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[遗传性神经病中的溃疡型致残性肢端骨病。鉴别诊断与发病机制]

[Ulcero-mutilating acro-osteopathy in hereditary neuropathies. Differential diagnosis and pathogenesis].

作者信息

Garbe C, Vogel H P, Tebbe B, Nüssel F, Marx P, Orfanos C E

机构信息

Hautklinik und Poliklinik, Klinikum Steglitz der Freien Universität Berlin.

出版信息

Dtsch Med Wochenschr. 1989 Apr 21;114(16):628-32. doi: 10.1055/s-2008-1066647.

Abstract

Two patients, aged 51 and 70 years, had indolent ulcerations at the sole of the foot with destructive osteolysis in the bones of the feet, lesions characteristic of mutilating ulcerative acro-osteopathy. Patient 1, who had pes cavus, developed ulcerations on the balls of the feet along the second metatarsal bones, and patient 2 with pes equinovarus developed ulcers in the area of the calcaneus. Sock-like hypesthesia/hypalgesia from the toes to the ankles was present in both patients, and electrophysiological tests confirmed the presence of axonal sensory-motor neuropathy. Diabetes mellitus and alcohol abuse was excluded in both patient. Clinical findings, history and neurological disturbances in both patients identified the disease as hereditary sensory neuropathy (type I).

摘要

两名患者,年龄分别为51岁和70岁,足底有惰性溃疡,足部骨骼出现破坏性骨质溶解,这些病变是致残性溃疡性肢端骨病的特征。患者1有高弓足,在第二跖骨沿足趾球部出现溃疡,患者2有马蹄内翻足,在跟骨区域出现溃疡。两名患者均存在从脚趾到脚踝的袜状感觉减退/痛觉减退,电生理检查证实存在轴索性感觉运动神经病变。两名患者均排除了糖尿病和酒精滥用。两名患者的临床表现、病史和神经功能障碍均将该病诊断为遗传性感觉神经病(I型)。

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