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Bone Joint J. 2017 Sep;99-B(9):1132-1139. doi: 10.1302/0301-620X.99B9.BJJ-2017-0487.

The mucopolysaccharidoses: advances in medical care lead to challenges in orthopaedic surgical care.

Author information

1
University of Adelaide, 72 King William St, North Adelaide, Australia.
2
Cambridge University Hospitals, Hills Road, Cambridge, CB2 0QQ, UK.
3
Great Ormond St Hospital for Children, London, WC1N 3JH, UK.

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. Cite this article: Bone Joint J 2017;99-B:1132-9.

KEYWORDS:

Mucopolysaccharidoses; Orthopaedic surgery; Outcomes

[Indexed for MEDLINE]

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