Kim Eun Young, Lee Jang Woo, Suh Mi Ri, Choi Won Ah, Kang Seong Woong, Oh Hyeon Jun
Department of Rehabilitation Medicine, Gangnam Severance Hospital, Seoul, Korea.
Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine, Seoul, Korea.
Ann Rehabil Med. 2017 Apr;41(2):306-312. doi: 10.5535/arm.2017.41.2.306. Epub 2017 Apr 27.
To investigate the relationship between serum creatine kinase (CK) level and pulmonary function in Duchenne muscular dystrophy (DMD).
A total of 202 patients with DMD admitted to the Department of Rehabilitation Medicine, Gangnam Severance Hospital were enrolled from January 1, 1999 to March 31, 2015. Seventeen patients were excluded. Data collected from the 185 patients included age, height, weight, body mass index, pulmonary function tests including forced vital capacity (FVC), peak cough flow, maximal expiratory pressure (MEP), and maximal inspiratory pressure (MIP), and laboratory measurements (serum level of CK, CK-MB, troponin-T, and B-type natriuretic peptide). FVC, MEP, and MIP were expressed as percentages of predicted normal values.
Serum CK activities were elevated above normal levels, even in the oldest DMD group. Serum CK level was strongly correlated with pulmonary functions of sitting FVC (p<0.001), supine FVC (p<0.001), MIP (p=0.004), and MEP (p<0.001).
Serum CK level is a reliable screening test even in patients with advanced DMD, and is a strong predictor of pulmonary functions.
探讨杜氏肌营养不良症(DMD)患者血清肌酸激酶(CK)水平与肺功能之间的关系。
选取1999年1月1日至2015年3月31日期间入住江南severance医院康复医学科的202例DMD患者。排除17例患者。收集的185例患者的数据包括年龄、身高、体重、体重指数、肺功能测试,包括用力肺活量(FVC)、峰值咳嗽流速、最大呼气压力(MEP)和最大吸气压力(MIP),以及实验室测量指标(血清CK、CK-MB、肌钙蛋白T和B型利钠肽水平)。FVC、MEP和MIP以预测正常值的百分比表示。
即使在年龄最大的DMD组中,血清CK活性也高于正常水平。血清CK水平与坐位FVC(p<0.001)、仰卧位FVC(p<0.001)、MIP(p=0.004)和MEP(p<0.001)的肺功能密切相关。
即使在晚期DMD患者中,血清CK水平也是一项可靠的筛查指标,并且是肺功能的有力预测指标。