Uncoupling of Bone Turnover may Compromise Skeletal Health of Young Patients With Haemophilia A |
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| Institution: | 1. Haemophilia Centre/Haemostasis and Thrombosis Unit, Aghia Sophia Children''s Hospital, Athens, Greece;2. Second Radiology Department, “Attikon” University Hospital, Athens, Greece;3. Department of Bone and Mineral Metabolism, Institute of Child Health, Αthens, Greece;4. First Paediatric Department – Athens Medical School, Aghia Sophia Children''s Hospital, Athens- Greece;1. School of Medicine, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, United Kingdom;2. Department of Paediatric Endocrinology, Royal Hospital for Children, Glasgow, United Kingdom;3. Human Nutrition, School of Medicine, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow Royal Infirmary, Glasgow, United Kingdom;1. Department of Nutrition, Federal University of Ouro Preto, Ouro Preto, Brazil;2. PG Fisiopatologia Medica, Universidade Estadual de Campinas, Campinas, Brazil;3. Department of Clinical Medicine, Medical School, Federal University of Minas Gerais, Belo Horizonte, Brazil;1. Department of Radiology, Beijing Jishuitan Hospital, Beijing, China;2. Department of Radiology, First Affiliated Hospital, University of Science and Technology of China, Hefei, China;3. Department of Radiology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China;4. Department of Radiology, Sichuan University West China Hospital, Chengdu, China;5. Centre For Medical Device Evaluation, NMPA, Beijing,China;6. Department of Sport and Exercise Sciences, Durham University, Durham, United Kingdom;7. School of Biomedical Engineering and Imaging Sciences, King''s College London, St Thomas’ Hospital, London, United Kingdom;1. Department of Medical and Surgical Science, University Magna Grecia, Catanzaro, Italy.;2. Department of Health Science, University Magna Grecia, Catanzaro, Italy.;3. Institute \"Beato Matteo\" (Hospital Group San Donato), Vigevano, Italy.;4. Department of Biomedical Science for Health, University of Milan, Milano, Italy.;5. Department of Molecular and Clinical Medicine, Sahlgrenska Center for Cardiovascolar and Metabolic Research, University of Gothenburg, Göteborg, Sweden.;6. Research Center for the Prevention and Treatment of Metabolic Diseases (CR METDIS), University Magna Grecia, Catanzaro, Italy.;7. Department of Clinical and Experimental Medicine, Magna Græcia University of Catanzaro, Catanzaro, Italy.;1. Department of Physical and Rehabilitation Medicine, La Paz University Hospital-IdiPaz, Madrid, Spain;2. Department of Orthopaedic Surgery, La Paz University Hospital-IdiPaz, Madrid, Spain;3. Department of Haematology, La Paz University Hospital-IdiPaz, Madrid, Spain |
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| Abstract: | There is evidence that bone mass is decreased and bone metabolism is dysregulated in children with haemophilia (CWH). The objective of this study was to investigate the impact of haemophilia on skeletal health in children, with regards to bone mineral density (BMD) and metabolic bone profile. This study included 51 male CWH A. Dual-energy X-ray absorptiometry (DXA) was performed to assess BMD in lumbar spine (LS) and total body less head (TBLH) and Z-scores were calculated (low BMD Z-score<-2, low-normal BMD Z-score between -1 and -2). Serum levels of osteocalcin (OC), procollagen type I C-terminal propeptide (PICP), bone alkaline phosphatase (bALP), bone tartrate-resistant acid phosphatase 5b (TRAP5b), vitamin D, parathormone (PTH), urinary calcium/creatinine (uCa/uCr) and urine deoxypyridinoline/creatinine (uDPD/uCr) were measured. Mean BMD Z-scores were lower than predicted at both sites of measurement. More specifically, 10% of CWH A had low and 20% low-normal BMD Z-scores in LS, whereas 9.1% had low-normal TBLH BMD Z-scores and there were no patients with low BMD Z-scores at this site of measurement. 36.7% of CWH had low vitamin D levels and 19.6% had a history of fracture. Also, patients with haemophilia had lower OC and higher uDPD/uCr levels while OC positively correlated to BMD Z-scores and uDPD/uCr negatively correlated to BMD Z-scores at both sites. No statistically significant differences were observed with regards to mode of treatment, number of haemorrhages and the presence of target-joints. CWH A had decreased BMD Z-scores at both sites with an uncoupling of bone turnover LS BMD seemed to be more affected than TBLH BMD. |
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