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

机译:B细胞缺陷的原发性免疫缺陷:菲律宾国家大专院区见到的6例患者

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摘要

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.
机译:初级免疫缺陷障碍虽然罕见,但患有患病患者及其家庭的生活质量构成了重大负担。这些疾病中最常见的是由B细胞缺陷引起的。从1996年至2018年,共有6名患者在菲律宾的全国第三级患者中被视为诊断。这些患者因各种感染而被录取,随后被诊断为具有B细胞缺陷。 6名患者中有四项遗传研究证实诊断X链状的阿霉素血症血症。一名患者在10岁时败血症败血症,而其余的是在菲律宾综合医院进行后续静脉内免疫球蛋白输注。

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