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黏多糖贮积症I型患者的全髋关节置换术——8年随访——病例报告并文献复习

Total hip arthroplasty in Hurler syndrome - 8 years follow up - A case report with review of literature.

作者信息

Gautam Deepak, Malhotra Rajesh

机构信息

Department of Orthopedics, All India Institute of Medical Sciences (AIIMS), New Delhi 110029, India.

出版信息

J Orthop. 2018 Feb 2;15(1):111-113. doi: 10.1016/j.jor.2018.01.036. eCollection 2018 Mar.

Abstract

Life expectancy in Hurler syndrome is significantly improved by enzyme therapy with bone marrow transplantation. However, the deterioration of skeletal abnormalities persists. Hip dysplasia is a common presentation which may progress to significant hip arthritis requiring total hip arthroplasty at later stage. We report a long-term outcome of cementless total hip arthroplasty in a patient with Hurler syndrome who was successfully treated with bone marrow transplant.

摘要

通过骨髓移植进行酶替代疗法可显著提高黏多糖贮积症I型(Hurler综合征)患者的预期寿命。然而,骨骼异常仍会持续恶化。髋关节发育不良是常见表现,可能会发展为严重的髋关节炎,后期需要进行全髋关节置换术。我们报告了1例接受骨髓移植成功治疗的Hurler综合征患者接受非骨水泥型全髋关节置换术的长期疗效。

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本文引用的文献

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Total Hip Arthroplasty in Mucopolysaccharidosis Type IH.黏多糖贮积症 I 型中的全髋关节置换术
Case Rep Orthop. 2011;2011:832439. doi: 10.1155/2011/832439. Epub 2012 Jan 26.
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Relative frequency of the Hurler and Hunter syndromes.
N Engl J Med. 1971 Jan 28;284(4):221-2. doi: 10.1056/NEJM197101282840425.

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