3 years ago

Pulmonary microvascular architecture in hereditary haemorrhagic telangiectasia

A 24-year-old Caucasian man was admitted with a known hereditary haemorrhagic telangiectasia (HHT) and heterozygous mutation of factor V Leiden following episodes of cerebral infarctions in occipital lobes, cerebellum and brainstem. In his case history, the patient underwent several interventional embolisation of arteriovenous (AV) malformations in the middle and lower lobes (figure 1). However, those were not completely successful as the malformations were diffuse. We performed video-assisted thoracoscopic surgery with a resection of the middle lobe and a wedge resection of segment 10.

HHT (Osler-Weber-Rendu disease) is an autosomal-dominant disease determined by multiple dilated vessels including mucocutaneous telangiectasias and vascular malformations of visceral organs resulting in recurrent epistaxis, gastrointestinal bleedings, paradoxical emboli, cerebral infarctions and abscesses.1 In about 55% of patients with HHT type 1 (HHT-1) and about 10% of patients with HHT type 2 (HHT-2), large pulmonary arteriovenous malformations (PAVMs) are found which...

Publisher URL: http://thorax.bmj.com/cgi/content/short/72/9/861

DOI: 10.1136/thoraxjnl-2016-209299

You might also like
Discover & Discuss Important Research

Keeping up-to-date with research can feel impossible, with papers being published faster than you'll ever be able to read them. That's where Researcher comes in: we're simplifying discovery and making important discussions happen. With over 19,000 sources, including peer-reviewed journals, preprints, blogs, universities, podcasts and Live events across 10 research areas, you'll never miss what's important to you. It's like social media, but better. Oh, and we should mention - it's free.

  • Download from Google Play
  • Download from App Store
  • Download from AppInChina

Researcher displays publicly available abstracts and doesn’t host any full article content. If the content is open access, we will direct clicks from the abstracts to the publisher website and display the PDF copy on our platform. Clicks to view the full text will be directed to the publisher website, where only users with subscriptions or access through their institution are able to view the full article.