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["板层状颅骨":库利氏病的一种特殊放射学特征]

[The "lamellar cranium": a particular radiologic feature of Cooley's disease].

作者信息

Orzincolo C, Castaldi G, Scutellari P N, Fogli B, Bariani L, Atti G

机构信息

Servizio di Radiologia, Arcispedale S. Anna, Ferrara.

出版信息

Radiol Med. 1989 Apr;77(4):317-21.

PMID:2727334
Abstract

The skull in homozygous beta-thalassemia (Cooley's anemia) may present with several abnormalities, such as osteopenia, widening of the diploic space, and a "hair on end" appearance. In some cases it presents also a particular stratified aspect owing to a variable number of osseous lamellae, arranged so as to be parallel to the inner table. This "lamellated skull" was observed in 16 patients, selected from 150 patients with Cooley's anemia (10.6%). The possible mechanisms are discussed. The pluristratified lamellar osseous production could be brought about by repeated periosteal osteoblastic reaction to the sinusoidal neovascularization associated with marrow hyperplasia in poorly or not at all transfused patients.

摘要

纯合子β地中海贫血(库利贫血)患者的颅骨可能出现多种异常,如骨质减少、板障间隙增宽和“头发直立”外观。在某些情况下,由于数量不等的骨板排列成与内板平行,颅骨还呈现出一种特殊的分层外观。从150例库利贫血患者中选取了16例(10.6%)观察到这种“分层颅骨”。本文讨论了可能的机制。多层板层状骨的形成可能是由于未输血或极少输血患者的骨髓增生伴发的窦状隙新生血管反复刺激骨膜成骨细胞反应所致。

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