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

Revista da Associação Médica Brasileira

Roberto José Negrão Nogueira²  Leonardo Ferreira Zimmerman¹  Yara Maria Franco Moreno¹  Cláudia Regina Comparini²  Danilo Vilela Viana¹  Tarsis Antonio Paiva Vieira²  Carlos Eduardo Steiner²  Vera Lúcia Gil-da-silva-lopes¹ 

[1] ,Universidade Estadual de CampinasCampinas SP ,Brazil

关键词: Natural history;    nutritional status;    body composition;    Williams syndrome;    História natural;    estado nutricional;    composição corporal;    síndrome de Williams;   

DOI  :  10.1590/S0104-42302011000600016

来源: SciELO

【 摘 要 】

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.

【 授权许可】

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