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Reduced bone mineral density in Chinese children with phenylketonuria

  • Kundi Wang , Ming Shen EMAIL logo , Honglei Li , Xiaowen Li and Chun He
Published/Copyright: May 9, 2017

Abstract

Background:

Phenylketonuria (PKU) is an autosomal recessive metabolic disorder. Dietary control of classic PKU needs restriction of natural proteins. The diet results in unbalanced nutrition, which might affect the physical development of the patients. Our aim was to evaluate bone mineral density (BMD) in children with PKU.

Methods:

To investigate the BMD of children with PKU, 41 children with PKU and 64 healthy controls were recruited (all 3–4 years of age). Body weight and height, BMD, Phe blood levels, thyroid function, calcium, phosphorus, iron metabolism markers, and vitamin D3 were measured.

Results:

Body height and BMD of patients were lower than in controls. The BMD of controls was positively associated with age, body weight and height. In patients, BMD was positively associated with body weight. There was no correlation between Phe blood levels and BMD in patients. Blood levels of alkaline phosphatase were higher in patients compared to controls. Blood calcium levels were higher in 4-year-old patients, while the body weight was lower compared to controls. Thyroid function, iron metabolism markers, vitamin D3 levels and IGF-1 levels were normal.

Conclusions:

Reduced BMD was observed in children with phenylketonuria, but the exact reasons for this remain to be elucidated.

Acknowledgments

The authors acknowledge the invaluable participation of the children and their parents. We also thank Professor Shiping Zhao and Chief Pharmacist Gang Cui of China-Japan Friendship Hospital for their help in patient data management.

  1. Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

  2. Research funding: The study was supported by the research fund of the China-Japan Friendship Hospital Grant no. 2010-MS-11.

  3. Employment or leadership: None declared.

  4. Honorarium: None declared.

  5. Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.

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Received: 2016-8-5
Accepted: 2017-4-3
Published Online: 2017-5-9
Published in Print: 2017-5-24

©2017 Walter de Gruyter GmbH, Berlin/Boston

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