Ando K, Miyano T, Fujimoto T, Ohya T, Lane G, Tawa T, Tokita A, Yabuta K
Department of Pediatric Surgery, Juntendo University, School of Medicine, Tokyo, Japan.
J Pediatr Surg. 1996 Sep;31(9):1302-4. doi: 10.1016/s0022-3468(96)90259-6.
Familial occurrence of extrahepatic biliary atresia (EHBA) or biliary dilatation has been reported; however, the genetic influences on these disorders are still obscure. The authors previously reported the occurrence of biliary dilatation and EHBA in sisters, which is extremely rare. Recently, a similar pair of sisters was treated; one had pancreaticobiliary maljunction with minimal dilatation of the common bile duct, and the other also had EHBA. The authors' investigations suggest that there may be common etiologic factors for EHBA and biliary dilatation, such as pancreaticobiliary maljunction; however, other factors are required to complete the process and cause biliary atresia.
已有报道肝外胆管闭锁（EHBA）或胆管扩张存在家族聚集性；然而，这些疾病的遗传影响仍不清楚。作者之前报道过姐妹俩均发生胆管扩张和EHBA的情况，这极为罕见。最近，又有一对类似的姐妹接受了治疗；其中一人患有胰胆管合流异常，胆总管仅有轻微扩张，另一人则患有EHBA。作者的研究表明，EHBA和胆管扩张可能存在共同的病因，如胰胆管合流异常；然而，还需要其他因素来促使疾病发展并导致胆管闭锁。
