Mahsa Rekabi, Farhad Seif, Farzad Nouri, A. Mahdaviani, Ali Akbar Velayat
{"title":"两例成功的骨髓移植改善慢性肉芽肿病患者的肺功能","authors":"Mahsa Rekabi, Farhad Seif, Farzad Nouri, A. Mahdaviani, Ali Akbar Velayat","doi":"10.18502/crcp.v7i1.9628","DOIUrl":null,"url":null,"abstract":"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.","PeriodicalId":34254,"journal":{"name":"Case Reports in Clinical Practice","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2022-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Two Successful Bone Marrow Transplantations Improved Lung Functions in Patients With Chronic Granulomatous Disease\",\"authors\":\"Mahsa Rekabi, Farhad Seif, Farzad Nouri, A. Mahdaviani, Ali Akbar Velayat\",\"doi\":\"10.18502/crcp.v7i1.9628\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"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.\",\"PeriodicalId\":34254,\"journal\":{\"name\":\"Case Reports in Clinical Practice\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-06-14\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Case Reports in Clinical Practice\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.18502/crcp.v7i1.9628\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Case Reports in Clinical Practice","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.18502/crcp.v7i1.9628","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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.