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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
09:18

Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy

Published on: January 12, 2019

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Does Body Mass Index Predict Premature Cardiomyopathy Onset for Duchenne Muscular Dystrophy?

Meghann McKane1, Jonathan H Soslow1, Meng Xu2

  • 11 Thomas P. Graham Division of Pediatric Cardiology Monroe Carell Jr Children's Hospital at Vanderbilt University, Nashville, TN, USA.

Journal of Child Neurology
|January 14, 2017
PubMed

Insights

Body mass index Z scores were not associated with the onset of cardiomyopathy in Duchenne muscular dystrophy. Corticosteroid use, not loss of mobility, correlated with earlier cardiomyopathy onset in these patients.

Area of Science:

  • Cardiology
  • Neurology
  • Genetics

Background:

  • Duchenne muscular dystrophy (DMD) is a progressive genetic disorder.
  • Cardiomyopathy is a common and serious complication of DMD, affecting nearly half of patients.
  • Understanding factors influencing cardiomyopathy onset is crucial for patient management.

Purpose of the Study:

  • To investigate the association between body mass index (BMI) Z scores and the age of cardiomyopathy onset in individuals with Duchenne muscular dystrophy.
  • To explore other potential clinical correlates of cardiomyopathy onset in DMD.

Main Methods:

  • Retrospective analysis of patient data, including BMI Z scores and age at cardiomyopathy diagnosis.
  • Cardiomyopathy defined by left ventricular ejection fraction (<55%) or fractional shortening (<28%).
  • Statistical modeling adjusted for relevant covariates.

Main Results:

  • No significant association was found between BMI Z score and the age of cardiomyopathy onset (HR 0.79, P = .17).
  • Duration of corticosteroid treatment was significantly associated with earlier cardiomyopathy onset (P = .01).
  • Loss of ambulatory ability was not significantly associated with cardiomyopathy onset (P = .47).

Conclusions:

  • Body mass index does not appear to be a predictor of cardiomyopathy onset in Duchenne muscular dystrophy.
  • Corticosteroid therapy is a significant factor influencing the timing of cardiomyopathy development in DMD patients.
  • Further research into therapeutic interventions and their impact on cardiac outcomes in DMD is warranted.

Keywords:
Duchenne muscular dystrophybody mass indexcardiomyopathy

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