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短指畸形C型中的天使状指骨:一例报告及发病机制推测

Angel-shaped phalanges in brachydactyly C: a case report, and speculation on pathogenesis.

作者信息

Castriota-Scanderbeg Alessandro, Garaci Francesco Giuseppe, Beluffi Giampiero

机构信息

Department of Radiology, Foundation Ospedale Card. G. Panico, via S. Pio X, 4, Tricase (Le), Italy.

出版信息

Pediatr Radiol. 2005 May;35(5):535-8. doi: 10.1007/s00247-004-1362-x. Epub 2004 Nov 23.

Abstract

We describe a woman and her daughter affected by brachydactyly type C. The unusual feature in the child included the striking 'angel-shaped' appearance of the proximal phalanges of the index and middle fingers of one hand, whereas more typical triangular epiphyses with elongation of their radial side were present at the same location in the opposite hand. It is suggested that this peculiar phalangeal configuration occurs as a transitory event in early or mid childhood in phalanges that are marked by severe ossification delay, which is most prominent at the level of the primary ossification centre.

摘要

我们描述了一位患有C型短指症的女性及其女儿。该患儿的不同寻常之处在于,一只手的食指和中指近节指骨呈现出明显的“天使形状”外观,而另一只手同一位置则出现了更典型的桡侧延长的三角形骨骺。有人认为,这种特殊的指骨形态是在儿童早期或中期,在严重骨化延迟的指骨中作为一种过渡性现象出现的,这种延迟在初级骨化中心水平最为明显。

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