25-hydroxyvitamin-d-2 and Phenylketonurias

A higher incidence of osteopenia is observed among children with inherited metabolic disorders (inborn errors of metabolism, or IEMs) who consume medical food-based diets that restrict natural vitamin D-containing food sources. We evaluated the vitamin D status of children with IEMs who live in the Pacific Northwest with limited sun exposure and determined whether bone mineral density (BMD) in children with phenylketonuria (PKU), the most common IEM, correlated with diet or biochemical markers of bone metabolism. We hypothesized that children with IEMs would have lower serum vitamin D concentrations than controls and that some children with PKU would have reduced bone mineralization. A retrospective record review of 88 patients with IEMs, and 445 children on unrestricted diets (controls) found the 25-hydroxyvitamin D concentrations were normal and not significantly different between groups (IEM patients, 27.1 ± 10.9; controls, 27.6 ± 11.2). Normal BMD at the hip or spine (-2

Topics: 25-Hydroxyvitamin D 2; Academic Medical Centers; Adolescent; Adult; Biomarkers; Bone Density; Bone Diseases, Developmental; Calcifediol; Child; Cohort Studies; Cross-Sectional Studies; Electronic Health Records; Food, Formulated; Humans; Incidence; Metabolism, Inborn Errors; Oregon; Phenylketonurias; Retrospective Studies; Risk; Vitamin D Deficiency; Young Adult