To describe the pattern of lung function abnormality and to investigate the changes in spirometry before and after autologus hematopoietic stem cell transplantation in one duchenne muscular dystrophy (DMD) patient.
Lung function was measured by maximum expiratory flow-volume loops and whole body plethysmography in one 14-yr old DMD patient before and three months after stem cell transplantation.
Lung function test was characterized by restrictive pattern manifested by lung volume reduction and increased FEV1/FVC due to muscular weakness. Before stem cell transplantation, the FVC, FEV1 and MVV were 1.4L, 1.4L and 59.5L respectively. 3 months after stem cell transplantation, the patient’s FVC, FEV1 and MVV were significantly increased to 2.12L, 2.12L and 118.0L respectively.
Although genetically modified myoblast transplantation remains controversial for DMD, a significant change in spirometry was found in this DMD patient after stem cell transplantation.
Spirometric measurement provides a simple and useful means of assessing disease progression in DMD patients and should be considered when stem cell transplantation is planning.Furthermore, since DMD characterized by gradually developing muscular weakness, pulmonary physical rehabilitation should focus on the training of respiratory accessory muscles.
Yubiao Guo, None.