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什么是戈林综合征?基于对37例患者的研究对基底细胞痣综合征的诊断与管理。

What is Gorlin's syndrome? The diagnosis and management of the basal cell naevus syndrome, based on a study of thirty-seven patients.

作者信息

Rayner C R, Towers J F, Wilson J S

出版信息

Br J Plast Surg. 1977 Jan;30(1):62-7. doi: 10.1016/s0007-1226(77)90037-6.

Abstract

A series of 37 patients with Gorlin's syndrome is described; 23 had the typical naevi, 19 had jaw cysts and only 10 had both. A wide range of associated deformities was noted. The need for early identification of patients at risk is stressed; follow-up is essential for 2 reasons: The naevi although indistinguishable histologically from basal cell carcinomas only rarely require radical removal. The jaw cysts are locally invasive and should be radically removed when small. In 8 patients examined there was a low level of alpha-globulins in the plasma. Spontaneous involution of the naevi may occur in un-irradiated skin.

摘要

本文描述了37例戈林综合征患者;23例有典型痣,19例有颌骨囊肿,仅有10例两者皆有。记录了一系列相关畸形。强调了早期识别高危患者的必要性;随访至关重要,原因有二:这些痣虽然在组织学上与基底细胞癌难以区分,但很少需要根治性切除。颌骨囊肿具有局部侵袭性,小的时候就应根治性切除。在8例接受检查的患者中,血浆中α球蛋白水平较低。痣可能在未受照射的皮肤中自发消退。

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