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Elevated vitamin A intake and serum retinol in preadolescent children with cystic fibrosis ^{1 ,2 ,3}

¹ From the Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA (RCG-M, JIS, BSZ, NS, and VAS), and the Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania School of Medicine, Philadelphia, PA (NS)

Background: Persons with cystic fibrosis (CF) and pancreatic insufficiency (PI) are at risk of vitamin A deficiency because of steatorrhea, despite pancreatic enzyme replacement. Long-standing vitamin A supplementation may increase the risk of vitamin A toxicity.

Objective: The aim was to describe the vitamin A intake and serum retinol concentrations of preadolescent children with CF, PI, and mild-to-moderate pulmonary disease, who were cared for under current practice recommendations.

Design: This cross-sectional study evaluated children aged 8.0–11.9 y with CF and PI from 13 US CF centers. Dietary and supplemental vitamin A intakes were compared with the Dietary Reference Intakes (DRIs) for healthy children, CF recommendations, and data from the National Health and Nutrition Examination Survey (NHANES), 1999–2000. Serum retinol concentrations were compared with NHANES data.

Results: The 73 subjects with CF had a dietary vitamin A intake of 816 ± 336 µg retinol activity equivalents (165 ± 69% of the recommended dietary allowance), which was similar to the NHANES value. The supplement intake provided 2234 ± 1574 µg retinol activity equivalents/d and exceeded recommendations in 21% of the subjects with CF. Total preformed retinol intake exceeded the DRI tolerable upper intake level in 78% of the subjects with CF. The serum retinol concentration was 52 ± 13 µg/dL (range: 26–98 µg/dL), which was significantly higher than the NHANES value (37 ± 10 µg/dL; range: 17–63 µg/dL; P < 0.001).

Conclusion: Although supplementation helps to prevent vitamin A deficiency in children with CF and PI, their high vitamin A intakes and serum retinol concentrations suggest that usual care may result in excessive vitamin A intake and possible toxicity that would increase the risk of CF-associated liver and bone complications.

Key Words: Vitamin A • retinol • hypervitaminosis A • cystic fibrosis • children • National Health and Nutrition Examination Survey • NHANES