Coakley Kathryn E, Porter-Bolton Suzanne, Salvatore Mary L, Blair Rosalynn B, Singh Rani H
Nutrition and Dietetics Program; Department of Individual, Family, and Community Education University of New Mexico Albuquerque New Mexico USA.
Chief Turnaround Office Georgia Department of Education Atlanta Georgia USA.
JIMD Rep. 2020 Mar 20;53(1):103-110. doi: 10.1002/jmd2.12115. eCollection 2020 May.
Phenylketonuria (PKU) is a genetic disorder characterized by insufficient metabolism of phenylalanine. Depending on severity, patients follow a low-phenylalanine diet and may consume medical food (MF) and low-protein modified foods; dietary and medical treatment can be expensive. This study assessed prevalence of food insecurity (FI), the lack of resources to access enough nutritious food to have an active, healthy life, in females with PKU and examined associations with diet and metabolic control. Participants were recruited from a research-based camp in 2018. Adult and adolescent modules of the USDA Household Food Security survey were utilized to categorize participants as food secure [high food security (FS) or marginal FS] or food insecure (low FS or very low FS); results were compared to the general U.S. population. Dietary intake via three-day food records and plasma amino acids were also assessed. Thirty females 11-58 years of age (mean = 21.4 years) participated. Twelve (40%), including seven adolescents (44%) and five adults (36%), were FI compared to the U.S. prevalence of 11.1%. MF protein intake was significantly lower in those with very low FS compared to high FS and low FS ( = .04). Age and intact protein intake were significantly higher in those with very low FS compared to high FS ( < .05). Our study suggests adolescent and adult females with PKU have a higher prevalence of FI than the general U.S. population. Those with very low FS were older, consumed more dietary phenylalanine and intact protein, and less MF protein. Clinicians should consider screening for FI in patients with PKU.
苯丙酮尿症（PKU）是一种遗传性疾病，其特征是苯丙氨酸代谢不足。根据病情严重程度，患者需遵循低苯丙氨酸饮食，可食用医用食品（MF）和低蛋白改良食品；饮食和医学治疗费用可能很高。本研究评估了苯丙酮尿症女性患者中粮食不安全（FI）的患病率，即缺乏获取足够营养食物以过上积极健康生活的资源，并研究了其与饮食和代谢控制的关联。参与者于2018年从一个基于研究的营地招募。利用美国农业部家庭粮食安全调查的成人和青少年模块将参与者分类为粮食安全[高粮食安全（FS）或边缘FS]或粮食不安全（低FS或极低FS）；结果与美国普通人群进行了比较。还评估了通过三天食物记录得出的饮食摄入量和血浆氨基酸。30名年龄在11至58岁（平均=21.4岁）的女性参与了研究。与美国11.1%的患病率相比，有12人（40%）粮食不安全，其中包括7名青少年（44%）和5名成年人（36%）。与高FS和低FS相比，极低FS者的MF蛋白质摄入量显著更低（=0.04）。与高FS相比，极低FS者的年龄和完整蛋白质摄入量显著更高（<0.05）。我们的研究表明，患有苯丙酮尿症的青少年和成年女性的粮食不安全患病率高于美国普通人群。极低FS者年龄更大，摄入的膳食苯丙氨酸和完整蛋白质更多，而MF蛋白质更少。临床医生应考虑对苯丙酮尿症患者进行粮食不安全筛查。
