November 2012

Document Type


Degree Name



Oregon Health & Science University


Background: Recent research in children with Phenylketonuria (PKU), the most common inherited disorder of amino acid metabolism, suggest they have decreased bone mineral density (BMD). Although the etiology of this problem is unknown, children with PKU and other inherited metabolic disorders consume specialized diets that often severely restrict vitamin D-containing food sources. The primary goals of this study were to evaluate the prevalence of vitamin D deficiency in a group of children with inherited metabolic disorders who are consuming a medical food diet, and determine whether BMD in children with PKU correlates with diet and/or biochemical markers of bone metabolism. Objectives; 1) To compare serum 25-hydroxy-vitamin 0 concentrations in children with inborn errors of metabolism (IEM) that are consuming a medically modified diet to those in a group of control children without the diagnosis of an IEM who consume an unrestricted diet. 2) To determine whether the BMD in a group o




Graduate Programs in Human Nutrition


School of Medicine



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