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[婴儿期暂时性低丙种球蛋白血症]

[Transient hypogammaglobulinemia of infancy].

作者信息

Bellutti Enders Felicitas, Conti Francesca, Candotti Fabio, Angelini Federica

机构信息

Service de pédiatrie, Département Femme - Mère - Enfant, CHUV, 1011 Lausanne.

Service d'immunologie et allergologie, Département de médecine, CHUV, 1011 Lausanne.

出版信息

Rev Med Suisse. 2017 Apr 5;13(557):739-742.

Abstract

Transient hypogammaglobulinemia of infancy is characterized as a reduction of one or more classes of immunoglobulins with a response to vaccines and normal subpopulations of lymphocytes B presenting in the first years of life. The diagnosis is made a posteriori, once the levels of immunoglobulins are normalized, in general between 2 and 4 years of age. Clinical presentation varies : the child may be either asymptomatic or present with recurrent infections, atopy and / or auto-immunity. There are no clinical or immunological features that distinguish this condition from a common variable immunodeficiency (CVID). Because of the risk of severe infections, it is necessary a follow up by a paediatric immunologist. Depending on the presentation and evolution, a prophylaxis with antibiotics or a substitution with immunoglobulins might be indicated.

摘要

婴儿期短暂性低丙种球蛋白血症的特征是在生命的最初几年中,一种或多种免疫球蛋白类别减少,对疫苗有反应,且存在正常的B淋巴细胞亚群。一旦免疫球蛋白水平恢复正常,通常在2至4岁之间,即可做出回顾性诊断。临床表现各不相同:儿童可能无症状,也可能出现反复感染、特应性和/或自身免疫性疾病。没有临床或免疫学特征能够将这种疾病与常见变异型免疫缺陷(CVID)区分开来。由于存在严重感染的风险,因此需要儿科免疫学家进行随访。根据临床表现和病情发展,可能需要使用抗生素进行预防或用免疫球蛋白进行替代治疗。

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