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Child , Humans , Galactosemias/physiopathology , Osteoporosis/etiology , Phenylketonurias/physiopathology , Bone Density , Diet , Absorptiometry, PhotonABSTRACT
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.
Subject(s)
Bone Density , Bone Resorption/etiology , Galactosemias/physiopathology , Phenylketonurias/physiopathology , Adolescent , Child , Child, Preschool , Diet , Female , Humans , MaleABSTRACT
Introducción. Con el fin de conocer mejor la fisiopatología de la osteoporosis que tienen riesgo de desarrollar los pacientes afectados de algunos errores congénitos del metabolismo se ha estudiado el recambio mineral óseo en 10 niños afectados de hiperfenilalaninemia media, siete de fenilcetonuria y seis de galactosemia, con un control estricto de la ingesta y unas recomendaciones de ejercicio físico adecuadas. Material y método. Se han analizando marcadores de resorción ósea, hidroxiprolina y piridinolina urinaria y de aposición ósea, osteocalcina y propéptido C terminal sérico. La densidad mineral ósea se ha analizado mediante densitometría por ultrasonidos. Resultados. Se objetiva que la densitometría ósea está disminuida respecto a la población normal sin llegar a ser estadísticamente significativo. El recambio mineral óseo está ligeramente disminuido en pacientes con fenilcetonuria, mientras que se encuentra dentro del rango de normalidad en pacientes con hiperfenilalaninemia media y galactosemia. Conclusión. El adecuado control de la ingesta dietética, tanto proteica como mineral, así como del régimen de vida saludable pueden prevenir la aparición de alteraciones significativas de la mineralización ósea
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
Subject(s)
Child , Adolescent , Child, Preschool , Humans , Galactosemias/physiopathology , Phenylketonurias/physiopathology , Bone Density , Bone Resorption/etiology , DietABSTRACT
Objetivo: Obtener el perfil normal de los marcadores del recambio óseo durante la infancia para poder valorar el estado de mineralización ósea de la población infantil normal y de la población de riesgo para osteoporosis. Pacientes y métodos: Se ha estudiado una población de 75 niños sanos de entre 6 meses y 14 años de edad. Se han determinado en suero los valores de osteocalcina y de propéptido carboxiterminal del procolágeno tipo I como marcadores de aposición ósea y de la hidroxiprolina y las piridinolinas en orina, como marcadores de resorción ósea. Se ha realizado el análisis estadístico de los resultados. Resultados: Los valores más elevados para estos marcadores se han obtenido en los primeros 4 años de vida. A continuación los de resorción ósea presentan una continua disminución estadísticamente significativa hasta los 14 años de edad (p < 0,05), mientras que los de aposición ósea descienden ligeramente a partir de los 4 años y se mantienen posteriormente estables hasta los 14 años. Discusión: Este comportamiento es compatible con la presencia de un intenso recambio óseo durante los 4 primeros años, y con un predominio de los fenómenos de aposición ósea a lo largo de los primeros 14 años de vida. El conocimiento de los valores normales de dichos marcadores permite valorar el estado de la mineralización ósea de la población infantil normal y de la población de riesgo para osteoporosis (AU)
Subject(s)
Humans , Infant , Child , Adolescent , Child, Preschool , Pediatrics , Palliative Care , Hospitals, Pediatric , Professional-Family Relations , Family , Hydroxyproline , Reference Values , Peptide Fragments , Delivery of Health Care , Attitude to Death , Procollagen , Terminal Care , Quality of Life , Osteocalcin , Amino Acids , Spain , Bone and Bones , SpainABSTRACT
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.
