| Literature DB >> 26798684 |
Audrey Courtois1, Betty V Nusgens1, Philippe Delvenne2, Michel Meurisse3, Jean-Olivier Defraigne4, Alain C Colige1, Natzi Sakalihasan4.
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is a risk factor for several cardiovascular disorders such as intracranial aneurysm or aortic dissection, preferentially occurring at the thoracic or abdominal level. A 47-year-old man suffering from ADPKD had renal transplantation. Sixteen hours after surgery, he presented with left leg pain. Clinical and ultrasound examination revealed thrombosis of the external left iliac artery. Therefore, we decided to perform intra-arterial angiography to evaluate the possibility of an endovascular treatment. Aorto-femorography showed an obstruction of the external left iliac artery that was found during emergency surgery, consecutive to a dissection, which occurred following the surgery for kidney transplantation. The resected segment of the dissected vessel was analyzed by histology. Collagen fibers organization and density in the adventitia and smooth muscle cells density in the media were similar in the dissected and a normal artery from a healthy donor. By contrast, an almost complete disappearance and fragmentation of elastic lamellae were observed in the media of the dissected artery, most likely responsible for the weakening of the arterial wall and its dissection. Association between ADPKD and single dissection of the iliac artery has been rarely reported. Relationship between inactivation of polycystin/PKD genes and elastic fibers degradation through elevated TGFβ signaling and matrix metalloproteinase 2 (MMP2) elastolytic activity, as recently reported in ADPKD, would be worth investigating.Entities:
Keywords: Arterial dissection; Autosomal dominant polycystic kidney disease; Elastic lamellae
Year: 2013 PMID: 26798684 PMCID: PMC4682711 DOI: 10.12945/j.aorta.2013.12.012
Source DB: PubMed Journal: Aorta (Stamford) ISSN: 2325-4637