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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.
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To determine the abnormal pulmonary function value in Korean Duchenne muscular dystrophy (DMD) patients, we performed a comparative analysis of the patients' pulmonary function value expressed as % of the overseas reference data and Korean healthy children and adolescent reference data.
We performed pulmonary function test (PFT) in a total of 27 DMD patients. We compared the patients' FVC% and FEV1% of the overseas reference data with those of the Korean children and adolescent reference data. Also, we compared the patients' MIP% and MEP% of the prediction equation data with those of the Korean children and adolescent reference data.
Age of the subjects ranged from 8 to 16 years (12.03±2.27 years). The mean maximal expiratory pressure (MEP), maximal inspiratory pressure (MIP), vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), and peak cough flow (PCF) were 36.93±9.5 cmH2O, 45.79±17.46 cmH2O, 1.4±0.43 L, 1.45±0.45 L, 1.40±0.41 L, and 206.25±61.21 L/min, respectively. The MIP%, MEP%, and FVC% of the Korean children and adolescent reference data showed statistically significant higher values than those of the prediction equation data.
We observed a clear numeric difference between Korean DMD patients' pulmonary function value expressed as % of the overseas data and inland data. To perform a precise assessment of respiratory function and to determine appropriate respiratory therapy, pulmonary function values of Korean DMD patients should be interpreted taking into account the inland normal pulmonary function test data.
To investigate the effectiveness of incentive spirometry on respiratory motion in healthy subjects using cine breathing magnetic resonance imaging (MRI).
Ten non-smoking healthy subjects without any history of respiratory disease were studied. Subjects were asked to perform pulmonary training using incentive spirometry every day for two weeks. To assess the effectiveness of this training, pulmonary function tests and cine breathing MRI were performed before starting pulmonary training and two weeks after its completion.
After training, there were significant improvements in vital capacity (VC) from 3.58±0.8 L to 3.74±0.8 L and in %VC from 107.4±10.8 to 112.1±8.2. Significant changes were observed in the right diaphragm motion, right chest wall motion, and left chest wall motion, which were increased from 55.7±9.6 mm to 63.4±10.2 mm, from 15.6±6.1 mm to 23.4±10.4 mm, and from 16.3±7.6 mm to 22.0±9.8 mm, respectively.
Two weeks of training using incentive spirometry provided improvements in pulmonary function and respiratory motion, which suggested that incentive spirometry may be a useful preoperative modality for improving pulmonary function during the perioperative period.
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