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褐黄病的骨科表现:病理生理学、临床表现、诊断与治疗

Orthopedic Manifestations of Ochronosis: Pathophysiology, Presentation, Diagnosis, and Management.

作者信息

Gil Joseph A, Wawrzynski Joseph, Waryasz Gregory R

机构信息

Department of Orthopedic Surgery, Warren Alpert Medical School, Brown University, Providence, RI.

Warren Alpert Medical School, Brown University, Providence, RI.

出版信息

Am J Med. 2016 May;129(5):536.e1-6. doi: 10.1016/j.amjmed.2016.01.010. Epub 2016 Feb 1.

DOI:10.1016/j.amjmed.2016.01.010
PMID:26844634
Abstract

Ochronotic arthropathy occurs in patients with alkaptonuria, manifesting first in the intervertebral discs of the lumbar spine, with subsequent degeneration most often observed in the knee, hip, and shoulder joints. Efforts at treatment are targeted at minimizing the damaging effects of the underlying metabolic disorder on the articular cartilage. Vitamin E and N-acetyl cysteine are potential therapies because of their scavenging of free radicals and consequent limitation of oxidative damage to joint tissue. Arthroscopy has been found to be an effective diagnostic tool in cases of suspected ochronosis. Arthroplasty performed in patients with ochronotic arthropathy suggests that the procedure is effective in the alleviation of joint pain and the improvement of mobility. Perioperative management of these patients may require more careful consideration pertinent to the associated comorbidities of this disorder.

摘要

褐黄病性关节病发生于患尿黑酸尿症的患者,首先出现在腰椎间盘,随后最常于膝关节、髋关节和肩关节出现退变。治疗的努力方向是尽量减少潜在代谢紊乱对关节软骨的损害作用。维生素E和N-乙酰半胱氨酸因其清除自由基及随之限制对关节组织的氧化损伤而成为潜在的治疗方法。关节镜检查已被发现是疑似褐黄病病例的有效诊断工具。对褐黄病性关节病患者实施关节成形术表明该手术在缓解关节疼痛和改善活动能力方面有效。这些患者的围手术期管理可能需要更仔细地考虑与该疾病相关的合并症。

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