Gautam Deepak, Malhotra Rajesh
Department of Orthopedics, All India Institute of Medical Sciences (AIIMS), New Delhi 110029, India.
J Orthop. 2018 Feb 2;15(1):111-113. doi: 10.1016/j.jor.2018.01.036. eCollection 2018 Mar.
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.
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。