Publication
Title
Loeys-Dietz syndrome: MDCT angiography findings
Author
Abstract
OBJECTIVE. Loeys-Dietz syndrome is a newly described phenotype caused by heterozygous mutations in the genes encoding type I or II transforming growth factor-beta (TGF-beta) receptor. Characterized by a unique constellation of clinical and pathologic findings, Loeys-Dietz syndrome manifests with aggressive vascular pathology. Aneurysms may form at a young age and have a propensity for arterial dissection. In addition, aneurysms rupture at diameters smaller than those used to dictate surgical intervention for other syndromes and disorders. This article presents the spectrum of arterial pathology that may be identified on MDCT angiography in patients with Loeys-Dietz syndrome. CONCLUSION. For patients with Loeys-Dietz syndrome, early diagnosis and rapid intervention are instrumental in averting catastrophic events. Serial imaging assessment by radiologists is an important component in the management of these patients.
Language
English
Source (journal)
The American journal of roentgenology. - Springfield, Ill., 1976, currens
Publication
Springfield, Ill. : 2007
ISSN
0361-803X [print]
1546-3141 [online]
Volume/pages
189 :1 (2007) , p. W29-W35
ISI
000247588100047
Full text (Publisher's DOI)
UAntwerpen
Publication type
Subject
External links
Web of Science
Record
Identification
Creation 05.11.2018
Last edited 04.09.2021