Evaluation of coronary artery disease in the Hurler syndrome by angiography

Elizabeth A Braunlin, David W. Hunter, William Krivit, Barbara A. Burke, Peter S. Hesslein, Priscilla T. Porter, Chester B Whitley

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Hurler syndrome (mucopolysaccharidosis type I-H) is an inherited lysosomal storage disease characterized by the widespread accumulation of mucopolysaccharide in tissues throughout the body.1 Although severe coronary artery luminal narrowing has been documented by postmortem examination of the heart of patients with Hurler syndrome,2 the only reported study in which coronary angiography was performed stated that the coronary arteries appeared normal.3 As part of an ongoing protocol at our institution, selective coronary angiography was performed in children with Hurler syndrome before bone marrow transplantation. The subsequent availability of postmortem material from 3 such patients has allowed us to perform a retrospective analysis of the usefulness of selective coronary angiography in detecting the presence of coronary artery disease in patients with the Hurler syndrome.

Original languageEnglish (US)
Pages (from-to)1487-1489
Number of pages3
JournalThe American Journal of Cardiology
Volume69
Issue number17
DOIs
StatePublished - Jun 1 1992

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Mucopolysaccharidosis I
Coronary Artery Disease
Angiography
Coronary Angiography
Coronary Vessels
Lysosomal Storage Diseases
Glycosaminoglycans
Bone Marrow Transplantation
Autopsy

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Evaluation of coronary artery disease in the Hurler syndrome by angiography. / Braunlin, Elizabeth A; Hunter, David W.; Krivit, William; Burke, Barbara A.; Hesslein, Peter S.; Porter, Priscilla T.; Whitley, Chester B.

In: The American Journal of Cardiology, Vol. 69, No. 17, 01.06.1992, p. 1487-1489.

Research output: Contribution to journalArticle

Braunlin, Elizabeth A ; Hunter, David W. ; Krivit, William ; Burke, Barbara A. ; Hesslein, Peter S. ; Porter, Priscilla T. ; Whitley, Chester B. / Evaluation of coronary artery disease in the Hurler syndrome by angiography. In: The American Journal of Cardiology. 1992 ; Vol. 69, No. 17. pp. 1487-1489.
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