Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence.
Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence.
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DOI：
10.1016/j.clim.2010.01.006
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发表时间：
2010-06
期刊：
影响因子：
--
通讯作者：
Yang O
中科院分区：
文献类型：
--
作者：
Kelesidis T;Yang O
Good综合征（GS）是一种罕见的胸腺瘤和免疫缺陷的关联，50多年前首次被描述。然而，这种综合征对临床医生来说仍然是个谜。我们系统地回顾了152例Good综合征患者的所有临床、实验室和免疫学结果。该综合征在世界范围内分布，大约一半的病例（47%）发生在欧洲。在42%的患者中，胸腺瘤的诊断先于低γ球蛋白血症、感染或腹泻的诊断，而在38%的患者中，这些诊断几乎是在两个月内同时被诊断出来的。我们发现该综合征患者死亡率显著（44.5%）。敏锐的临床敏锐度和提高对该综合征的临床和免疫学概况的认识可能会增加对该综合征的早期认识并预防死亡。需要进一步的研究来阐明这一临床实体。
Good syndrome (GS) is a rare association of thymoma and immunodeficiency first described more than 50 years ago. However, this syndrome still remains a mystery to clinicians. We systematically reviewed all the clinical, laboratory and immunologic findings from 152 patients with Good syndrome. The syndrome has a worldwide distribution and approximately half of the cases (47%) have been described in Europe. The diagnosis of thymoma preceded the diagnosis of hypogammaglobulinemia, infection, or diarrhea in 42% of patients whereas in 38% of patients the diagnoses were made almost simultaneously within 2 months of each other. We found significant mortality in patients with this syndrome (44.5%). Astute clinical acumen and increased awareness about the clinical and immunological profile of this syndrome may increase early recognition of this syndrome and prevent mortality. Further studies are needed to elucidate this clinical entity.
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