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科威特镰状细胞病患儿股骨头缺血性坏死的MRI随访及自然病程

MRI follow-up and natural history of avascular necrosis of the femoral head in Kuwaiti children with sickle cell disease.

作者信息

Gupta Renu, Adekile Adekunle D

机构信息

Departments of Radiology and Paediatrics, Faculty of Medicine, Kuwait University, Safat, Kuwait.

出版信息

J Pediatr Hematol Oncol. 2004 Jun;26(6):351-3. doi: 10.1097/00043426-200406000-00004.

Abstract

PURPOSE

: To document the MRI progression and the natural history of avascular necrosis of the femoral head (AVNFH) in Arab children with sickle cell disease.

PATIENTS AND METHODS

Twenty-three SS and 7 SbetaThal patients (aged 6-17 years) were screened for AVNFH between 1998 and 1999. Eight (26.7%) were identified with varying degrees of AVNFH. Seventeen of the original 30 patients have now been followed for 1 to 4 (mean 2.0 +/- 1.2) years, with repeat MRI of the hips. Spin-echo T1-and T2-weighted images and T2 fat-saturation sequences were obtained using a 1.5-Tesla GE unit with superconductors. AVNFH was graded I (mild), II (moderate), or III (severe).

RESULTS

Eleven (64.7%) of the 17 patients had significant progression of their lesions; at the initial study, 9 were normal, 7 were grade I, 1 was grade II, and none was grade III. At the end of the follow-up period, two were normal, seven were grade I, one was grade II, and seven were grade III. Of the nine who were initially normal, two still had no lesions, while four were grade I and three were grade III on follow-up. Of the seven who were classified as grade I initially, four remained at grade I, one moved to grade II, and two became grade III. The one patient who was initially grade II progressed to III.

CONCLUSIONS

AVNFH is a common, chronic, and unrelenting complication in children with sickle cell disease, and it is usually progressive.

摘要

目的

记录患有镰状细胞病的阿拉伯儿童股骨头缺血性坏死(AVNFH)的MRI进展情况及自然病史。

患者与方法

1998年至1999年间,对23例SS型和7例Sβ地中海贫血患者(年龄6 - 17岁)进行了AVNFH筛查。其中8例(26.7%)被确诊患有不同程度的AVNFH。最初的30例患者中有17例现已接受了1至4年(平均2.0±1.2年)的随访,并对髋关节进行了重复MRI检查。使用配备超导磁体的1.5特斯拉GE设备获取自旋回波T1加权、T2加权图像以及T2脂肪抑制序列图像。AVNFH被分为I级(轻度)、II级(中度)或III级(重度)。

结果

17例患者中有11例(64.7%)病情出现显著进展;在初始研究时,9例正常,7例为I级,1例为II级,无III级患者。随访期末,2例正常,7例为I级,1例为II级,7例为III级。最初正常的9例患者中,2例仍无病变,4例为I级,3例在随访时为III级。最初分类为I级的7例患者中,4例仍为I级,1例进展为II级,2例变为III级。最初为II级的1例患者进展为III级。

结论

AVNFH是患有镰状细胞病儿童常见、慢性且持续存在的并发症,通常呈进行性发展。

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