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Pingel, Simon; Pausewang, Kristin Solveig; Passon, Sebastian Gorgonius; Blatzheim, Anna Katharina; Gliem, Martin; Issa, Peter Charbel; Hendig, Doris; Horlbeck, Fritz; Tuleta, Izabela; Nickenig, Georg; Schahab, Nadjib; Skowasch, Dirk; Schaefer, Christian Alexander

Increased vascular occlusion in patients with pseudoxanthoma elasticum

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  • Media type: E-Article
  • Title: Increased vascular occlusion in patients with pseudoxanthoma elasticum
  • Contributor: Pingel, Simon; Pausewang, Kristin Solveig; Passon, Sebastian Gorgonius; Blatzheim, Anna Katharina; Gliem, Martin; Issa, Peter Charbel; Hendig, Doris; Horlbeck, Fritz; Tuleta, Izabela; Nickenig, Georg; Schahab, Nadjib; Skowasch, Dirk; Schaefer, Christian Alexander
  • imprint: Hogrefe Publishing Group, 2017
  • Published in: Vasa
  • Language: English
  • DOI: 10.1024/0301-1526/a000583
  • ISSN: 0301-1526; 1664-2872
  • Keywords: Cardiology and Cardiovascular Medicine
  • Origination:
  • Footnote:
  • Description: <jats:p> Abstract. Background: Pseudoxanthoma elasticum (PXE) is an autosomal recessive inherited multisystem disorder of the connective tissue caused by a loss-of-function mutation of the ABCC6 gene. It can affect the cardiovascular system, presumably leading to a high prevalence of atherosclerosis. Patients and methods: 46 PXE patients and 18 controls underwent an angiological examination consisting of measurement of ankle-brachial index (ABI), strain-gauge arterial reserve (SGAR), arterial resting perfusion, pulse wave index (PWI), central pulse wave velocity, and ultrasound examination. Results: With an average age of 51.4 ± 12.4 years, 35/46 (76.1 %) of the PXE patients had atherosclerotic lesions, and 10 of them (28.6 %) had a chronic vascular occlusion of one or more peripheral vessels. 34/46 (73.9 %) had a pathologic ABI &lt; 0.9, 15/42 (35.7 %) had a pathological SGAR &lt; 10 mL/100 mL tissue/min, and 23/38 (60.5 %) had a pathological PWI &gt; 180. The differences between the groups were statistically significant for ABI, arterial reserve, and PWI. Conclusions: In PXE patients atherosclerosis was found with a much higher prevalence than expected. Moreover, they were at very high risk for total vessel occlusions.
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