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Published on: January 29, 2018
Decreased bone mineral density in children with phenylketonuria
J R Allen1, I R Humphries, D L Waters
1James Fairfax Institute of Paediatric Clinical Nutrition, Children's Hospital, Camperdown, New South Wales, Australia.
Children with phenylketonuria (PKU) exhibit reduced bone mineralization, specifically lower total body and spine bone mineral density (BMD). This occurs despite adequate dietary intake of key minerals, indicating a complex issue requiring further investigation.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Bone Health
Background:
- Previous research indicates potential bone mineralization deficits in children with phenylketonuria (PKU).
- Understanding bone health in PKU is crucial for long-term patient outcomes.
Purpose of the Study:
- To investigate and quantify bone mineral density (BMD) in prepubertal children with PKU.
- To compare BMD between children with PKU and age-matched controls.
- To assess the influence of dietary intake on bone mass in this population.
Main Methods:
- Bone mineral density (BMD) of the total body (TBMD) and spine (SBMD) was measured using dual-energy X-ray absorptiometry (DXA).
- Measurements were taken in 32 prepubertal children with PKU and 95 control subjects.
- Dietary intake of calcium, phosphorus, and magnesium was assessed in subsets of participants.
Main Results:
- Children with PKU showed significantly lower TBMD and SBMD compared to control subjects, even after adjusting for height and weight.
- The PKU group had a higher intake of calcium, phosphorus, and magnesium than controls.
- Lower BMD in PKU children occurred despite adequate dietary intake based on current recommendations.
Conclusions:
- Prepubertal children with PKU have reduced bone mineral density.
- The observed deficit in bone mass is not explained by inadequate dietary intake of key minerals.
- Further research is necessary to determine the underlying causes and potential nutritional interventions for low BMD in PKU.
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