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Association Between Body Mass Index and Disability in Children With Charcot-Marie-Tooth Disease
Gabrielle A Donlevy1, Sarah P Garnett2, Kayla M D Cornett2
1From the University of Sydney (G.A.D., S.P.G., M.P.M.), Faculty of Medicine and Health; Children's Hospital at Westmead (G.A.D., S.P.G., K.M.D.C., J.B., M.P.M.); University of Sydney (K.M.D.C., M.J.M., J.B.), School of Health Sciences; Faculty of Health and Medicine (J.N.B.), University of Newcastle, Australia; Departments of Pediatrics (R.R.S.) and Neurology (M.E.S.), Carver College of Medicine, University of Iowa, Iowa City; Division of Neurology (S.W.Y.) and Department of Occupational Therapy (T.E.), Children's Hospital of Philadelphia; Department of Neurology (S.W.Y., T.E.), Perelman School of Medicine, University of Pennsylvania, Philadelphia; Fondazione IRCCS Istituto Neurologico Carlo Besta (I.M., M.F., E.P., D.P.), Milan, Italy; Centre for Neuromuscular Diseases (M.L., M.M.R.), University College London, Queen Square; University College London Institute of Child Health & Great Ormond Street Hospital (T.B., F.M.), London, England; Translational Neurosciences (Pediatrics) (R.S.F.), St. Jude Children's Research Hospital, Memphis, TN; and Department of Neurology (J.E.S., K.J.E., D.N.H.), University of Rochester, NY. gabrielle.donlevy@health.nsw.gov.au.
Insights
Children with Charcot-Marie-Tooth disease (CMT) show higher rates of being underweight or obese. Both underweight and obesity in pediatric CMT patients correlate with increased disability compared to healthy-weight peers.
Area of Science:
- Pediatric Neurology
- Metabolic Disorders
- Genetics
Background:
- Charcot-Marie-Tooth disease (CMT) is a group of inherited disorders affecting peripheral nerves.
- Understanding factors influencing disability in pediatric CMT is crucial for management.
- Body Mass Index (BMI) is a key indicator of nutritional status and metabolic health.
Purpose of the Study:
- To investigate the association between Body Mass Index (BMI) categories and disability levels in children diagnosed with Charcot-Marie-Tooth disease (CMT).
- To compare BMI distribution in pediatric CMT patients with healthy, age- and sex-matched controls.
Main Methods:
- A cross-sectional analysis was performed on 477 pediatric patients (3-20 years) with CMT and 316 healthy children.
- Body Mass Index (BMI) was categorized using International Obesity Task Force (IOTF) criteria.
- Disability was assessed using the CMT Pediatric Scale (CMTPedS) scores.
Main Results:
- Children with CMT exhibited a higher prevalence of severe underweight, underweight, and obesity compared to healthy controls.
- Patients with CMT who were severely underweight or obese demonstrated significantly higher disability scores (CMTPedS) than those with a healthy weight.
- No significant difference in overweight prevalence was observed between CMT patients and controls.
Conclusions:
- Pediatric populations with CMT are disproportionately affected by underweight and obesity.
- Both underweight and obesity are associated with increased disability in children with CMT.
- These findings highlight the importance of monitoring BMI in pediatric CMT for better disability management.
Background And Objectives:
This study examined the association between body mass index (BMI) and disability in children with Charcot-Marie-Tooth disease (CMT).
Methods:
We conducted a cross-sectional analysis of 477 patients with CMT who were 3 to 20 years of age from the Inherited Neuropathy Consortium and 316 age- and sex-matched healthy children from the 1,000 Norms Project. BMI was categorized according to the International Obesity Task Force (IOTF) criteria, and BMI categorization was compared with healthy children. IOTF categories (adult equivalent BMI cut points) were severely underweight (BMI <17 kg/m2), underweight (BMI ≥17-<18.5 kg/m2), healthy weight (BMI ≥18.5-<25 kg/m2), overweight (BMI ≥25-<30 kg/m2), and obese (BMI ≥30 kg/m2). Scores on the 0 to 44-point CMT Pediatric Scale (CMTPedS), a well-validated measure of disability, were examined in relation to BMI.
Results:
There was a higher proportion of children with CMT categorized as severely underweight (5.7% vs 0.3%), underweight (10.3% vs 5.1%), and obese (7.3% vs 3.8%) (p < 0.05). Fewer children with CMT were categorized as healthy weight (61.8% vs 74.4%) (p < 0.05), and the proportion of overweight (14.9% vs 16.5%) between groups was similar. CMTPedS scores (mean ± SD) for weight categories were as follows: severely underweight 27 ± 9, underweight 20 ± 8, healthy weight 17 ± 9, overweight 17 ± 9, and obese 22 ± 10. Compared to children with a healthy weight with CMT, being severely underweight was associated with being more disabled (p < 0.001), as was being obese (p = 0.015).
Discussion:
The proportion of children with CMT who are underweight or obese is higher compared to age- and sex-matched healthy children. In children with CMT, being underweight or obese is associated with greater disability, when compared to children with CMT of healthy weight.
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