Maternal microchimerism in biliary atresia
Maternal microchimerism in biliary atresia
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DOI：
10.1016/j.jpedsurg.2007.01.051
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发表时间：
2007-06-01
影响因子：
2.4
通讯作者：
Gittes, George K.
中科院分区：
文献类型：
--
作者：
Kobayashi, Hiroyuki;Tamatani, Takuya;Gittes, George K.
背景：本研究的目的是确定胆道闭锁（BA）患者肝脏中母体微嵌合的存在和程度。方法：根据研究对象的性别进行两组调查。系列1的研究对象为男性，其中6人拥有文学学士学位。用X和Y染色体探针和荧光原位杂交分析肝脏。第二组的受试者为女性。9例BA患者及其母亲均为HLA 1型。对女儿的肝脏也进行了母体和其他HLA抗体的检测。新生儿肝炎2例，Alagille综合征2例，Byler综合征1例作为对照。结果：所有男性BA肝脏均含有I和2 X染色体（即XY或XX）的混合细胞。所有男性对照的肝脏只有1条X染色体（ic， XY）。所有女性BA受试者的胆管上皮和肝细胞中均存在不同强度的抗母体HLA I类（HLA- a）抗体（强，5，-轻度，3；弱，1）。女性对照组的肝脏未显示任何抗母体HLA I类抗体（HLA- ab）。结论：我们的初步数据似乎表明母体微嵌合存在于进行性产后BA患者的肝脏中。我们认为BA实际上可能是一种移植物抗宿主病，伪装成由母体微嵌合引发的自身免疫反应，我们打算进一步研究这一假设，以澄清BA的病因。(c) 2007爱思唯尔公司版权所有。
Background: The aim of this study was to determine the existence and extent of maternal microchimerism in the livers of biliary atresia (BA) patients.Methods: Two series of investigations were performed based on the sex of our subjects. Subjects for series I were men, of which 6 had BA. Livers were analyzed using X and Y chromosome probes and fluorescent in situ hybridization. Subjects for series II were woman. Nine BA cases and their mothers were HLA typed (class 1). Daughter livers were also tested for antibodies to maternal and other HLA. Two cases of neonatal hepatitis, 2 cases of Alagille syndrome, and I case of Byler syndrome acted as controls.Results: All male BA livers were found to contain a mixture of cells with I and 2 X chromosomes (ie, XY or XX). All livers from male controls had only 1 X chromosome (ic, XY). All female BA subjects had varying intensities of antimaternal HLA class I (HLA-A) antibodies in their bile duct epithelium and hepatocytes (strong, 5,- mild, 3; weak, 1). The liver from the female control did not display any antimaternal HLA class I antibodies (HLA-Ab).Conclusion: Our preliminary data appear to show that maternal microchimerism is present within the livers of patients with progressive postnatal type BA. We suggest that BA could in fact be a graft-vs-host disease masquerading as an autoimmune reaction triggered by matemal microchimerism, and we intend to pursue this hypothesis further to clarify the etiology of BA. (c) 2007 Elsevier Inc. All rights reserved.