首页 | 本学科首页   官方微博 | 高级检索  
     


Congenital Structural Abnormalities in Biliary Atresia: Evidence for Etiopathogenic Heterogeneity and Therapeutic Implications
Authors:THEMIS R. SILVEIRA  FRANCISCO M. SALZANO  EDWARD R. HOWARD  ALEX P. MOWAT
Affiliation:Departments of Child Health, King's College Hospital, London, UK;Pediatrics Child Health, King's College Hospital, London, UK;Genetics, Federal University of Rio Grande do Sul, Porto Alegre, Brazil and Departments of Child Health, King's College Hospital, London, UK;Surgery and Child Health, King's College Hospital, London, UK
Abstract:ABSTRACT. The clinical, surgical, laboratory and histological data of 237 children with extrahepatic biliary atresia were reviewed. Forty-seven patients (20%) had associated congenital anomalies, and of these, 28 had cardiovascular, 22 digestive and 19 splenic malformations. Of the 19 patients with splenic malformations, 13 showed the polysplenia syndrome and two had asplenia. Chromosome studies were performed in eight children, six having associated anomalies, and two of them showed karyotype abnormalities (46,XX,del 18 p- and 49,XXXXY). These observations indicated that biliary atresia could be subdivided into four distinct etiopathogenic subgroups, three involving a congenital form that could arise through a malformation, a disruption or a chromosome abnormality, and the remaining to agents active in the perinatal period (the acquired form). The surgical outcome in 171 patients operated on by an experienced surgeon was not influenced by the presence of anomalies but by the timing of surgery. Seventy-one percent of 24 patients operated on by 8 weeks of age were jaundice-free as opposed to only 34% of those who had later surgery ( p <0.01)
Keywords:biliary atresia    neonatal jaundice    congenital malformations    surgery of biliary atresia
设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司  京ICP备09084417号