BACKGROUND/OBJECTIVES: Nutritional issues that are associated with Duchenne muscular dystrophy (DMD) remain poorly understood. The aim of this analysis was to describe and explore longitudinal observations of body mass index (BMI) in a cohort of children with DMD. SUBJECTS/METHODS: Anthropometric and clinical characteristic's were collected retrospectively and longitudinally. for boys with DMD seen in two large neuromuscular clinics. BMI Z-scores were determined using the Centers for Disease Control and Prevention reference values for children (2000). RESULTS: Medical records (n =193) were examined from which 75% were included for analysis. The mean age of the cohort at the time of data collection was 11.9 years, with 72% of patients currently or previously using steroids. The highest prevalence of obesity based on the BMI Z-score was 50% at the age of 10 years. Longitudinally, BMI Z-scores from the age of 2 to 12 years plot approximately one s.d. above the mean, after which there is a marked and progressive decline. BMI gainers were identified for whom BMI Z-score increased by 1.65 units compared with the 0.09 units in non-gainers. BMI gainers were younger when they had their first BMI measurement (5.9 vs 7.2 years), and this measure was significantly lower compared with the non-gainers (BMI Z-score: 0.04 vs 1.17). In this cohort, BMI was associated with age, ambulatory status and lung function. CONCLUSIONS: This study demonstrates that boys with DMD using steroid therapy experience shifts in BMI. A declining BMI appears to be associated with increasing age. Interpretation of growth patterns is limited here by a lack of normative growth references in DMD.
机构:
Royal Hosp Sick Children, Bone & Endocrine Res Grp, Glasgow, Lanark, ScotlandRoyal Hosp Sick Children, Bone & Endocrine Res Grp, Glasgow, Lanark, Scotland
McDevitt, H.
McWilliam, R.
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Royal Hosp Sick Children, Bone & Endocrine Res Grp, Glasgow, Lanark, ScotlandRoyal Hosp Sick Children, Bone & Endocrine Res Grp, Glasgow, Lanark, Scotland
McWilliam, R.
Quayum, N.
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Royal Hosp Sick Children, Bone & Endocrine Res Grp, Glasgow, Lanark, ScotlandRoyal Hosp Sick Children, Bone & Endocrine Res Grp, Glasgow, Lanark, Scotland
Quayum, N.
Ahmed, S.
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Royal Hosp Sick Children, Bone & Endocrine Res Grp, Glasgow, Lanark, ScotlandRoyal Hosp Sick Children, Bone & Endocrine Res Grp, Glasgow, Lanark, Scotland
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Univ Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USAUniv Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USA
Fowler, Eileen G.
Staudt, Loretta A.
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Univ Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USAUniv Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USA
Staudt, Loretta A.
Heberer, Kent R.
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Univ Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USAUniv Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USA
Heberer, Kent R.
Sienko, Susan E.
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Shriners Hosp Children Portland, Portland, OR USAUniv Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USA
Sienko, Susan E.
Buckon, Cathleen E.
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Shriners Hosp Children Portland, Portland, OR USAUniv Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USA
Buckon, Cathleen E.
Bagley, Anita M.
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Shriners Hosp Children Northern Calif, Sacramento, CA USAUniv Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USA
Bagley, Anita M.
Sussman, Michael D.
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Shriners Hosp Children Portland, Portland, OR USAUniv Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USA
Sussman, Michael D.
McDonald, Craig M.
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Shriners Hosp Children Northern Calif, Sacramento, CA USA
Univ Calif Davis, Med Ctr, Sacramento, CA 95817 USAUniv Calif Los Angeles, Dept Orthopaed Surg, 22-70 Rehab Ctr,1000 Vet Ave, Los Angeles, CA 90095 USA
机构:
Virginia Commonwealth Univ, Dept Neurol, 1101 East Marshall St,POB 980599, Richmond, VA 23298 USAVirginia Commonwealth Univ, Dept Neurol, 1101 East Marshall St,POB 980599, Richmond, VA 23298 USA
Thangarajh, Mathula
Elfring, Gary L.
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PTC Therapeut Inc, South Plainfield, NJ 07080 USAVirginia Commonwealth Univ, Dept Neurol, 1101 East Marshall St,POB 980599, Richmond, VA 23298 USA
Elfring, Gary L.
Trifillis, Panayiota
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PTC Therapeut Inc, South Plainfield, NJ 07080 USAVirginia Commonwealth Univ, Dept Neurol, 1101 East Marshall St,POB 980599, Richmond, VA 23298 USA