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纯合子镰状细胞病患者股骨头缺血性坏死的放射学特征。

The radiological features of avascular necrosis of the femoral head in homozygous sickle cell disease.

作者信息

Lee R E, Golding J S, Serjeant G R

出版信息

Clin Radiol. 1981 Mar;32(2):205-14. doi: 10.1016/s0009-9260(81)80162-6.

DOI:10.1016/s0009-9260(81)80162-6
PMID:7214838
Abstract

Out of a total of 1031 patients with homozygous sickle cell (SS) disease attending a sickle cell clinic in Jamaica, 52 patients were found to have changes of avascular necrosis of the femoral head in 76 hips. Hip involvement was unilateral in 28 patients (31 males and 21 females). The age of onset varied from nine to 45 years in the 40 patients in whom data appeared reasonably reliable and 82% developed this lesion between 10 and 29 years. Radiological change occurred, by definition, in the femoral head in all hips, in the acetabulum in 55 (72%) hips, femoral head migration occurred in 37 (49%) hips, and periosteal new bone formation in 24 (32%) hips. Two broad patterns of involvement were discernible according to the age at which avascular necrosis had occurred. Involvement of the immature femoral head epiphysis resulted in a flattened femoral head, epiphysio-metaphyseal overlap, a wide femoral neck and a mushroom deformity of the mature femoral head. In these lesions the articular surface and joint space was generally well preserved and little or no disability resulted in adult patients. Involvement of the mature femoral head was typical segmental, most commonly antero-superior, and with continued weight bearing resulted in collapse of the head and disruption of the articular surface. These lesions caused pain, limitation of movement, and commonly changes of osteo-arthrosis.

摘要

在牙买加一家镰状细胞诊所就诊的1031例纯合子镰状细胞(SS)病患者中,发现52例患者的76个髋关节出现了股骨头缺血性坏死的变化。28例患者(31名男性和21名女性)的髋关节受累为单侧。在40例数据似乎相当可靠的患者中,发病年龄从9岁到45岁不等,82%的患者在10岁至29岁之间出现了这种病变。根据定义,所有髋关节的股骨头均出现了放射学改变,55个(72%)髋关节的髋臼出现改变,37个(49%)髋关节出现股骨头移位,24个(32%)髋关节出现骨膜新生骨形成。根据缺血性坏死发生的年龄,可以辨别出两种广泛的受累模式。未成熟股骨头骨骺受累导致股骨头扁平、骨骺-干骺端重叠、股骨颈变宽以及成熟股骨头呈蘑菇状畸形。在这些病变中,关节面和关节间隙通常保存良好,成年患者很少或没有残疾。成熟股骨头受累典型为节段性,最常见于前上方,持续负重会导致股骨头塌陷和关节面破坏。这些病变会引起疼痛、活动受限,并且通常会出现骨关节炎的改变。

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引用本文的文献

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Radiological manifestation of avascular necrosis (AVN) in sickle cell disease (SCD): a review of diagnostic imaging.镰状细胞病(SCD)中缺血性坏死(AVN)的放射学表现:诊断影像学综述。
Acta Biomed. 2023 Jun 14;94(3):e2023177. doi: 10.23750/abm.v94i3.14714.
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Sickle cell disease: new opportunities and challenges in Africa.镰状细胞病:非洲的新机遇与挑战
ScientificWorldJournal. 2013;2013:193252. doi: 10.1155/2013/193252. Epub 2013 Sep 19.
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Beyond the definitions of the phenotypic complications of sickle cell disease: an update on management.
超越镰状细胞病表型并发症的定义:管理更新
ScientificWorldJournal. 2012;2012:949535. doi: 10.1100/2012/949535. Epub 2012 Aug 1.
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Aseptic necrosis of femoral head complicating thalassemia.地中海贫血并发股骨头无菌性坏死。
Skeletal Radiol. 1986;15(7):541-4. doi: 10.1007/BF00361051.