Article Information
Article Type: Research Article
Citation: Marcelino CA, Oliveira CR, Gelelete CB, Horovitz DDG, da Silva RTB, et al. (2015) Body Composition of Individuals with Mucopolysaccharidosis. Int J Endocr Metab Disord 1 (1): http://dx.doi.org/10.16966/2380-548X.101
Copyright:© 2015 Marcelino CA et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited
Publication history:
Authors :
Cristiano A Marcelino1* Camila R Oliveira1 Cristina B Gelelete1 Dafne DG Horovitz2 Raquel TB da Silva3 Patrícia S Vigário4 Márcia G Ribeiro5
1Internal Medicine Postgraduate Program, Federal University of Rio de Janeiro (UFRJ), RJ – Brazil2 Department of Medical Genetics, Fernandes Figueira Institute (IFF), Oswaldo Cruz Foundation (FIOCRUZ), RJ – Brazil3 Department of Medical Genetics, Pedro Ernesto University Hospital (HUPE), State University of Rio de Janeiro (UERJ), RJ – Brazil4 Rehabilitation Science Postgraduate Program, Augusto Motta University Center (UNISUAM), RJ – Brazil5 Department of Medical Genetics, Martagão Gesteira Institute of Pediatrics (IPPMG), UFRJ, RJ – Brazil
*Corresponding author: Cristiano de Abreu Marcelino, Internal Medicine Postgraduate Program, Federal University of Rio de Janeiro (UFRJ), Rua Maurício de Abreu, 2070 – Neves, São Gonçalo, RJ – Brazil 24425-300; E-mail: cmarcelino@ufrj.br
Abstract
Objective: To describe body composition of individuals with mucopolysaccharidosis (MPS).
Methods: A cross-sectional study was carried on with a convenience sample of MPS individuals of both sexes, five years of age or older. Exclusion criteria: physical deformity, neurological problems or other associated syndrome, precluding full measure’s accomplishment. Body composition was estimated by anthropometric method, according to international standards. Accomplished measurements of skinfold, body girth, bone diameters, total body mass and stature were made. Body mass index (BMI), body fat percent (BF%) and body mass were calculated. Body composition was also evaluated through Dual-energy X-ray Absorptiometry (DXA). Results: Twenty-three individuals were identified; three were excluded (cognitive deficit) and five losses occurred. Fifteen individuals were evaluated (average age = 12.1 ± 7.7 years); three MPS I, five MPS II, one MPS IV and six MPS VI. For DXA, eight were included (average age = 14.1 ± 9.8 years), six common to the anthropometric assessment. BMI average: 19.8 ± 3.8 kg/m²; BF% average: 13.9 ± 5.1%. Short stature was present in 76.9%, obesity in 25.0%, macrocephaly in 26.6%; osteoporosis in 25.0%. Perimeters’ measures increased with the increasing of age. Bone lengths were not always shortened and there was no pattern related to age and MPS type. No significant differences were observed between anthropometry and DXA. The results varied in the same type of MPS and in the same age group. Conclusions: Physical parameters were in agreement with literature, as short stature and macrocephaly. Measurements’ variations between different MPS types and among individuals of the same type strengthen the heterogeneity of this disease.
Keywords
Mucopolysaccharidosis; Anthropometry; Body composition; Children and adolescents
Figure 1: Distribution of body circumferences in relation to age
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