of metabolism Alpha-1 antitrypsin deficiency Wilson disease Hemochromatosis,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Metabolism screening fasting blood glucose ,blood gas analysis ,blood ammonia , blood lactate , blood lipid , AFP, ceruloplasmin , serum protein electrophoresis Tandem mass spectrometry Liver biopsy light microscopy and electron microscopy Gene analysis,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,GSD-4,Hemochromatosis,Niemann-Pick Disease,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Metabolism screening fasting blood glucose ,blood gas analysis ,blood ammonia , blood lactate , blood lipid , AFP, ceruloplasmin , serum protein electrophoresis Tandem mass spectrometry Liver biopsy light microscopy and electron microscopy Gene analysis,Etiology and Clinical manifestations,Divide of gastroenterology and infectious diseases, Childrens hospital, CQMU,Divide of gastroenterology and infectious diseases, Childrens hospit