INHERITED DEFECT OF NEUTROPHIL MOBILITY IN SHWACHMAN SYNDROME
INHERITED DEFECT OF NEUTROPHIL MOBILITY IN SHWACHMAN SYNDROME
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DOI：
10.1016/s0022-3476(79)80578-8
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发表时间：
1979-01-01
影响因子：
5.1
通讯作者：
SOOTHILL, JF
中科院分区：
文献类型：
--
作者：
AGGETT, PJ;HARRIES, JT;SOOTHILL, JF
对14例Shwachman综合征患者的免疫功能进行了评估。9名患者为血小板减少症，4名患者IG[免疫球蛋白]A或IgM水平低。神经元的流动性显着缺陷的患者组作为一个整体（在12个低于正常下限），并在他们的父母。未发现其他一致的免疫异常。中性粒细胞移动性缺陷是Shwachman综合征的一个特征，这可能导致这些患者容易频繁感染。该缺陷似乎是一种原发性遗传缺陷，遗传为常染色体隐性遗传特征，与假定的Shwachman综合征遗传一致。
Selected immunologic functions were assessed in 14 patients with the Shwachman syndrome. Nine patients were neutropenic and 4 had low levels of Ig[immunoglobulin]A or IgM. Neutrophil mobility was significantly defective in the group of patients as a whole (in 12 it was below the lower limit of normal) and in their parents. No other consistent abnormality in immunity was found. The defective neutrophil mobility is a feature of Shwachman syndrome which may contribute to the vulnerability of these patients to frequent infections. The defect appears to be a primary genetic one, inherited as an autosomal recessive characteristic consistent with the assumed inheritance of Shwachman syndrome.