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[成人镰状细胞贫血的管理]

[Management of adults with sickle cell anemia].

作者信息

Bachir D

机构信息

Centre de la Drépanocytose, CHU Henri Mondor, Créteil, France.

出版信息

Pathol Biol (Paris). 1999 Jan;47(1):39-45.

Abstract

The management of adults with sickle cell disease should be geared to the profile of the disease in adulthood. The chronic hemolytic anemia impacts everyday activities. Paroxysmal complications include painful vasoocclusive crises, acute chest syndrome, priapism, and infections. Potentially life-threatening chronic complications should be detected and treated early; they include cardiopulmonary, renal, and hepatic involvement. Osteonecrosis of the hip can result in functional impairment. Pregnancy and anesthesia require special precautions. A multifaceted personalized management program, if possible at a sickle cell disease center working closely with other health care providers and social workers, offers the best hope for providing ever-increasing gains in quality of life for sickle cell disease patients.

摘要

成年镰状细胞病患者的管理应根据该疾病在成年期的特征进行。慢性溶血性贫血会影响日常活动。阵发性并发症包括疼痛性血管闭塞危象、急性胸部综合征、阴茎异常勃起和感染。应尽早发现并治疗潜在的危及生命的慢性并发症;这些并发症包括心肺、肾脏和肝脏受累。髋部骨坏死可导致功能障碍。怀孕和麻醉需要特别预防措施。一个多方面的个性化管理方案,若能在镰状细胞病中心实施,并与其他医疗保健提供者和社会工作者密切合作,为提高镰状细胞病患者的生活质量带来不断增加的收益提供了最大希望。

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