血友病患者肌肉骨骼并发症的处理:文献回顾和专家建议。

Q2 Medicine
E Carlos Rodriguez-Merchan
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引用次数: 2

摘要

在血友病患者中,如果缺陷因子的浓度降到正常水平的1%以下,在儿童和青少年时期进行血液学预防可以避免肌肉骨骼并发症(关节和肌肉)的发生。由于青少年骨骼对疾病的不良事件非常敏感,因此及时处理至关重要;剧烈的结构缺陷可能迅速出现。重要的是,关节出血和关节滑膜的根深蒂固的肥大必须大力治疗,以防止关节变性(血友病关节病)。在极端的关节疾病是在地方与强烈的痛苦,目标必须是重建活动,同时减少对病人的危险。关节镜下关节清创是实现这一目标的一种有效的手术技术,特别是在膝关节或踝关节周围,并且可能被考虑作为踝关节融合术或踝关节或膝关节置换术的后援。最终,关节置换通常可以重建不健康关节的关节活动和功能。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Management of Musculoskeletal Complications in Patients with Hemophilia: Literature Review and Expert Recommendations.

In people with hemophilia, hematological prophylaxis during childhood and adolescence could elude the occurrence of musculoskeletal complications (in joints and muscles) if the concentration of the defective factor is averted from decreasing under 1% of normal. Prompt management is of capital significance as the juvenile skeleton is hypersensitive to the adverse events of the disease; intense structural defects might appear rapidly. Important, articular bleeds and inveterate hypertrophy of the articular synovial membrane must be treated vigorously to preclude joint degeneration (hemophilic arthropathy). At the moment that extreme joint disease is in place with intense affliction, the goal must be to reestablish activity whilst at the same time reducing the peril to the patient. Arthroscopic articular debridement is an efficacious surgical technique to accomplish this goal, particularly around the knee or ankle, and maybe contemplated to be a backup to ankle arthrodesis or ankle or knee replacement in patients of younger age. Eventually, joint replacement can commonly reestablish both articular mobility and function in an unhealthy articulation.

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来源期刊
Cardiovascular and Hematological Disorders - Drug Targets
Cardiovascular and Hematological Disorders - Drug Targets Medicine-Cardiology and Cardiovascular Medicine
CiteScore
1.90
自引率
0.00%
发文量
36
期刊介绍: Cardiovascular & Hematological Disorders - Drug Targets aims to cover all the latest and outstanding developments on the medicinal chemistry, pharmacology, molecular biology, genomics and biochemistry of contemporary molecular targets involved in cardiovascular and hematological disorders e.g. disease specific proteins, receptors, enzymes, genes. Each issue of the journal contains a series of timely in-depth reviews written by leaders in the field covering a range of current topics on drug targets involved in cardiovascular and hematological disorders. As the discovery, identification, characterization and validation of novel human drug targets for cardiovascular and hematological drug discovery continues to grow.
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