The association between dietary factors and body weight and composition in boys with Duchenne muscular dystrophy

Abstract

Background: Duchenne muscular dystrophy (DMD) is a X-linked neuromuscular disorder. Boys with DMD have high rates of obesity, although little is known about dietary factors that may contribute to weight gain in this population. The present study aimed to explore the relationship between dietary factors, body mass index (BMI) z-score, body composition and motor function and to describe dietary intake in boys with DMD. Methods: A cross-sectional analysis of 3-day food diaries from ambulant and steroid treated boys with DMD aged 5-13 years was conducted. Correlation analysis explored the relationship between dietary factors, BMI z-score, fat mass percentage (FM%) and lean mass percentage (LM%). Results: The median age was 8.5 years (interquartile range [IQR] = 7.2-10.5 years). Median energy kg -1 day -1 in those within a healthy weight range (n = 11) was 316 kJ kg -1 day -1 (IQR = 276-355 kJ kg -1 day -1 ) and greater than estimated requirements and, for those above a healthy weight (n = 26), energy intake was 185 kJ kg -1 day -1 (IQR = 143-214 kJ kg -1 day -1 ) and lower than estimated requirements. Energy kg -1 day -1 was negatively associated with BMI z-score (r = −0. 650) and FM% (r = −0.817) but positively associated with LM% (r = 0.805; all analyses p = <0.01). Younger age was associated (r = −0.609 p = <0.01) with a higher energy kg -1 day -1 . For all participants, vegetable, grains, meat/alternatives and dairy intakes were sub-optimal. Conclusions: Younger boys with DMD within a healthy weight range are overconsuming energy dense nutrient poor foods. A focus on improving diet quality during early childhood may prove to be a useful strategy for reducing excess weight gain and supporting healthier eating habits in this vulnerable clinical population.

Fracture risk and impact in boys with Duchenne muscular dystrophy: A retrospective cohort study

“…The importance of preventable interventions on weight management such as dietary optimization should not be underestimated. Previous studies have observed mismatches in caloric intake being excessive in pre‐school and school‐age groups 14,23 . Greater emphasis on weight‐related strategies and regular nutrition counseling early in the disease course should be at the forefront of bone health in boys with DMD 6,23 .…”

“…21 Mechanisms of how higher BMI can correlate with fracture rates, particularly lower limb fractures may include inappropriate adaptation of the axial skeleton with body size and impaired balance. 11,23 Unique to dystrophin deficiency, boys with DMD also exhibit progressive replacement of lean muscle mass with intramuscular adipose tissue; both reducing energy expenditure, increasing body fat mass, and impacting the structural integrity of the axial skeleton.…”

“…Previous studies have observed mismatches in caloric intake being excessive in pre-school and school-age groups. 14,23 Greater emphasis on weight-related strategies and regular nutrition counseling early in the disease course should be at the forefront of bone health in boys with DMD. 6,23 Further studies investigating the association of obesity on lower-limb fracture risk are needed, with emphasis on adjusting for the effects of steroid use (dosage, regimen, etc.).…”

“…14,23 Greater emphasis on weight-related strategies and regular nutrition counseling early in the disease course should be at the forefront of bone health in boys with DMD. 6,23 Further studies investigating the association of obesity on lower-limb fracture risk are needed, with emphasis on adjusting for the effects of steroid use (dosage, regimen, etc.). Furthermore, interventions to optimize caloric intake in boys with DMD would be beneficial.…”

Exploring caregivers' attitudes and beliefs about nutrition and weight management for young people with Duchenne muscular dystrophy

“…13,14 In two prior investigations of dietary intake of individuals with DMD, higher total energy intakes compared to estimated requirements and displacement of core food groups with discretionary (non-core) foods were observed. 15,16 Qualitative research has identified challenges associated with weight management such as competing health care priorities, the complexities of the high level of care needs that comes with DMD, and balancing dietary restriction and the enjoyment associated with food. 17,18 This study aimed to extend existing knowledge by exploring specific barriers and enablers that influence nutritionand weight-related behaviors as well as attitudes and beliefs about nutrition and weight management in the home environment.…”