Cystic biliary atresia: an etiologic and prognostic subgroup
Cystic biliary atresia: an etiologic and prognostic subgroup
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DOI：
10.1016/j.jpedsurg.2007.12.058
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发表时间：
2008-09-01
影响因子：
2.4
通讯作者：
Davenport, Mark
中科院分区：
文献类型：
--
作者：
Caponcelli, Enrica;Knisely, Alex S.;Davenport, Mark
摘要：囊性胆道闭锁（CBA）是胆道闭锁（BA）的一种少见的变型，其预后相对较好，但容易误诊为胆总管囊肿，可能为胆道闭锁的病因研究提供新的思路。由于有些病例可以在产前检测到，CBA一般可能起源于子宫内的生命。我们评估了我们在CBA的经验。方法：对1994年1月至2006年12月13年间的CBA患儿进行单中心回顾性分析。数据以中位数（范围）给出。结果：270例BA患儿中29例（男9例）确诊为CBA。在平均妊娠22周（17-34周）的12例（41%）婴儿中，产前超声检查发现所有异常。所有婴儿都接受了产后切除和Kasai门肠造口术（KP）。产前检测到CBA的患者手术时间较早（36天[14-67天]vs 48天[35-147天]，P = 0.004）。20个囊肿（69%）有纤维炎壁，没有胆上皮，6个（26%）含有胆汁。根据Q时获得的肝活检材料评估，KP时的年龄与肝纤维化呈显著正相关（r = 0.46, P = 0.01），但与“肝细胞紊乱”等级无关（P = 0.74）。20名婴儿（69%）清除了黄疸（胆红素）
Introduction: Cystic biliary atresia (CBA) is an uncommon variant of biliary atresia (BA) in which prognosis may be relatively favorable but liable to misdiagnosis as choledochal cyst, and potentially offers insights into the etiology of BA. Because some cases can be detected antenatally, CBA in general may have its origins in utero, life. We assessed Our experience with CBA.Methods: Single-center retrospective review of infants with CBA over a 13-year period (January 1994 to December 2006) was done. Data are given as medians (range).Results: Of 270 infants with BA, 29 (9 male) were identified as CBA. Antenatal ultrasonography had detected ail abnormality in 12 (41%) infants at a median of 22 weeks (17-34 weeks) of gestation. All infants underwent postnatal excision and Kasai portoenterostomy (KP). Those with antenatally detected CBA came to surgery younger (36 [14-67] vs 48 days [35-147 days], P =.004). Twenty cysts (69%) had a fibroinflammatory wall with no biliary epithelial lining and 6 (26%) contained bile. Age at KP was significantly and positively correlated (r = 0.46, P = .01) with liver fibrosis, as assessed in liver biopsy materials obtained at Q but not with grade of "hepatocyte disarray" (P = .74), Twenty infants (69%) cleared their jaundice (bilirubin