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[放射学在结节性硬化症中的地位(作者译)]

[The place of radiology in tuberous sclerosis (author's transl)].

作者信息

Teske H J, Heckemann R, Mayer H J, Odenwälder H, Krautzun K

出版信息

Rofo. 1978 Dec;129(6):770-7. doi: 10.1055/s-0029-1231203.

Abstract

Tuberous sclerosis is a rare disease in which hamartomas may be found in the brain, the retina, the skin and in other internal organs. The classic form of the disease showing mental retardation, epilepsy and adenoma sebaceum is easily recognised. Incomplete forms, however, can provide considerable diagnostic difficulties. Angiographically, appearances are found which cannot be differentiated with certainty from those of malignant tumours. Malignant tumours are very rare in this condition. Nephrectomy can be avoided if the diagnosis is made at an early stage. Two female patients are reported, in one of whom the diagnosis was made by renal angiography. Despite the presence of large tumours in both kidneys, these organs could be preserved by surgery. In the second patient there were also bilateral renal hamartomas, but surgery was not carried out. Both cases showed typical changes in the fingers and toes as well as intracerebral calcification, and in one the lungs were affected.

摘要

结节性硬化症是一种罕见疾病,在大脑、视网膜、皮肤和其他内脏器官中可能会发现错构瘤。具有智力迟钝、癫痫和皮脂腺瘤的典型疾病形式很容易识别。然而,不完全形式可能会带来相当大的诊断困难。在血管造影中,会出现一些外观表现,无法与恶性肿瘤的表现明确区分。在这种情况下,恶性肿瘤非常罕见。如果早期做出诊断,可以避免肾切除术。报告了两名女性患者,其中一名通过肾血管造影做出诊断。尽管双肾都存在大肿瘤,但通过手术保住了这些器官。第二名患者也有双侧肾错构瘤,但未进行手术。两例均表现出手指和脚趾的典型变化以及脑内钙化,其中一例肺部也受到影响。

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