Huo M H, Friedlaender G E, Marsh J S
Dept. of Orthopaedics and Rehabilitation, Yale University School of Medicine, New Haven, CT 06510.
Yale J Biol Med. 1990 May-Jun;63(3):195-207.
Sickle-cell disease is a well-recognized clinical entity. The pathophysiology of this hemoglobinopathy has been described in detail by numerous investigators since the first case report appeared in 1910. Orthopaedic manifestations of sickle-cell disease account for much of the morbidity associated with this disorder, including pain, osteonecrosis, arthritis, and sepsis. Effective management of these bone and joint sequelae reflect accurate diagnosis, understanding of this disorder's pathophysiology, and knowledge of available medical and surgical treatment alternatives. In this review, the authors summarize the major orthopaedic manifestations of sickle-cell disease with special emphasis placed upon osteonecrosis and osteomyelitis, since these conditions are the most disabling and serious complications in patients with sickle-cell disease.
镰状细胞病是一种广为人知的临床病症。自1910年首例病例报告出现以来,众多研究者已详细描述了这种血红蛋白病的病理生理学。镰状细胞病的骨科表现是该疾病相关发病的主要原因,包括疼痛、骨坏死、关节炎和败血症。对这些骨与关节后遗症的有效管理反映出准确的诊断、对该病症病理生理学的理解以及对可用的药物和手术治疗选择的了解。在本综述中,作者总结了镰状细胞病的主要骨科表现,特别强调了骨坏死和骨髓炎,因为这些病症是镰状细胞病患者中最致残且最严重的并发症。