Anthropometric and body-mass composition suggests an intrinsic feature in Williams-Beuren syndrome

• Autor(a) principal: Nogueira, Roberto José Negrão
• Data de Publicação: 2011
• Outros Autores: Zimmerman, Leonardo Ferreira, Moreno, Yara Maria Franco, Comparini, Cláudia Regina, Viana, Danilo Vilela, Vieira, Tarsis Antonio Paiva, Steiner, Carlos Eduardo, Lopes, Vera Lúcia Gil da Silva
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da UFMS
• Texto Completo: https://repositorio.ufms.br/handle/123456789/1393; http://dx.doi.org/10.1590/S0104-42302011000600016.
• topic: História Natural; Estado Nutricional; Composição Corporal; Síndrome de Williams; Natural History; Nutritional Status; Body Composition; Williams Syndrome

OBJECTIVE:Although considered a well-known condition, there is only one study describing the body composition among individuals with Williams-Beuren syndrome. The aim was to characterize the nutritional status in Brazilian individuals with this condition. METHODS: Cross-sectional study was designed to evaluate clinical and nutritional data of 17 Brazilian patients. Z-scores for height, weight, body mass index, triceps and subscapular skinfold thickness, arm circumference, arm muscle area, arm fat area were calculated. Wilcoxon's test was used to investigate differences between the z-scores of the anthropometrical measures and zero. RESULTS: Four children were considered stunted and two severely malnourished. The z-score mean value for height was -1.14 ± 1.00 (p-value = 0.004), for weight, -0.67 ± 1.19 (p-value = 0.0443), for arm circumference, -0.94 ± 1.14 (p-value = 0.0222), for triceps skinfold thickness, -0.59 ± 0.63 (p-value = 0.0042) and for arm fat area -0.67 ± 0.67 (p-value = 0.0061). CONCLUSION: Short stature seen in this series confirms a previous study describing this feature in a German population, which would suggest it as an intrinsic feature in Williams-Beuren syndrome. In addition, skinfold thickness measures have not been previously performed in this syndrome and detected abnormalities in fat stores in this sample. Considering this method a fast and low-cost way to evaluate body composition, similar studies could be performed in other populations in order to better characterize this issue. Morbidity related with this genetics condition and information for clinical investigation and clinical follow-up are also discussed.