Pseudoxanthoma elasticum with cardiac involvement : a case report and review of the literature

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dc.contributor.authorPrzybojewski, J. Z.
dc.contributor.authorMaritz, F.
dc.contributor.authorTiedt, F. A. C.
dc.contributor.authorVan der Walt, J. J.
dc.date.accessioned2011-03-18T14:59:50Z
dc.date.available2011-03-18T14:59:50Z
dc.date.issued1981-02
dc.descriptionThe original publication is available at http://www.samj.org.za
dc.description.abstractA young Black man with many features of pseudoxanthoma elasticum (PXE), confirmed by skin biopsy, complained of classic angina pectoris, decreasing effort tolerance, and palpitations. Clinically he was in severe congestive cardiac failure which was confirmed by echocardiography and cardiac catheterization, investigations which indicated the presence of a 'congestive' cardiomyopathy. Selective coronary arteriography showed normal epicardial vessels. Antemortem endomyocardial biopsy in this condition is described for the first time in the literature. This showed abnormal light microscopic and electron microscopic features. It is postulated that the predominant cause of congestive cardiac failure and angina pectoris in this disease is a diffuse arteriopathy secondary to elastic fibre dysgenesis, involving the small intramural coronary vessels ('small-vessel disease'). Hitherto it has been accepted that the endocardial changes have been most important in the pathophysiology. A review of the literature as it applied to cardiac involvement in PXE is undertaken.en_ZA
dc.description.versionPublishers' version
dc.identifier.citationPrzybojewski, J. Z., Maritz, F., Van der Walt, J. J., 1981. Pseudoxanthoma elasticum with cardiac involvement : a case report and review of the literature. SA medical journal, 21 February: 268-275
dc.identifier.issn2078-5135 (online) 0256-9574 (print)
dc.identifier.urihttp://hdl.handle.net/10019.1/8082
dc.language.isoen
dc.publisherHMPG
dc.rights.holderThe authors
dc.subjectPseudoxanthoma elasticumen_ZA
dc.titlePseudoxanthoma elasticum with cardiac involvement : a case report and review of the literatureen_ZA
dc.typeArticle
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