Clinical Guidelines for Management of Bone Health in Rett Syndrome Based on Expert Consensus and Available Evidence
| Autor: | Jenny Downs, Meir Lotan, Giangennaro Coppola, Gunilla Larsson, Suzanne Geerts, Bruria Ben-Zeev, Helen J Woodhead, Leanne M Ward, Jay R. Shapiro, Sue Fyfe, Batia Weiss, Torkel B. Brismar, Sue Thompson, Michael Freilinger, Kathleen J. Motil, Jane B. Lane, Daniel C. Tarquinio, Peter Humphreys, Alan K. Percy, Anne Marie Bisgaard, Amanda Jefferson, Mercedes Pineda, Carolyn Ellaway, Ingegerd Witt Engerström, Mary Jones, Helen Leonard, Steven A. Skinner, Craig F Munns, Birgit Syhler, Aris Siafarikas |
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| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
Genetics and Molecular Biology (all)
0301 basic medicine Critical Care and Emergency Medicine Bone density Osteoporosis Organic chemistry lcsh:Medicine Biochemistry Pediatrics Diagnostic Radiology Families 0302 clinical medicine Absorptiometry Photon Bone Density Medicine and Health Sciences Sjukgymnastik Disease management (health) Vitamin D Connective Tissue Diseases lcsh:Science Physiotherapy Children Trauma Medicine Multidisciplinary Bone Density Conservation Agents Diphosphonates Medicine (all) Radiology and Imaging Pediatrik Disease Management Vitamins Photon Bone Imaging 3. Good health Physical sciences Chemistry Connective Tissue Bone Fracture Meta-analysis Practice Guidelines as Topic Anatomy Traumatic Injury Research Article medicine.medical_specialty Consensus Imaging Techniques Expert Testimony Humans Rett Syndrome Agricultural and Biological Sciences (all) Biochemistry Genetics and Molecular Biology (all) MEDLINE Rett syndrome Research and Analysis Methods 03 medical and health sciences Chemical compounds Rheumatology Diagnostic Medicine Organic compounds medicine Absorptiometry Intensive care medicine Bone business.industry lcsh:R Biology and Life Sciences Bone fracture medicine.disease 030104 developmental biology Biological Tissue Age Groups People and Places Physical therapy Population Groupings lcsh:Q business 030217 neurology & neurosurgery |
| Zdroj: | PLoS ONE, Vol 11, Iss 2, p e0146824 (2016) PLoS One r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu instname PLoS ONE r-FSJD: Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu Fundació Sant Joan de Déu |
| ISSN: | 1932-6203 |
| Popis: | Objectives We developed clinical guidelines for the management of bone health in Rett syndrome through evidence review and the consensus of an expert panel of clinicians. Methods An initial guidelines draft was created which included statements based upon literature review and 11 open-ended questions where literature was lacking. The international expert panel reviewed the draft online using a 2-stage Delphi process to reach consensus agreement. Items describe the clinical assessment of bone health, bone mineral density assessment and technique, and pharmacological and non-pharmacological interventions. Results Agreement was reached on 39 statements which were formulated from 41 statements and 11 questions. When assessing bone health in Rett syndrome a comprehensive assessment of fracture history, mutation type, prescribed medication, pubertal development, mobility level, dietary intake and biochemical bone markers is recommended. A baseline densitometry assessment should be performed with accommodations made for size, with the frequency of surveillance determined according to individual risk. Lateral spine x-rays are also suggested. Increasing physical activity and initiating calcium and vitamin D supplementation when low are the first approaches to optimizing bone health in Rett syndrome. If individuals with Rett syndrome meet the ISCD criterion for osteoporosis in children, the use of bisphosphonates is recommended. Conclusion A clinically significant history of fracture in combination with low bone densitometry findings is necessary for a diagnosis of osteoporosis. These evidence and consensus-based guidelines have the potential to improve bone health in those with Rett syndrome, reduce the frequency of fractures, and stimulate further research that aims to ameliorate the impacts of this serious comorbidity. |
| Databáze: | OpenAIRE |
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