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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence.
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Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence.

机译：55年后，Good's综合征仍然是一个谜：对科学证据的系统回顾。

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摘要

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.
机译：良好综合征（GS）是胸腺瘤和免疫缺陷的罕见关联，最早在50多年前就被描述出来。但是，这种综合征仍然是临床医生的一个谜。我们系统地回顾了152例Good综合征患者的所有临床，实验室和免疫学发现。该综合征在世界范围内分布，大约一半的病例（47％）已在欧洲描述。在42％的患者中，胸腺瘤的诊断先于低球蛋白血症，感染或腹泻的诊断，而在38％的患者中，彼此之间几乎同时在两个月内做出诊断。我们发现患有这种综合征的患者死亡率很高（44.5％）。敏锐的临床敏锐度和对这种综合征的临床和免疫学特征的认识的增强，可能会增强对该综合征的早期识别并预防死亡率。需要进一步研究以阐明该临床实体。

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