两例成功的骨髓移植改善慢性肉芽肿病患者的肺功能

Mahsa Rekabi, Farhad Seif, Farzad Nouri, A. Mahdaviani, Ali Akbar Velayat
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引用次数: 0

摘要

慢性肉芽肿病(CGD)是一种罕见的遗传性原发性免疫缺陷疾病,吞噬细胞呼吸爆发活动缺陷,导致复发性化脓性感染。在本研究中,我们描述了两例接受骨髓移植(BMT)的CGD患者。由于骨髓移植(BMT)是该疾病的最终治疗方法,我们评估了骨髓移植前后的肺功能。在这两名患者中,BMT都来自他们的兄弟姐妹。病例1患者肺功能(PFT) BMT前为:FEV1: 34, FVC: 40, FEV1 / FVC: 72%, BMT后为:FEV1: 66, FVC: 40 × 49, FEV1 / FVC: 64%。病例2患者BMT前PFT为:FEV1: 22, FVC: 36, FEV1/FVC: 41%, BMT后PFT为:FEV1: 47, FVC: 33, FEV1/FVC: 43%。BMT显著改善了他们的肺部问题和临床前(PFT)。此外,经BMT治疗后,两例患者耐受性良好的临床体征及感染率、住院次数均有所下降。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Two Successful Bone Marrow Transplantations Improved Lung Functions in Patients With Chronic Granulomatous Disease
Chronic Granulomatous Disease (CGD) is a rare inherited primary immune deficiency disorder with defective respiratory burst activity in phagocytes, resulting in recurrent pyogenic infections. In this study, we described two CGD patients who had done bone marrow transplantation (BMT). As Bone marrow transplantation (BMT) is the definitive treatment of the disease, we evaluated the function of their lungs before and after BMT. In both patients, the BMT was from their siblings. In case 1, the patient’s pulmonary function (PFT) before BMT was: FEV1: 34, FVC: 40, FEV1 / FVC: 72%, and after BMT was: FEV1: 66, FVC: 40 by 49, FEV1 / FVC: 64%. In case 2, the patient’s PFT before BMT was: FEV1: 22, FVC: 36, FEV1 / FVC: 41%, and after BMT was: FEV1: 47, FVC: 33, FEV1/FVC: 43%. BMT significantly improved their Pulmonary Problems and Preclinical (PFT). In addition, after BMT, both patients’ well-tolerated clinical signs and the infection rate, and the number of hospitalizations in both patients decreased.
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