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儿童期神经纤维瘤病(雷克林霍增氏病)的临床、组织学及电子显微镜检查结果(作者译)

[Clinical, histological and electron microscopic findings in neurofibromatosis (Recklinghausen's disease) occurring in childhood (author's transl)].

作者信息

Watzek G, Matras H, Lassmann G, Lassmann H

出版信息

Wien Klin Wochenschr. 1977 Mar 18;89(6):193-8.

PMID:403684
Abstract

A follow-up study was carried out of the clinical symptoms and the radiological and histological alterations in five cases of neurofibromatosis (Recklinghausen's disease) with onset during early childhood. The clinical manifestation of the disease was predominantly restricted to the face. In the histological ultrastructural investigation different types of neurofibroma were detected (plexiform neurofibroma, argyrophilic neurofibroma and diffuse neurofibrma). Preliminary results are presented on the basis of a correlation between the clinical findings and the histological types of neurofibroma with special reference to the demarcation of the tumours, recurrence following surgical removal and the prognosis as to the clinical course of the disease. Nevertheless, definite conclusions will be possible only by continuous observation of a larger number of patients by means of standardized clinical, radiological and histological methods of investigation.

摘要

对5例儿童早期发病的神经纤维瘤病(冯雷克林霍增氏病)患者的临床症状、放射学及组织学改变进行了一项随访研究。该疾病的临床表现主要局限于面部。在组织学超微结构研究中,检测到了不同类型的神经纤维瘤(丛状神经纤维瘤、嗜银神经纤维瘤和弥漫性神经纤维瘤)。根据临床发现与神经纤维瘤组织学类型之间的相关性,特别提及肿瘤的边界、手术切除后的复发情况以及疾病临床进程的预后,给出了初步结果。然而,只有通过标准化的临床、放射学和组织学研究方法持续观察更多患者,才能得出明确结论。

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