An earlier version of this research was presented in 2011: C.A. Canapari, L.B. Bliss, B.S. Tseng. Truncal fat mass correlates with decreased vital capacity in children with Duchenne muscular dystrophy. Am. J. Respir. Crit. Care Med., May 2011; 183: A1888. American Thoracic Society Conference, Denver 2011.
Truncal fat distribution correlates with decreased vital capacity in Duchenne muscular dystrophy
Article first published online: 18 MAR 2014
© 2014 Wiley Periodicals, Inc.
Volume 50, Issue 1, pages 63–70, January 2015
How to Cite
Canapari, C. A., Barrowman, N., Hoey, L., Walker, S. W., Townsend, E., Tseng, B. S. and Katz, S. L. (2015), Truncal fat distribution correlates with decreased vital capacity in Duchenne muscular dystrophy. Pediatr. Pulmonol., 50: 63–70. doi: 10.1002/ppul.23004
- Issue published online: 23 DEC 2014
- Article first published online: 18 MAR 2014
- Manuscript Accepted: 10 JAN 2014
- Manuscript Revised: 20 DEC 2013
- Manuscript Received: 17 OCT 2013
- Harvard Clinical and Translational Science Center, National Center for Research Resources. Grant Number: 1 UL1 RR025758-04
- muscular dystrophy;
- restrictive lung disease;
- body mass index
Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder associated with progressive muscle weakness and respiratory failure. Oral corticosteroids are the mainstay of treatment, but are associated with obesity with a central distribution. This study is designed to determine the relationship between body mass index, central adiposity, and lung function in subjects with DMD.
Retrospective fat distribution data was obtained in boys with DMD from studies using dual X-ray absorptiometry (DXA). Fat distribution data was reviewed at two tertiary academic institutions and compared with concurrent height, weight, body mass index (BMI), measures of lung function, and sleep study data when available. Truncal fat mass used as a measure of central adiposity.
Forty-four subjects (age 12.0 ± 3.4 years) were included. Mean BMI was 22.1 ± 5.9 kg/m2. Sixty-eight percent (30 patients) were on corticosteroid therapy. Truncal fat mass percentage was inversely correlated with forced vital capacity (% predicted FVC) (Pearson coefficient −0.37, P = 0.01). Linear regression showed that truncal fat distribution, but not total fat mass, age or corticosteroid use negatively predicted FVC (r2 = 0.24, P = 0.048). BMI had a positive effect (P = 0.04). However, fat distribution did not predict the rate of change of lung function in a smaller sample. Fat distribution, BMI, or age did not predict measures of sleep disordered breathing.
Truncal fat distribution is a significant predictor of lower vital capacity in boys with DMD, whereas BMI has a positive effect. Pediatr Pulmonol. 2015; 50:63–70. © 2014 Wiley Periodicals, Inc.