与成人镰状细胞病相关的距骨骨坏死：从早期到晚期的自然演变
Talar Osteonecrosis Related to Adult Sickle Cell Disease: Natural Evolution from Early to Late Stages.
作者信息
Hernigou Philippe, Flouzat-Lachaniette Charles Henri, Daltro Gildasio, Galacteros Frederic
机构信息
Hôpital Henri Mondor, Creteil, France
Hôpital Henri Mondor, Creteil, France.
出版信息
J Bone Joint Surg Am. 2016 Jul 6;98(13):1113-21. doi: 10.2106/JBJS.15.01074.
BACKGROUND
Little is known about the rate of, and factors affecting, progression of talar osteonecrosis related to sickle cell disease. Adult patients with sickle cell disease who presented with hip osteonecrosis were evaluated for talar osteonecrosis with radiographs and magnetic resonance imaging (MRI). Forty-five of them (75 tali) were diagnosed with talar osteonecrosis, and this group was evaluated for factors influencing the progression of the disease.
METHODS
Forty-five patients with sickle cell disease and osteonecrosis of the talus were identified with radiographs and MRI between 1985 and 1995. Seven of these patients were homozygous for hemoglobin S (S/S genotype), 26 had hemoglobin S/hemoglobin C, and 12 had hemoglobin S/beta-thalassemia. The talar osteonecrosis was graded with radiographs and MRI. The patients were followed with clinical examination and radiographs every 6 months until talar collapse and every year after the collapse.
RESULTS
The osteonecrosis was unilateral in 15 patients and bilateral in 30 at the time of the initial examination. Forty-five ankles were asymptomatic and 30 were symptomatic at the initial evaluation. MRI performed at the time of the most recent follow-up, and compared with MRI performed at diagnosis, did not show partial or total regression of the osteonecrosis in any of the patients, even those with asymptomatic stage-I osteonecrosis. At the time of the most recent follow-up (mean, 20 years; range, 15 to 25 years), pain and collapse had developed in all except 12 ankles. The stage of the osteonecrosis at the initial visit, pain, the genotype of the sickle cell disease, and the extent and location of the lesion in the talus were risk factors for progression of the disease.
CONCLUSIONS
In the majority of the patients with sickle cell disease, osteonecrosis of the talus should be expected to show relevant clinical and radiographic evidence of progression over a long period.
LEVEL OF EVIDENCE
Prospective Level II. See Instructions for Authors for a complete description of levels of evidence.
背景
关于镰状细胞病相关距骨骨坏死的发生率及影响其进展的因素，目前所知甚少。对出现髋部骨坏死的成年镰状细胞病患者，采用X线片和磁共振成像（MRI）对距骨骨坏死进行评估。其中45例（75个距骨）被诊断为距骨骨坏死，并对该组患者影响疾病进展的因素进行评估。
方法
1985年至1995年间，通过X线片和MRI确定了45例患有镰状细胞病和距骨骨坏死的患者。其中7例为血红蛋白S纯合子（S/S基因型），26例为血红蛋白S/血红蛋白C，12例为血红蛋白S/β地中海贫血。根据X线片和MRI对距骨骨坏死进行分级。每6个月对患者进行临床检查和X线片检查，直至距骨塌陷，塌陷后每年检查一次。
结果
初次检查时，15例患者的骨坏死为单侧，30例为双侧。初次评估时，45个踝关节无症状，30个有症状。在最近一次随访时进行的MRI检查与诊断时的MRI检查相比，即使是无症状的I期骨坏死患者，也未显示任何患者的骨坏死有部分或完全消退。在最近一次随访时（平均20年；范围15至25年），除12个踝关节外，所有踝关节均出现疼痛和塌陷。初次就诊时骨坏死的分期、疼痛、镰状细胞病的基因型以及距骨病变的范围和位置是疾病进展的危险因素。
结论
在大多数镰状细胞病患者中，预计距骨骨坏死会在很长一段时间内出现相关的临床和影像学进展证据。
证据水平
前瞻性II级。有关证据水平的完整描述，请参阅作者指南。
Zotero 插件