Suppr超能文献

关于视神经牵牛花综合征的汉德曼异常的临床症状?(作者译)

[On the clinical picture of Handmann's anomaly of the optic nerve Morning glory syndrome? (author's transl)].

作者信息

Rieger G

出版信息

Klin Monbl Augenheilkd. 1977 May;170(5):697-706.

PMID:894968
Abstract

The author reports on the case of a 20-year-old patient who shows the characteristic changes of the central vessels, described by Handmann in 1929 to be a "herditary degeneration - ppresumably congenital and glial - of the optic nerve in which the central vessels are particularly involved". The optic papilla of about normal size is surrounded here by a slightly protruding ridge, which shows separate pigmentary sediments on its edge. The retinal vessels arise out of the depth in increased number on the edge of the papilla. An excavation of the papilla cannot be seen. In its place a whitish-yellow mass surrounded by grayish-pink colored tissue is evident, which obstructs the view into the deeper layers. Kinkler described very similar cases in 1970 and named the characteristic changes "morning glory syndrome". However, in the cases he described, the papillas were apparently greatley enlarged. In addition to the changes in the papillary area and the vessel structure in our case, there are definite changes in the macula (on both sides). Due to the conspicuous distribution of vessels within the papillary region in the father of the patient, similar to those found in Handmann's anomaly of the nerve, the author believes that he can safely assume a developmental disturbance of the origin of the retinal vessels of the papillary area in this case also and that therefore a hereditary component of this developmental disturbance could be described for the first time since Handmann in 1929. In the kin which the author examined, a further apparently familial renal hypoplasia was noted.

摘要

作者报告了一名20岁患者的病例,该患者呈现出中央血管的特征性变化,这种变化在1929年被汉德曼描述为“视神经的遗传性变性——可能是先天性和神经胶质的——其中中央血管尤其受累”。此处,大小约正常的视乳头被一条略微突出的嵴所环绕,嵴的边缘有散在的色素沉着。视网膜血管在视乳头边缘从深处增多穿出。视乳头未见凹陷。在视乳头处可见一个灰白色肿块,周围是灰粉色组织,这使得无法看清更深层的结构。金克勒在1970年描述了非常相似的病例,并将这种特征性变化命名为“牵牛花综合征”。然而,在他所描述的病例中,视乳头明显增大。除了我们病例中视乳头区域和血管结构的变化外,黄斑(双侧)也有明确变化。由于患者父亲视乳头区域血管分布明显,与汉德曼神经异常中发现的情况相似,作者认为在这种情况下也可以肯定地认为视乳头区域视网膜血管起源存在发育障碍,因此自1929年汉德曼以来首次可以描述这种发育障碍的遗传成分。在作者检查的亲属中,还发现了另一种明显的家族性肾发育不全。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验