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Type: Journal article
Title: The mucopolysaccharidoses: advances in medical care lead to challenges in orthopaedic surgical care
Author: Williams, N.
Challoumas, D.
Ketteridge, D.
Cundy, P.
Eastwood, D.
Citation: The Bone & Joint Journal, 2017; 99B(9):1132-1139
Publisher: British Editorial Society of Bone and Joint Surgery
Issue Date: 2017
ISSN: 2049-4408
Statement of
N. Williams, D. Challoumas, D. Ketteridge, P. J. Cundy, D. M. Eastwood
Abstract: The mucopolysaccharidoses (MPS) are a group of inherited lysosomal storage disorders with clinical manifestations relevant to the orthopaedic surgeon. Our aim was to review the recent advances in their management and the implications for surgical practice. The current literature about MPSs is summarised, emphasising orthopaedic complications and their management. Recent advances in the diagnosis and management of MPSs include the recognition of slowly progressive, late presenting subtypes, developments in life-prolonging systemic treatment and potentially new indications for surgical treatment. The outcomes of surgery in these patients are not yet validated and some procedures have a high rate of complications which differ from those in patients who do not have a MPS. The diagnosis of a MPS should be considered in adolescents or young adults with a previously unrecognised dysplasia of the hip. Surgeons treating patients with a MPS should report their experience and studies should include the assessment of function and quality of life to guide treatment.
Keywords: Mucopolysaccharidoses
Rights: ©2017 The British Editorial Society of Bone & Joint Surgery
DOI: 10.1302/0301-620X.99B9.BJJ-2017-0487
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Appears in Collections:Aurora harvest 3
Orthopaedics and Trauma publications

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