Rothenberg M E, White F V, Chilmonczyk B, Chatila T
Division of Immunology, Harvard Medical School, Children's Hospital, Boston, MA, 02115, USA.
Immunodeficiency. 1995;5(3):171-8.
Multiple intestinal atresias (MIA) is a severe form of intestinal atresias throughout the gastrointestinal tract. In two reports, MIA have been associated with severe immunodeficiency. We report a newborn girl who had profound humoral and B cell immunodeficiency and impaired T cell function. The patient had agammaglobulinemia and decreased blood lymphocytes, with virtually no B cells in the blood or in intestinal lymph nodes. T cells were reduced in number and weakly proliferated to mitogens. These data suggest that a syndrome involving the development of MIA is associated with various forms of severe immunodeficiency, and therefore newborns with MIA should be examined for immunodeficiency.
多发性肠闭锁（MIA）是一种累及整个胃肠道的严重肠闭锁形式。在两份报告中，MIA与严重免疫缺陷有关。我们报告了一名患有严重体液和B细胞免疫缺陷以及T细胞功能受损的新生女婴。该患者存在无丙种球蛋白血症且血液淋巴细胞减少，血液和肠淋巴结中几乎没有B细胞。T细胞数量减少且对有丝分裂原的增殖反应较弱。这些数据表明，一种涉及MIA发生的综合征与各种形式的严重免疫缺陷有关，因此应对患有MIA的新生儿进行免疫缺陷检查。
