Ann Rehabil Med.  2016 Oct;40(5):851-861. 10.5535/arm.2016.40.5.851.

Analysis of Pulmonary Function Test in Korean Patients With Duchenne Muscular Dystrophy: Comparison of Foreign and Korean Reference Data

Affiliations
  • 1Department of Rehabilitation Medicine, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea.
  • 2Department of Rehabilitation Medicine, Pusan National University Hospital, Pusan National University School of Medicine, Busan, Korea. yi0314@gmail.com
  • 3Biomedical Research Institute, Pusan National University Hospital, Busan, Korea.

Abstract


OBJECTIVE
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.
METHODS
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.
RESULTS
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 cmHâ‚‚O, 45.79±17.46 cmHâ‚‚O, 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.
CONCLUSION
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.

Keyword

Duchenne muscular dystrophy; Respiratory function test; Reference values

MeSH Terms

Adolescent
Child
Cough
Forced Expiratory Volume
Humans
Muscular Dystrophy, Duchenne*
Reference Values
Respiratory Function Tests*
Respiratory Therapy
Vital Capacity

Figure

  • Fig. 1 Bland-Altman plot showing individual differences between the two tests. The solid line indicates the mean difference between paired measurements and the dotted lines indicate the 95% limits of agreement. It shows that 95% of the differences were within two standard deviations from the mean, pointing to the fact that both tests are very similar in terms of FVC (A) and FEV1 (B) values. FVC, forced vital capacity; FEV1, forced expiratory volume in 1 second.

  • Fig. 2 Comparison of FVC and FEV1% of the Korean reference data and the overseas reference data (A). On comparison of two inland and overseas data, FVC% of the Korean children and adolescent reference data was higher than that of the overseas data and the differences were statistically significant. FEV1% of the overseas data showed a slightly higher value than that of the inland data, but the difference was not statistically significant (*p<0.05, **p<0.001). Comparison of MIP% and MEP% of the Korean reference data and the overseas reference data (B). On comparison of the inland and overseas prediction equation data, MIP% and MEP% of the Korean children and adolescent reference data were significantly higher than those of the three overseas prediction equation data (*p<0.05, **p<0.001).

  • Fig. 3 Comparison of MIP (A) and MEP (B) values predicted by the reference data proposed in our study and three previous studies according to age. We found a decrease rate of 8.76% per year when MIP expressed as % of this study reference data, which is higher than that in the previous study data, but the decrease rate of MIP expressed as % of the Tomalak and Arnall prediction equation data was similar to that in a previous study. MEP% of the present study reference data did not show a significant decrease in the age range from 8 to 16 years, whereas MEP% of the Wilson, Tomalak and Arnall prediction equation data showed a significant correlation with age.

  • Fig. 4 Comparison of FVC expressed as % of the overseas reference data and Korean reference data according to age. FVC% of the Korean reference data tended to be higher than that of the overseas reference data in the age group of less than 13.7 years, whereas it was opposite in the age group of more than 13.7 years. FVC, forced vital capacity.


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