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儿童常见可变型低丙种球蛋白血症。30例患者的临床和免疫学观察

Common variable hypogammaglobulinemia in children. Clinical and immunologic observations in 30 patients.

作者信息

Hausser C, Virelizier J L, Buriot D, Griscelli C

出版信息

Am J Dis Child. 1983 Sep;137(9):833-7. doi: 10.1001/archpedi.1983.02140350011004.

Abstract

We made clinical and immunologic observations of 30 children with common variable hypogammaglobulinemia. The mean age at diagnosis was 10.5 years, five years after clinical onset. Diagnosis was initially made based on a history of recurrent otobronchopulmonary infections, diarrhea, or both. The most common complications included short stature, bronchiectasis, and malabsorption, often associated with giardiasis or sprue. Nine patients had associated autoimmune diseases (eg, atrophic gastritis, arthritis, and hemolytic anemia). Three patients died, one of chronic respiratory insufficiency, one of chronic persisting hepatitis, and one of osteogenic sarcoma. Humoral and cellular immune functions of all patients were examined.

摘要

我们对30例普通变异型低丙种球蛋白血症患儿进行了临床和免疫学观察。诊断时的平均年龄为10.5岁,临床发病后5年。最初根据反复发生的耳支气管肺部感染、腹泻或两者兼有的病史进行诊断。最常见的并发症包括身材矮小、支气管扩张和吸收不良,常与贾第虫病或口炎性腹泻有关。9例患者伴有自身免疫性疾病(如萎缩性胃炎、关节炎和溶血性贫血)。3例患者死亡,1例死于慢性呼吸功能不全,1例死于慢性持续性肝炎,1例死于骨肉瘤。对所有患者的体液和细胞免疫功能进行了检查。

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