巴西苯丙酮尿症患者的身体成分和营养状况分析。
Analysis of body composition and nutritional status in Brazilian phenylketonuria patients.
作者信息
Mazzola Priscila Nicolao, Nalin Tatiele, Castro Kamila, van Rijn Margreet, Derks Terry G J, Perry Ingrid D S, Mainieri Alberto Scofano, Schwartz Ida Vanessa D
机构信息
Programa de Pós-Graduação em Ciências Biológicas: Bioquímica, Universidade Federal do Rio Grande do Sul (UFRGS), Ramiro Barcelos 2600 anexo, 90035-003, Porto Alegre, Brazil; Beatrix Children's Hospital, Section of Metabolic Diseases, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700 RB, Groningen, The Netherlands.
Post-Graduation Program in Genetics and Molecular Biology, UFRGS, Bento Gonçalves 9500/43323M, PO Box 15053, Porto Alegre, Brazil.
出版信息
Mol Genet Metab Rep. 2016 Jan 9;6:16-20. doi: 10.1016/j.ymgmr.2015.12.003. eCollection 2016 Mar.
BACKGROUND
Phenylketonuria (PKU) is characterized by phenylalanine (Phe) accumulation to toxic levels due to the low activity of phenylalanine-hydroxylase. PKU patients must follow a Phe-restricted diet, which may put them in risk of nutritional disturbances. Therefore, we aimed to characterize body composition parameters and nutritional status in Brazilian PKU patients also considering their metabolic control.
METHODS
Twenty-seven treated PKU patients older than 5 years, and 27 age- and gender-matched controls, were analyzed for anthropometric features and body composition by bioelectrical impedance (BIA). Patients' metabolic control was assessed by historical Phe levels.
RESULTS
There was no effect of PKU type, time of diagnosis, or metabolic control for any analyzed parameter. About 75% of patients and controls were eutrophic, according to their BMI values. There were no difference between groups regarding body composition and other BIA-derived parameters.
CONCLUSIONS
Brazilian PKU patients do not show differences in body composition and nutritional status in comparison with controls, regardless metabolic control. Although similar to controls, PKU patients may be in risk of disturbed nutritional and metabolic markers as seen for the general population.
背景
苯丙酮尿症（PKU）的特征是由于苯丙氨酸羟化酶活性低下，苯丙氨酸（Phe）积累至毒性水平。PKU患者必须遵循低苯丙氨酸饮食，这可能使他们面临营养紊乱的风险。因此，我们旨在描述巴西PKU患者的身体成分参数和营养状况，并考虑他们的代谢控制情况。
方法
对27名5岁以上接受治疗的PKU患者以及27名年龄和性别匹配的对照者进行人体测量特征分析，并通过生物电阻抗（BIA）测量身体成分。通过历史苯丙氨酸水平评估患者的代谢控制情况。
结果
PKU类型、诊断时间或代谢控制对任何分析参数均无影响。根据BMI值，约75%的患者和对照者营养状况良好。两组在身体成分和其他BIA衍生参数方面无差异。
结论
与对照组相比，巴西PKU患者在身体成分和营养状况方面没有差异，无论其代谢控制情况如何。尽管与对照组相似，但PKU患者可能像普通人群一样面临营养和代谢指标紊乱的风险。
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