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[青少年和成人特发性免疫球蛋白缺乏症。随访研究]

[Idiopathic immunoglobulin deficiency in juveniles and adults. Catamnestic studies].

作者信息

Grob P W, Pfister E W, Grob P J

出版信息

Schweiz Med Wochenschr. 1980 Apr 19;110(16):596-604.

PMID:7394475
Abstract

In 1974 and 1975 7000 patients' sera were tested for levels of IgG, IgA and IgM. In 330 patients at least one of the three Ig classes was low. In most instances secondary immunodeficiency was present in association with myeloma, etc. However, 81 patients above 8 years of age fulfilled the criteria of idiopathic late onset Ig deficiency. In 44 of these patients clinical follow-up and repeated measurements of Ig levels were possible 1--8 years after the initial diagnosis. Selective IgA deficiency was present initially (15 patients) most frequently and persisted most often (14 patients). 4 patients had initially low IgG and 6 patients low IgM, findings which were only rarely confirmed later on. In 19 patients 2 or 3 Ig classes were initially low, with persistence of Ig deficiency in 12 individuals. In no instance had clinical symptoms appeared in the first two years of life. The following diseases were documented in the 44 patients studied (28 individuals with persistent and 16 with transitory Ig deficiency): recurrent infections (16 patients), atopic disease (8 times), rheumatoid arthritis (6 times), epilepsy (4 times), SLE (3 times) and enteropathies (twice). Seven patients also had a malignancy, 4 diabetes, and 2 hyperthyroidism.

摘要

1974年和1975年,对7000名患者的血清进行了IgG、IgA和IgM水平检测。330名患者中至少有一类Ig水平较低。多数情况下,继发性免疫缺陷与骨髓瘤等相关。然而,81名8岁以上患者符合特发性迟发性Ig缺乏的标准。其中44名患者在初次诊断后1至8年可进行临床随访并重复检测Ig水平。最初选择性IgA缺乏最为常见(15例患者),且持续存在的情况也最多(14例患者)。4例患者最初IgG水平低,6例患者最初IgM水平低,这些结果后来很少得到证实。19例患者最初2类或3类Ig水平低,其中12例患者持续存在Ig缺乏。在生命的头两年均未出现临床症状。在研究的44例患者(28例持续性Ig缺乏和16例暂时性Ig缺乏)中记录到以下疾病:反复感染(16例患者)、特应性疾病(8例)、类风湿关节炎(6例)、癫痫(4例)、系统性红斑狼疮(3例)和肠病(2例)。7例患者还患有恶性肿瘤,4例患有糖尿病,2例患有甲状腺功能亢进症。

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