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[低丙种球蛋白血症患儿支气管肺病理及乙型肝炎的表现特征]

[Features of the manifestations of bronchopulmonary pathology and hepatitis B in children with hypogammaglobulinemia].

作者信息

Tsinzerling A V, Bogdanova A V, Zagorskaia N K, Gordova I I, Proshunina V K

出版信息

Arkh Patol. 1984;46(3):65-70.

PMID:6721706
Abstract

Two cases of hypogammaglobulinemia (Bruton's syndrome) in brothers are described. Their sisters had no this disease. Pronounced changes in the lymph nodes, spleen and thymus typical for this condition were combined in both cases with lung and kidney malformations as well as with chronic pneumonia and inanition. The both boys have viral respiratory infections that followed by marked antibody formation and clear-cut structural alterations characteristic of different stages of these diseases. Besides this the older brother was found to have chronic viral hepatitis B with an unusual giant-cell reaction.

摘要

本文描述了两例兄弟患低丙种球蛋白血症(布鲁顿氏综合征)的病例。他们的姐妹未患此病。两例患者均出现了该病典型的淋巴结、脾脏和胸腺的显著变化,同时伴有肺部和肾脏畸形以及慢性肺炎和消瘦。两个男孩都有病毒性呼吸道感染,随后出现明显的抗体形成以及这些疾病不同阶段特有的明确结构改变。此外,还发现哥哥患有慢性乙型病毒性肝炎,并伴有异常的巨细胞反应。

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