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1、cZhao Ruiqiu2014.05 cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU Infantile Hepatitis Syndrome A group of symptom complex which includes hepatocellular jaundice, pathologic physical sign of liver and abnormal liver function occurred in infancy.l Unknown causesl Compli
2、cated causesl（ Europe and America ） neonatal hepatitis cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMUsimilar characteristics occurrence in infancyjaundiceabnormal liver functionnutritional disturbancehepatomegaly anti-HCV Ruv-IgM; HSV-IgM cDivide of gastroenterology an
3、d infectious diseases, Childrens hospital, CQMU I. Examination of virology.II. Bacteriologic tests blood culture , urine culture III. Other pathogens tests ATAB Treponema pallidum antigens/antibodies Examinations of infections cDivide of gastroenterology and infectious diseases, Childrens hospital,
4、CQMUBiliary atresia, Cholangiectasis, Caroli disease, etcmetabolic 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
5、abnormalOtheretiologies Disorders of metabolismIHSetiology cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU Malformations of the bile ducts 1. Biliary atresia2. Congenital cholangiectasis classification： Intrahepatic & extrahepatic etiology： congenital infection congenit
6、al anomaly bile ducts 3.Caroli disease4. Alagille syndrome cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU lsimilar clinical feature in abnormal bile duct relevance disease： pale stool， dark urine Conjunctive hyperbilirubinemia increased AKP, -GT level cDivide of gastro
7、enterology and infectious diseases, Childrens hospital, CQMU 1. Biliary atresia2. Congenital cholangiectasis classification： Intrahepatic & extrahepatic etiology： congenital infection congenital anomaly bile ducts 3.Caroli disease4. Alagille syndromeMalformations of the bile ducts cDivide of gastroe
8、nterology and infectious diseases, Childrens hospital, CQMUCaroli disease cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU 1. Biliary atresia2. Congenital cholangiectasis classification： Intrahepatic & extrahepatic etiology： congenital infection congenital anomaly bile d
9、ucts 3.Caroli disease4. Alagille syndromeMalformations of the bile ducts cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMUAlagille syndrome cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU Examinations of abnormal bile ducts lImaging investiga
10、tions B mode ultrasound Magnetic Resonance Cholangiopancreatography (MRCP) X-Ray lRadioisotope scanning (Technetium-99 )lEndoscopic Retrograde Cholangiopancreatography ERCP lLiver biopsy cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMUradioisotope scanning cDivide of gas
11、troenterology and infectious diseases, Childrens hospital, CQMU Examinations of abnormal bile ducts lImaging investigations B mode ultrasound MRCP X-RaylRadioisotope scanning (Technetium-99 )lEndoscopic Retrograde Cholangiopancreatography ERCP lLiver biopsy cDivide of gastroenterology and infectious
12、 diseases, Childrens hospital, CQMUERCP cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU Examinations of abnormal bile ducts lImaging investigations lRadioisotope scanning (Technetium-99 )lEndoscopic Retrograde Cholangiopancreatography ERCP lLiver biopsy cDivide of gastr
13、oenterology and infectious diseases, Childrens hospital, CQMUBiliary obstruction Alagille syndrome cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMUBiliary atresia, Cholangiectasis, Caroli disease, etcmetabolic abnormalities of carbohydrate ,amino-acid, protein, lipid, bi
14、lirubin, bile acid etc Hepatic primary infection & hepatic involvement of general infection Disorders of metabolisminfection Unclear etiology and classifications Intrahepatic & extrahepatic duct abnormalOtheretiologies IHSetiology cDivide of gastroenterology and infectious diseases, Childrens hospit
15、al, 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 e
16、g： Niemann-Pick disease Gaucher ， s disease Metabolic disorders cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU 4. Disorders of bilirubin metabolism Gilbert syndrome Crigler-Najjar syndrome Rotor syndrome Dubin Johnson syndrome5. Disorders of bile acid metabolism Progre
17、ssive Familial Intrahepatic Cholestasis, PFIC6. Other disorders of metabolism Alpha-1 antitrypsin deficiency Wilson disease HemochromatosisMetabolic disorders cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU Phenotype Current condition name Gene defectInfantile low GGTCh
18、olestasis PFIC 1 FIC1-ATP8B1Infantile low GGTCholestasis BSEP deficiency PFIC 2 ABCB11Infantile Cholestasis high GGT MDR3 deficiency PFIC 3 ABCB4progressive familial intrahepatic cholestasis, PFIC cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU 4. Disorders of bilirubin
19、 metabolism Gilbert syndrome Crigler-Najjar syndrome Rotor syndrome Dubin Johnson syndrome5. Disorders of bile acid metabolism Progressive Familial Intrahepatic Cholestasis, PFIC6. Other disorders of metabolism Alpha-1 antitrypsin deficiency Wilson disease HemochromatosisMetabolic disorders cDivide
20、of gastroenterology and infectious diseases, Childrens hospital, CQMU lMetabolism screening fasting blood glucose ,blood gas analysis ,blood ammonia , blood lactate , blood lipid , AFP, ceruloplasmin , serum protein electrophoresis lTandem mass spectrometry lLiver biopsy light microscopy and electro
21、n microscopy lGene analysis Examinations of metabolism cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMUGSD-4 Hemochromatosis Niemann-Pick Disease cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU lMetabolism screening fasting blood glucose ,bl
22、ood gas analysis ,blood ammonia , blood lactate , blood lipid , AFP, ceruloplasmin , serum protein electrophoresis lTandem mass spectrometry lLiver biopsy light microscopy and electron microscopy lGene analysis Examinations of metabolism cDivide of gastroenterology and infectious diseases, Childrens
23、 hospital, CQMU cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMUIHSetiology Biliary atresia, Cholangiectasis, Carol
24、i disease, etcmetabolic 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 abnormalOtheretiolo
25、gies cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU lDiagnosis of IHS： Four factorsletiological diagnosis ： important but difficult based on medical history clinical manifestation laboratory examinations cDivide of gastroenterology and infectious diseases, Childrens ho
26、spital, CQMU cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU 1. Etiological treatment 2. Symptomatic and supportive treatment 3. Chinese medicine treatment 4. Liver transplantation cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU 概 述 定 義 （ 重 點 、 掌 握 ）病 因 及 發(fā) 病 機 理 (難 點 、 了 解 )臨 床 表 現 （ 共 同 點 重 點 、 掌 握 ） 巨 細 胞 病 毒 感 染 的 臨 床 表 現 （ 掌 握 ）實 驗 室 檢 查 （ 了 解 ）診 斷 （ 重 點 、 掌 握 ） 治 療 （ 了 解 ）Sub titleSummary cDivide of gastroenterology and infectious diseases, Childrens hospital, CQMU
《兒科學教學課件》18、infantilehepatitissyndrome(上)
