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   two successful bone marrow transplantations improved lung functions in patients with chronic granulomatous disease  
   
نویسنده rekabi mahsa ,seif farhad ,nouri farzad ,mahdaviani alireza ,velayati ali akbar
منبع case reports in clinical practice - 2022 - دوره : 7 - شماره : 1 - صفحه:9 -12
چکیده    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.
کلیدواژه bone marrow transplantation ,chronic granulomatous disease ,lung function ,patient’s pulmonary function
آدرس national research institute of tuberculosis and lung, pediatric respiratory disease research center, iran, academic center for education, culture and research, department of immunology and allergy, iran. iran university of medical sciences, neuroscience research center, iran, national research institute of tuberculosis and lung, pediatric respiratory disease research center, iran, national research institute of tuberculosis and lung, pediatric respiratory disease research center, iran, national research institute of tuberculosis and lung, pediatric respiratory disease research center, iran
پست الکترونیکی velayati@theaasm.org
 
     
   
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