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Tipo: Artigo de Periódico
Título: Anthropometric and body-mass composition suggests an intrinsic feature in Williams-Beuren syndrome
Autor(es): Nogueira, Roberto José Negrão
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
Abstract: 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.
Palavras-chave: História Natural
Estado Nutricional
Composição Corporal
Síndrome de Williams
Natural History
Nutritional Status
Body Composition
Williams Syndrome
Editor: Revista da Associação Médica Brasileira
Citação: NOGUEIRA, Roberto José Negrão et al . Anthropometric and body-mass composition suggests an intrinsic feature in Williams-Beuren syndrome. Rev. Assoc. Med. Bras., São Paulo, v. 57, n. 6, Dec. 2011 . Available from <>. access on 17 May 2012.
Tipo de Acesso: Acesso Aberto
Identificador DOI:
Data do documento: Nov-2011
Aparece nas coleções:FAMED - Artigos publicados em periódicos

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