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婴儿期暂时性低丙种球蛋白血症:40例新病例的临床和免疫学特征

Transient hypogammaglobulinemia of infancy: clinical and immunologic features of 40 new cases.

作者信息

Kiliç S S, Tezcan I, Sanal O, Metin A, Ersoy F

机构信息

Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

出版信息

Pediatr Int. 2000 Dec;42(6):647-50. doi: 10.1046/j.1442-200x.2000.01301.x.

DOI:10.1046/j.1442-200x.2000.01301.x
PMID:11192522
Abstract

BACKGROUND

Transient hypogammaglobulinemia of infancy (THI) results from a delay in the maturation of immunoglobulin production.

METHODS

The clinical and immunologic data of 40 patients with THI are presented. Clinically, the majority of patients presented with recurrent respiratory infections and otitis media, bronchitis and/or bronchial asthma and recurrent gastroenteritis.

RESULTS

Ten of 40 children had isolated low IgG; isolated low IgA and isolated low IgM were detected in one and three patients, respectively. At initial evaluation, levels of all three immunoglobluins were low in nine patients. Ten patients had diminished IgG and IgA levels, six had diminished IgA and IgM levels and one had low IgG and IgM levels. Two patients were given intravenous immunoglobulin replacement therapy for 1 year. None of the patients has experienced serious infections during their follow-up period.

CONCLUSIONS

Prospective evaluation of patients (age range 5-60 months) revealed that immunoglobulin levels in 33 patients recovered before 36 months of age. Seven patients still had low immunoglobluin levels at 40-57 months of age. Three of these patients had low levels of both IgG and IgA, while two patients had low IgM levels and a further two patients had low IgA levels.

摘要

背景

婴儿期暂时性低丙种球蛋白血症(THI)是由免疫球蛋白产生成熟延迟所致。

方法

介绍了40例THI患者的临床和免疫学数据。临床上,大多数患者表现为反复呼吸道感染、中耳炎、支气管炎和/或支气管哮喘以及反复胃肠炎。

结果

40名儿童中有10名仅有低IgG;分别在1名和3名患者中检测到仅有低IgA和仅有低IgM。在初始评估时,9名患者的所有三种免疫球蛋白水平均较低。10名患者的IgG和IgA水平降低,6名患者的IgA和IgM水平降低,1名患者的IgG和IgM水平较低。2名患者接受了1年的静脉注射免疫球蛋白替代治疗。所有患者在随访期间均未发生严重感染。

结论

对患者(年龄范围5 - 60个月)的前瞻性评估显示,33名患者的免疫球蛋白水平在36个月龄前恢复。7名患者在40 - 57个月龄时免疫球蛋白水平仍较低。其中3名患者的IgG和IgA水平均较低,2名患者的IgM水平较低,另外2名患者的IgA水平较低。

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