PDF(Pubmed)
Abstract：
Morphofunctional assessment was developed to evaluate disease-related malnutrition. However, it can also be used to assess cardiometabolic risk, as excess adiposity increases this risk. Phenylketonuria (PKU) is the most prevalent inherited metabolic disease among adults, and obesity in PKU has recently gained interest, although fat mass correlates better with cardiometabolic risk than body mass index. In this systematic review, the objective was to assess whether adult patients with PKU have higher fat mass than healthy controls. Studies of adult PKU patients undergoing dietary treatment in a metabolic clinic reporting fat mass were included. The PubMed and EMBASE databases were searched. Relevance of articles, data collection, and risk of bias were evaluated by two independent reviewers. Ten articles were evaluated, six with a control group, including 310 subjects with PKU, 62 with mild hyperphenylalaninemia, and 157 controls. One study reported a significant and four a tendency towards an increased fat mass in all patients or only females with PKU. Limitations included not having a healthy control group, not reporting sex-specific results and using different techniques to assess fat mass. Evaluation of fat mass should be included in the morphofunctional assessment of cardiometabolic risk in adult patients with PKU.
摘要：
形态功能评估是为了评估疾病相关的营养不良。然而,它也可以用来评估心脏代谢风险，过度肥胖会增加这种风险。苯丙酮尿症（PKU）是成年人中最常见的遗传代谢疾病，PKU的肥胖最近引起了人们的兴趣，尽管与体重指数相比，脂肪量与心脏代谢风险的相关性更好。在这次系统审查中，目的是评估成人PKU患者的脂肪量是否高于健康对照组.包括在代谢诊所接受饮食治疗的成年PKU患者的研究，报告脂肪量。检索PubMed和EMBASE数据库。文章的相关性，数据收集，和偏倚风险由两名独立评审员进行评估.评估了十篇文章，六个和一个对照组，包括310名PKU患者，62患有轻度高苯丙氨酸血症，157个控件。一项研究报告了所有PKU患者或仅女性患者的脂肪量增加的趋势，而有四个趋势。限制包括没有健康对照组，不报告性别特异性结果，并使用不同的技术来评估脂肪量。脂肪量的评估应包括在成人PKU患者心脏代谢风险的形态功能评估中。
