Positivo University, Pedro Viriato Parigot de Souza St, 5300, Campo Comprido, Curitiba, Parana State, 81280-330, Brazil.
Programa de Pós-Graduação em Saúde da Criança e do Adolescente, Federal University of Parana, 181 Carneiro St, Alto da Glória, Curitiba, Parana State, 80060-900, Brazil.
Clin Nutr ESPEN. 2020 Jun;37:44-49. doi: 10.1016/j.clnesp.2020.03.024. Epub 2020 Apr 15.
Considering that phenylalanine-poor diets may be monotonous and compromise the development and nutritional status of children and adolescents with phenylketonuria, the aim of this study was to evaluate the anthropometric and biochemical characteristics of children and adolescents with this condition.
Retrospective study with anthropometric and biochemical data collection from patients with phenylketonuria in the age group 2-19.9 years. Nutritional status was classified according to the World Health Organization. Biochemical tests were compared to current recommendations.
A total of 84 patients (71.8%) were eligible, with a median age of 10.7 years (2.4-19.9 years). There was predominance of adequate (n = 58, 69%) with presence of overweight and obesity in 24 (28.5%) patients. The biochemical tests revealed hyperphosphatemia in 46 (55%), hypertriglyceridemia in 27 (50%), vitamin B12 elevated in 34 (41.2%), selenium deficiency in 10 (13.7%), insufficient zinc in 7 (8.9%), low globulin in 21 (26.9%), low HDL in 35 (59.3%) and elevated phenylalanine level in 28 (34.5%) patients in the sample. Overweight and obesity were correlated with low HDL (p = 0.04) and lowest adequate frequency of LDL (p = 0.09). Higher phosphorus values were associated with lower body weight (r = -0.72) and age (r = -0.75), as well as vitamin B12 in the same parameters (r = -0.67 and r = -0, 68). A positive correlation of phenylalanine with body weight and age (r = 0.62 and r = 0.66) was observed.
Most patients presented adequate according to anthropometric parameters and appropriate biochemical tests, except HDL, and moderate metabolic control of the disease. However, attention should be paid to the presence of overweight and need for biochemical monitoring of triglycerides, selenium, zinc, HDL, and phenylalanine.
考虑到苯丙氨酸缺乏饮食可能单调，并影响苯丙酮尿症患儿和青少年的发育和营养状况，本研究旨在评估该疾病患儿和青少年的人体测量学和生化特征。
回顾性研究，收集年龄在 2-19.9 岁的苯丙酮尿症患者的人体测量学和生化数据。根据世界卫生组织的标准对营养状况进行分类。比较生化检测结果与当前推荐值。
共有 84 名（71.8%）患者符合条件，中位年龄为 10.7 岁（2.4-19.9 岁）。其中，足够的营养状况（n=58，69%）占主导地位，24 名（28.5%）患者超重和肥胖。生化检测结果显示，46 名（55%）患者存在高磷血症，27 名（50%）患者存在高三酰甘油血症，34 名（41.2%）患者存在维生素 B12 升高，10 名（13.7%）患者存在硒缺乏，7 名（8.9%）患者存在锌不足，21 名（26.9%）患者存在低球蛋白血症，35 名（59.3%）患者存在低 HDL，28 名（34.5%）患者存在高苯丙氨酸水平。超重和肥胖与低 HDL 相关（p=0.04），与 LDL 最低的适当频率相关（p=0.09）。较高的磷值与较低的体重（r=-0.72）和年龄（r=-0.75）相关，以及与维生素 B12 相关（r=-0.67 和 r=-0.68）。还观察到苯丙氨酸与体重和年龄呈正相关（r=0.62 和 r=0.66）。
大多数患者根据人体测量学参数和适当的生化检测结果显示为足够，除了 HDL，并且疾病的代谢控制处于中等水平。然而，应注意超重的存在和需要对三酰甘油、硒、锌、HDL 和苯丙氨酸进行生化监测。
