胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,胆盐代谢及转运和肝内胆汁淤积,分子医学和临床的相互促进,王建设,复旦大学附属儿科医院,复旦大学儿童肝病中心,胆盐代谢及转运和肝内胆汁淤积,1,特发性”新生儿肝炎,GGT and the outcome,July 1, 1981-Jan 1, 1985, 186 infants, 29,diagnosed as INHS, followed up for at least,1 year, or until death,17 with increased GGt(=2.1 normal,upper limit), All but 1 in good prognosis,12 with normal gGt, All poor prognosis,Maggiore G, et al.J Pediatr, 1987: 112: 251-252,特发性”新生儿肝炎,2,Kings病例入选标准,Aug 1991 to Nov 2000, Conjugated,hyperbilirubinemia under 3 months of,age(973 cases),No specific etiologic factor can be,ascertained after comprehensive work,up,Followed up for at least one year or until,died,Kings病例入选标准,3,Table l conjugated hy perbilirubinaemia workup,Blood Tests,Pyruvate,Radiology,Full blood count,Lactate,Ultrasonography of,Reticulocyte count,CL-l-antitrypsin phenotype hepatobiliary system,Direct coombs test,Serum iron and ferritin,pine x-ray,cultures,Complement levels,TORCH Screen,Autoantibodies,Others,Adenovirus,Serum amino acid,Liver biopsy,Hepatit is A and B serology Immuno-reactive trypsin Opthalmology examinat ion,Free thyroxine and thyroid Plasma very long chain,Sweat test,stimulating hormone level fattv acid,Cortisol level,Where indicated,Galactose-1-phosphate,Urine Tests,Bone marrow aspiration,uridyl transferase activity Urine cultures,Muscle biopsy,Ammonia,id,Urine bile acids,Wang JS, Eur J Pediatr, 2019, in press,Table l conjugated hy perbilir,4,病例排除标准,INR1.2 and not be fully corrected after,vitamin K injection,Follow up interval longer than 3 months,Other severe congenital abnormalities,G6PD deficiency,Evidence of active CMV infection in,spite of no inclusion found on liver,biopsy,uss demonstrated bile duct dilation,病例排除标准,5,Basic information,128 cases elected, 110 biopsyed,6 patients diagnosed as PFIC l or 2,1,recurred jaundice,GGTlevel,with endpoints without endpoints,Presentation 29-84,52.9%100,Peak,36-93,13.2%15%,腹部B超、同位素肝胆显像、遗传代谢病筛査等除,外先天性胆道闭锁、胆道扩张及其他先天性异常,(CMV指标阳性,但无多系统受累的不除外),专科门诊随访至黄疸消退、死亡或一年以上,ekyy入选标准,9,结果,最终有38例患者符合以上条件,入院时的一GT按50U进行分组,50U组6例,5例预后不良,50UL组32例,3例预后不良(P=0001),峰值-GT100U进行分组,100U组28例,2例预后不良(P=0.002),血清GGT水平和预后的有关(和CMV状态无关),王中林.肝脏2019,(4),结果,10,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,11,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,12,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,13,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,14,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,15,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,16,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,17,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,18,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,19,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,20,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,21,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,22,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,23,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,24,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,25,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,26,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,27,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,28,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,29,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,30,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,31,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,32,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,33,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,34,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,35,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,36,胆盐代谢及转运及肝内胆汁淤积分子医学及临床的相互促 ppt课件,37,