Albina Tummolo1, Giacomina Brunetti2, Mario Giordano3, Vincenza Carbone3, Maria Felicia Faienza4, Maurizio Aricò5, Sabino Pesce6. 1. Department of Metabolic Diseases, Clinical Genetics and Diabetology, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, Via Amendola 207, 70126, Bari, Italy. albina.tummolo@policlinico.ba.it. 2. Section of Human Anatomy and Histology, Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari A. Moro, Bari, Italy. 3. Pediatric Nephrology and Dialysis Unit, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, Bari, Italy. 4. Department of Biomedical Sciences and Human Oncology, Pediatric Section, University "A. Moro", Bari, Italy. 5. Rare Diseases Unit, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, Bari, Italy. 6. Department of Metabolic Diseases, Clinical Genetics and Diabetology, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, Via Amendola 207, 70126, Bari, Italy.
Abstract
PURPOSE: To evaluate the use of QUS for the bone status assessment in children cared because of a chronic disease such as: inherited metabolic disorder, kidney disease and endocrine defect and considered by the attending physician as at specific risk. METHODS: QUS outputs were calculated for each disorder and compared to: sex, age, Tanner stage, Z-score for height, weight and BMI (body mass index). RESULTS: One-hundred-sixty-eight subjects aged between 3.5 and 18 years met the inclusion criteria. The overall bone quality indexes were under the normal range in all the groups considered. Impairment of bone quality parameters was more evident in the group of patients with inherited metabolic disorders, in which 65% of patients in charge were studied by QUS. Older age and sexual development were associated with less pronounced bone quality impairment, as measured by QUS, in the vast majority of conditions. Overall, the diseases for which the prediction of outcome was the strongest were: hyperphenylalaninemia, nephrotic syndrome and insulin dependent diabetes mellitus. CONCLUSIONS: QUS is capable to provide information on skeletal status in children. Initial evaluation by QUS may allow defining patients with chronic disorders who deserve further, more invasive diagnostic studies. Inherited metabolic disorders warrant specific attention and strict monitoring for their potential effect on bone.
PURPOSE: To evaluate the use of QUS for the bone status assessment in children cared because of a chronic disease such as: inherited metabolic disorder, kidney disease and endocrine defect and considered by the attending physician as at specific risk. METHODS: QUS outputs were calculated for each disorder and compared to: sex, age, Tanner stage, Z-score for height, weight and BMI (body mass index). RESULTS: One-hundred-sixty-eight subjects aged between 3.5 and 18 years met the inclusion criteria. The overall bone quality indexes were under the normal range in all the groups considered. Impairment of bone quality parameters was more evident in the group of patients with inherited metabolic disorders, in which 65% of patients in charge were studied by QUS. Older age and sexual development were associated with less pronounced bone quality impairment, as measured by QUS, in the vast majority of conditions. Overall, the diseases for which the prediction of outcome was the strongest were: hyperphenylalaninemia, nephrotic syndrome and insulin dependent diabetes mellitus. CONCLUSIONS: QUS is capable to provide information on skeletal status in children. Initial evaluation by QUS may allow defining patients with chronic disorders who deserve further, more invasive diagnostic studies. Inherited metabolic disorders warrant specific attention and strict monitoring for their potential effect on bone.
Authors: Marc-Antoine Krieg; Reinhart Barkmann; Stefano Gonnelli; Alison Stewart; Douglas C Bauer; Luis Del Rio Barquero; Jonathan J Kaufman; Roman Lorenc; Paul D Miller; Wojciech P Olszynski; Catalina Poiana; Anne-Marie Schott; E Michael Lewiecki; Didier Hans Journal: J Clin Densitom Date: 2008 Jan-Mar Impact factor: 2.617
Authors: Giampiero I G L I Baroncelli; Giovanni Federico; Silvano Bertelloni; Federica Sodini; Francesca De Terlizzi; Ruggero Cadossi; Giuseppe Saggese Journal: Pediatr Res Date: 2003-04-16 Impact factor: 3.756
Authors: S Bertelloni; A Balsamo; L Giordani; R Fischetto; G Russo; M Delvecchio; M Gennari; A Nicoletti; M C Maggio; D Concolino; L Cavallo; A Cicognani; G Chiumello; O Hiort; G I Baroncelli; M F Faienza Journal: J Endocrinol Invest Date: 2009-05-12 Impact factor: 4.256