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    Clinical guidelines for management of bone health in Rett syndrome based on expert consensus and available evidence

    238982_238982.PDF (277.3Kb)
    Access Status
    Open access
    Authors
    Jefferson, A.
    Leonard, H.
    Siafarikas, A.
    Woodhead, H.
    Fyfe, S.
    Ward, L.
    Munns, C.
    Motil, K.
    Tarquinio, D.
    Shapiro, J.
    Brismar, T.
    Ben-Zeev, B.
    Bisgaard, A.
    Coppola, G.
    Ellaway, C.
    Freilinger, M.
    Geerts, S.
    Humphreys, P.
    Jones, M.
    Lane, J.
    Larsson, G.
    Lotan, M.
    Percy, A.
    Pineda, M.
    Skinner, S.
    Syhler, B.
    Thompson, S.
    Weiss, B.
    Engerström, I.
    Downs, Jennepher
    Date
    2016
    Type
    Journal Article
    
    Metadata
    Show full item record
    Citation
    Jefferson, A. and Leonard, H. and Siafarikas, A. and Woodhead, H. and Fyfe, S. and Ward, L. and Munns, C. et al. 2016. Clinical guidelines for management of bone health in Rett syndrome based on expert consensus and available evidence. PLoS ONE. 11 (2): e0146824.
    Source Title
    Microstructural and Compositional Features of the Fibrous and Hyaline Cartilage on the Medial Tibial Plateau Imply a Unique Role for the Hopping Locomotion of Kangaroo
    DOI
    10.1371/journal.pone.0146824
    ISSN
    1932-6203
    School
    School of Physiotherapy and Exercise Science
    Funding and Sponsorship
    http://purl.org/au-research/grants/nhmrc/1004384
    Remarks

    This open access article is distributed under the Creative Commons license http://creativecommons.org/licenses/by/4.0/

    Data available at http://doi.org/10.4225/06/5697464CB9EE4

    URI
    http://hdl.handle.net/20.500.11937/44685
    Collection
    • Curtin Research Publications
    Abstract

    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.

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