Intrinsic factor within parietal cells of patients with juvenile pernicious anemia. A retrospective immunohistochemical study.
Intrinsic factor within parietal cells of patients with juvenile pernicious anemia. A retrospective immunohistochemical study.
复制标题
青少年恶性贫血患者壁细胞内的内在因素。
DOI：
10.1016/s0016-5085(85)80071-8
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发表时间：
1985
期刊：
影响因子：
29.4
通讯作者：
Allen,RH
中科院分区：
文献类型：
--
作者：
Levine,JS;Allen,RH
引起儿童恶性贫血的多种疾病之一，青少年恶性贫血，其特征是正常的酸分泌，正常的胃粘膜组织学，在外源性胃内因子（IF）存在下，钴胺素的肠吸收充足，但从出生起IF的“生产”不足。从刺激胃分泌物中测得的IF的缺乏中推断出生产不足。为了评估免疫原性IF是否普遍存在于青少年恶性贫血受试者的壁细胞内，我们研究了来自最大的儿童恶性贫血报告系列的石蜡包埋活检材料，使用一种具有良好特征的间接免疫过氧化物酶法。初步研究能够在储存长达27年的胃底粘膜活检标本中识别IF。在一项盲法评价中，来自青少年恶性贫血儿童的9份胃底活检标本中有6份表现出免疫原性IF。两组兄弟姐妹的细胞内IF存在或不存在一致，来自伊默斯伦德综合征患者的六份胃活检样本均呈IF阳性。这些研究结果表明，青少年恶性贫血是一组异质性疾病，其相似的临床表现可能是由以下原因引起的：（a）IF合成不足，（B）IF分泌阻滞，（c）分泌不与钴胺素结合的异常IF，或（d）分泌可能包含许多其他功能缺陷的其他异常IF。
One of the diverse group of disorders that cause pernicious anemia in childhood, juvenile pernicious anemia, has been characterized by normal acid secretion, normal gastric mucosal histology, adequate intestinal absorption of cobalamin in the presence of exogenous gastric intrinsic factor (IF), but the inadequate “production” of IF from birth. Inadequate production has been inferred from the absence of measured IF in stimulated gastric secretions. To assess whether immunogenic IF was commonly present within the parietal cells of subjects with juvenile pernicious anemia, we studied paraffin-embedded biopsy material from the largest reported series of childhood pernicious anemia, using a well-characterized indirect immunoperoxidase method. Preliminary studies were able to identify IF in fundic mucosal biopsy specimens that had been stored for as long as 27 yr. In a blinded evaluation, six of the nine fundic biopsy specimens from children with juvenile pernicious anemia demonstrated immunogenic IF. Twos sets of siblings were concordant for the presence or absence of intracellular IF, and six gastric biopsy specimens from patients with Imerslund's syndrome were all positive for IF. These findings indicate that juvenile pernicious anemia is a heterogeneous group of disorders whose similar clinical expression might be caused by (a) inadequate synthesis of IF, (b) a block in IF secretion, (c) the secretion of an abnormal IF that does not bind to cobalamin, or (d) the secretion of other abnormal IFs that could contain a number of other functional defects.