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镰状细胞贫血患者颅骨板障的分层现象。

Lamellation of the diploe in the skulls of patients with sickle cell anaemia.

作者信息

Williams A O, Lagundoye S B, Johnson C L

出版信息

Arch Dis Child. 1975 Dec;50(12):948-52. doi: 10.1136/adc.50.12.948.

Abstract

This study describes the radiological and pathological findings in a necropsy series of 70 skulls of Nigerian patients with sickle cell gene, and 70 controls matched for sex and age with no sickle cell gene. 6 (35%) of the 17 patients with sickle anaemia, or 17% of 35 patients with sickle cell gene excluding the trait, all under age 10 years, were shown to have bone trabeculae within the diploe arranged in stripes parallel to the curvature of the cranial tables on radiography. Histological examination of the skulls with curvilinear stripes showed long trabeculae of bone within the diploe similarly arranged in parallel rows but joint at variable intervals by short bridges of bone. The radiolucent areas between trabeculae corresponded to areas of marrow hyperplasia. A skull radiograph in an African child presenting with this radiological sign should raise the suspicion of sickel cell disease.

摘要

本研究描述了对70例携带镰状细胞基因的尼日利亚患者及70例年龄和性别匹配的无镰状细胞基因对照者进行尸检时的影像学和病理学发现。17例镰状细胞贫血患者中有6例(35%),或35例携带镰状细胞基因(不包括性状)且年龄均在10岁以下的患者中有17%,在X线片上显示板障内骨小梁呈与颅板曲率平行的条纹状排列。对具有曲线状条纹的颅骨进行组织学检查发现,板障内长骨小梁同样平行排列,但由短骨桥以不同间隔相连。小梁之间的透光区对应于骨髓增生区域。出现这种影像学征象的非洲儿童的颅骨X线片应引起对镰状细胞病的怀疑。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c1c2/1545834/682404a13743/archdisch00834-0045-a.jpg

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