苯丙酮尿症青少年患者糖代谢状态及其与饮食的相关性

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Abstract Objective To investigate the relationship between glucose metabolism and diet control in adolescents with phenylketonuria (PKU). Methods A total of 96 PKU patients for regular reexamination in Chengde Maternal and Child health Care Hospital (“our hospital” for short) were selected from September 2016 to March 2019, and they were divided into PKU group (phenylalanine concentration in 240-600 μmol/L was good control group, and phenylalanine concentration ＞600 μmol/L was poorly control group), 100 healthy adolescents of the same age who came to our hospital for physical examination in the same period were selected as the control group. The relationship between dietary status, Phe control level and insulin resistance and the risk factors of diabetes mellitus in PKU group were analyzed. Results The daily carbohydrate intake, incidence of abnormal glucose tolerance, fasting glucose, blood glucose level at 2 h after meal and homeostasis insulin resistance index (HOMA-IR) in the well-controll group (64 cases) were higher than those in the poorly controll group (32 cases), with statistically significant differences (all P ＜ 0.05). Daily carbohydrate intake was positively correlated with HOMA-IR, while Phe control level was negatively correlated with HOMA-IR (all P ＜ 0.05). High daily carbohydrate intake was an independent risk factor for abnormal glucose tolerance and diabetes in adolescents (OR = 1.048, P ＜ 0.05), and Phe control level was a protective factor (OR = 0.992, P ＜ 0.05). Conclusion The risk of abnormal glucose tolerance and insulin resistance in adolescents with PKU may be associated with higher carbohydrate intake.

Key words： Phenylketonuria Glucose metabolism Phenylalanine Diet control

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	ZUO Xuemei JIN yuqi QIU Hainan

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ZUO Xuemei JIN yuqi QIU Hainan. Glucose metabolism status and the correlation with diet in adolescents with phenylketonuria[J]. 中国医药导报, 2020, 17(8): 156-159.

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http://www.yiyaodaobao.com.cn/EN/ OR http://www.yiyaodaobao.com.cn/EN/Y2020/V17/I8/156

[1] 黄奎奎，郝胜菊，王兴，等.甘肃省2009年-2016年苯丙酮尿症筛查现况与发病趋势分析[J].中国优生与遗传杂志，2017，25（2）：95-96.
[2] 韩宗兰，王兰英，王海楠，等.影响苯丙酮尿症患儿生活质量的相关因素及干预措施[J].中国医药导报，2019， 16（6）：90-93.
[3] Andrade F，Villate O，Couce ML，et al. Asymmetric dimethylarginine as a potential biomarker for management and follow-up of phenylketonuria [J]. Eur J Pediatr，2019，178（6）：903-911.
[4] 秦玉兰，苏雅洁. 苯丙酮尿症治疗研究新进展[J].国际儿科学杂志，2018，45（8）：624-628.
[5] Andrade F，Lopez-Suarez O，Llarena M，et al. Influence of phenylketonuria′s diet on dimethylated arginines and methylation cycle [J]. Medicine （Baltimore），2017，96（27）：e7392.
[6] Verduci E，Banderali G，Moretti F，et al. Diet in children with phenylketonuria and risk of cardiovascular disease: A narrative overview [J]. Nutr Metab Cardiovasc Dis，2016， 26（3）：171-177.
[7] 王卫平.儿科学[M].8版.北京：人民卫生出版社，2013：77-79.
[8] Kumru B，Ozturk HB，Kaplan DS，et al. Studying the effect of large neutral amino acid supplements on oxidative stress in phenylketonuric patients [J]. J Pediatr Endocrinol Metab，2019，32（3）：269-274.
[9] 中华医学会糖尿病学分会.中国2型糖尿病防治指南（2013年版）[J].中华内分泌代谢杂志，2014，30（10）：893-942.
[10] 邱婷，许吟，王丽珍，等.早期饮食治疗的苯丙酮尿症儿童生活质量现状分析[J].中国儿童保健杂志，2018，26（7）：796-798.
[11] Hozyasz KK，Zolkowska J. Diversity of phenylalanine tolerance in pregnant phenylketonuria patients homozygous for the p.R408W mutation: the need for improved understanding of phenylalanine homeostasis [J]. J Biol Regul Homeost Agents，2019，33（2）：491-497.
[12] Muniz JRC，Szeto NW，Frise R，et al. Role of protein structure in variant annotation: structural insight of mutations causing 6-pyruvoyl-tetrahydropterin synthase deficiency [J]. Pathology，2019，51（3）：274-280.
[13] Kumru B，Ozturk HB，Kaplan DS，et al. Studying the effect of large neutral amino acid supplements on oxidative stress in phenylketonuric patients [J]. J Pediatr Endocrinol Metab，2019，32（3）：269-274.
[14] Couce ML，Sánchez-Pintos P，Vitoria I，et al. Carbohydrate status in patients with phenylketonuria [J]. Orphanet J Rare Dis，2018，13（1）：103.
[15] Kanufre VC，Soares RD，Alves MR，et al. Metabolic syndrome in children and adolescents with phenylketonuria [J]. J Pediatr （Rio J），2015，91（1）：98-103.
[16] Moretti F，Pellegrini N，Salvatici E，et al. Dietary glycemic index，glycemic load and metabolic profile in children with phenylketonuria [J]. Nutr Metab Cardiovasc Dis，2017， 27（2）：176-182.
[17] 刘翛然，陈曦，夏燕，等. 新疆地区经典型苯丙酮尿症青少年治疗依从性影响因素分析[J].中国青少年保健杂志，2016，24（2）：213-216.
[18] 韩宗兰，王兰英，王海楠，等.影响苯丙酮尿症患儿生活质量的相关因素及干预措施[J].中国医药导报，2019， 16（6）：90-93.
[19] Pinto A，Almeida MF，Ramos PC，et al. Nutritional status in patients with phenylketonuria using glycomacrope-ptide as their major protein source [J]. Eur J Clin Nutr，2017，71（10）：1230-1234.
[20] Alfheeaid H，Gerasimidis K，Nastase AM，et al. Impact of phenylketonuria type meal on appetite，thermic effect of feeding and postprandial fat oxidation [J]. Clin Nutr，2018，37（3）：851-857.
[21] Sumanszki C，Kiss E，Simon E，et al. The Association of Therapy Adherence and Thyroid Function in Adult Patients with Phenylketonuria [J]. Ann Nutr Metab，2019， 75（1）：16-23.