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B细胞缺陷导致的原发性免疫缺陷:菲律宾一家国立三级医院诊治的6例病例系列

Primary immunodeficiency from B-cell defect: a case series of 6 patients seen in a national tertiary hospital in the Philippines.

作者信息

Tan-Lim Carol Stephanie C, Castor Mary Anne R

机构信息

Department of Pediatrics, Section of Allergy and Immunology, Philippine General Hospital, Manila, the Philippines.

出版信息

Asia Pac Allergy. 2021 Jan 26;11(1):e7. doi: 10.5415/apallergy.2021.11.e7. eCollection 2021 Jan.

Abstract

Primary immunodeficiency disorders, although rare, pose a significant burden in the quality of life of afflicted patients and their families. The most common of these disorders are caused by B-cell defects. A total of 6 patients were seen and diagnosed in a national tertiary hospital in the Philippines from 1996 to 2018. These patients were admitted due to various infections, and were subsequently diagnosed to have B-cell defects. Four out of the 6 patients have genetic studies confirming the diagnosis of X-linked agammaglobulinemia. One patient succumbed to sepsis at 10 years of age, while the rest are on follow-up at the Philippine General Hospital for intravenous immunoglobulin infusion.

摘要

原发性免疫缺陷疾病虽然罕见,但给患病患者及其家庭的生活质量带来了沉重负担。这些疾病中最常见的是由B细胞缺陷引起的。1996年至2018年期间,菲律宾一家国立三级医院共接诊并诊断了6例患者。这些患者因各种感染入院,随后被诊断为B细胞缺陷。6例患者中有4例经基因研究确诊为X连锁无丙种球蛋白血症。1例患者在10岁时死于败血症,其余患者在菲律宾总医院接受随访,进行静脉注射免疫球蛋白治疗。

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本文引用的文献

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X-Linked Agammaglobulinaemia: Outcomes in the modern era.X连锁无丙种球蛋白血症:现代的治疗结果
Clin Immunol. 2017 Oct;183:54-62. doi: 10.1016/j.clim.2017.07.008. Epub 2017 Jul 17.
5
Practice parameter for the diagnosis and management of primary immunodeficiency.原发性免疫缺陷诊断与管理的实践参数
Ann Allergy Asthma Immunol. 2005 May;94(5 Suppl 1):S1-63. doi: 10.1016/s1081-1206(10)61142-8.
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Primary immunodeficiency diseases in Norway.挪威的原发性免疫缺陷病
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