A Survey of Eating Attitudes and Behaviors in Adolescents and Adults With Phenylalanine Hydroxylase Deficiency.

Autor:	Luu S; Master of Genetic Counselor Studies Training Program, University of Wisconsin-Madison, Madison, Wisconsin, luusm8@gmail.com., Breunig T; UW Biochemical Genetics Clinic, University of Wisconsin-Madison, Madison, Wisconsin., Drilias N; UW Biochemical Genetics Clinic, University of Wisconsin-Madison, Madison, Wisconsin., Kuhl A; UW Biochemical Genetics Clinic, University of Wisconsin-Madison, Madison, Wisconsin., Scott Schwoerer J; UW Biochemical Genetics Clinic, University of Wisconsin-Madison, Madison, Wisconsin., Cody P; Division of General Pediatrics and Adolescent Medicine, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.
Jazyk:	angličtina
Zdroj:	WMJ : official publication of the State Medical Society of Wisconsin [WMJ] 2020 Mar; Vol. 119 (1), pp. 37-43.
Abstrakt:	Introduction: Phenylalanine hydroxylase deficiency, commonly known as phenylketonuria (PKU), is an inborn error of metabolism that manifests in severe neurological damage when left untreated. Routine newborn screening has made early identification and treatment of affected individuals possible, changing the prognosis of PKU from devastating to excellent. The most effective treatment for PKU involves lifelong dietary restriction of protein, nutrition supplementation via medical foods, and frequent monitoring of amino acid levels in the blood. However, it has been observed that imposing strict medical control over daily dietary habits can lead to destructive attitudes towards eating and body image. This study investigated whether people with PKU are at increased risk of disordered eating behaviors and attitudes. Methods: Fifteen patients with PKU between the ages of 12 and 35 from the University of Wisconsin (UW) Biochemical Genetics Clinic were surveyed about their metabolic management and eating attitudes and behaviors. Results: While this study was too small to make conclusions of clinical significance, our findings did suggest that patients with poor metabolic control exhibited symptoms of disordered eating at a higher frequency than those with good metabolic control. Conclusions: There is currently no validated screening tool to evaluate for disordered eating behaviors in individuals with PKU, which makes identifying and treating disordered eating and related conditions difficult. The development of this project emphasized the importance of tailored screening and provider awareness for disordered eating for populations with chronic illnesses. (Copyright© Board of Regents of the University of Wisconsin System and The Medical College of Wisconsin, Inc.)
Databáze:	MEDLINE
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