Multiple contemporary arterial dissection in Ehlers-Danlos syndrome type IV

Submitted: 25 February 2014
Accepted: 6 April 2014
Published: 20 March 2015
Abstract Views: 1927
PDF: 901
HTML: 866
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

Authors

We report a case of multiple spontaneous arteries dissection in a 52-year-old female; the patient had a relevant family history of vascular complications and typical features so we hypothesized vascular Ehlers-Danlos syndrome (EDS) that was confirmed by genetic analysis of COL3A1 gene. We adopted a conservative approach: the patient was treated with heparin in the acute phase followed by aspirin and then celiprolol was started on the basis of a recent trial that demonstrates a reduction in arterial events in EDS patient treated. A careful follow-up was done with Doppler ultrasound and computed tomography scan, as non-invasive diagnostic techniques are preferred in these patients, and no other vascular symptomatic events have occurred. We tested all living relatives: half of them had COL3A1 mutation, they were referred to another center specialized in rare diseases and EDS for long-term follow-up and genetic counseling. This case demonstrates as a careful evaluation of clinical signs, clinical history of the patient and his family has allowed a definitive diagnosis, proper management of the patient during the acute event and in terms of prophylaxis of recurrence.

Dimensions

Altmetric

PlumX Metrics

Downloads

Download data is not yet available.

PlumX Metrics

PlumX Metrics  provide insights into the ways people interact with individual pieces of research output (articles, conference proceedings, book chapters, and many more) in the online environment. Examples include, when research is mentioned in the news or is tweeted about. Collectively known as PlumX Metrics, these metrics are divided into five categories to help make sense of the huge amounts of data involved and to enable analysis by comparing like with like.

Citations

Supporting Agencies

No contribution

How to Cite

Rocci, A., Paganelli, E., Rossetti, A., Marcato, C., Martorana, D., Tassoni, M. I., & Quintavalla, R. (2015). Multiple contemporary arterial dissection in Ehlers-Danlos syndrome type IV. Italian Journal of Medicine, 9(1), 77–81. https://doi.org/10.4081/itjm.2015.494

Similar Articles

You may also start an advanced similarity search for this article.