ICNC Abstracts, ICNC 2018

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Pulmonary functions in children with dystrophinopathy aged 5-18 years on oral steroid therapy for at least six months
Ankit Meena, Biswaroop Chakrabarty, Kana Ram Jat, S K Kabra, Sheffali Gulati

Last modified: 2018-09-09

Abstract


Introduction

Duchenne’s Muscular Dystrophy is an X linked recessive disease characterized by progressive proximal muscular weakness also affects respiratory muscle leads to restrictive pattern of lung function impairment.

Methods

During the study period we evaluated 133 diagnosed cases of DMD attending, fulfilling predefined inclusion and exclusion criteria. Pulmonary function test, 6minute walk test, functional assessment of muscular strength by Vignos and Brooke functional scale were performed on all patients.

Results

The prevalence of pulmonary dysfunction in terms percentage predicted FVC <80% is 48.12% (39.8-56.54). The prevalence of pulmonary dysfunction in terms percentage predicted FVC <80% in age group 5-7.5 years is 50% (33.63-66.37). In age group 7.5 to 10years prevalence of pulmonary dysfunction in terms of percentage predicted FVC <80% is 39.29 %( 27.58-52.37). In age group 10 -18 prevalence of pulmonary dysfunction in terms of percentage predicted FVC < 80% is 57.78%(43.3-71.03).There is significant association of mean walk duration (P=.019), walk distance (P=.004), Brooke functional score (P = 0.017), Vignos functional score (P=.001)and percentage predicted FVC.

Conclusion

There is trend that comes through in the current study that shows that impaired pulmonary function tests can be seen even early in the course of the disease and prevalence is comparable to later age groups. Longitudinal follow up study of individual patients in large numbers is essential for definite conclusion regarding progression of lung functions in dystrophinopathy.


Keywords


Duchenne muscular dystrophy, pulmonary function tests, children

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