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Introduction: Patients with any type of congenital metabolism error are at risk for developing osteoporosis. To gain further insight into the physiopathology of this disease, we studied bone mineral turnover in 10 children with hyperphenylalaninemia, seven with phenylketonuria and six with galactosemia. Oral intake was strictly controlled and the children followed recommendations for physical exercise., Material and Method: Markers of bone resorption (hydroxyproline and pyridinoline in urine samples) and markers of bone formation (levels of osteocalcin and C-terminal procollagen peptide type I) were analyzed. Bone mineral density was analyzed by ultrasound densitometry., Results: A non-significant reduction in bone densitometry with respect to the normal population was observed. Bone mineral turnover was slightly diminished in patients with phenylketonuria but was within the normal range in patients with hyperphenylalaninemia and galactosemia., Conclusion: Adequate control of dietary intake of both proteins and minerals, as well as a healthy lifestyle, can prevent the development of significant alterations in bone mineralization.

Objective: To determine the normal profile of bone turnover markers in childhood in order to enable evaluation of bone mineralization status in the healthy pediatric population and in the population at risk of osteoporosis., Patients and Methods: A population of 75 healthy children aged between 6 months and 14 years was studied. Levels of osteocalcin and C-terminal procollagen peptide type I, as markers of bone apposition, were determined in serum. Levels of hydroxyproline and pyridinoline, as markers of bone resorption, were determined in urine samples. Statistical analysis of the results was performed., Results: The highest levels of the four markers were obtained in the first 4 years of life. Markers of bone resorption showed a continuous statistically significant decrease until the age of 14 years (p < 0.05), whereas markers of bone apposition decreased slightly after the age of 4 years and then remained stable until the age of 14 years., Discussion: These findings are compatible with the presence of intense bone turnover in the first 4 years of life and with a predominance of the phenomenon of bone apposition throughout the first 14 years of life. Knowledge of the normal profile of these markers allows evaluation of bone mineralization status in the healthy pediatric population and in the population at risk of osteoporosis.