Vascular Ehlers-Danlos syndrome presenting as rapidly progressive multiple arterial aneurysms and dissections

被引：12

作者：

Barbosa, Eduardo J. Mortani, Jr. ^{[1
]}

Pyeritz, Reed E. ^{[2
,3
]}

Litt, Harold ^{[1
]}

Desjardins, Benoit ^{[1
]}

机构：

[1] Univ Penn, Dept Radiol, Raymond & Ruth Perelman Sch Med, Philadelphia, PA 19104 USA

[2] Raymond & Ruth Perelman Univ Penn, Dept Med, Sch Med, Philadelphia, PA USA

[3] Raymond & Ruth Perelman Univ Penn, Dept Genet, Sch Med, Philadelphia, PA USA

来源：

AMERICAN JOURNAL OF MEDICAL GENETICS PART A | 2011年 / 155A卷 / 12期

关键词：

vascular Ehlers-Danlos syndrome; arterial aneurysm; arterial dissection; vascular imaging; SYNDROME TYPE-IV; ENDOVASCULAR TREATMENT; SPONTANEOUS RUPTURE; PATIENT; THROMBOSIS; FEATURES; REPAIR;

D O I：

10.1002/ajmg.a.34332

中图分类号：

Q3 [遗传学];

学科分类号：

071007 ; 090102 ;

摘要：

Life expectancy in vascular EhlersDanlos syndrome (EDS) is shortened due to spontaneous rupture of arteries, the colon and the gravid uterus. Two adolescent males with vascular EDS illustrate rapid progression of arterial aneurysms, dissections, and rupture. Radiologic imaging played an important role in initially diagnosing and monitoring the evolution of arterial involvement. Both prophylactic and emergency management remain largely ineffective in this connective tissue disorder; however, noninvasive imaging may provide important prognostic information. (C) 2011 Wiley Periodicals, Inc.

引用

页码：3090 / 3094

页数：5

共 33 条

[1]

Asherson RA, 2006, J RHEUMATOL, V33, P2091

[2] Mimics of Vasculitis: Vascular Ehlers-Danlos Syndrome Masquerading as Polyarteritis Nodosa [J].

Badawi, Ramy A. ;

Brent, Lawrence H. ;

Feinstein, David E. .

JOURNAL OF RHEUMATOLOGY, 2009, 36 (08) :1845-1847

[3]

Barabas AP, 2000, NEW ENGL J MED, V343, P366

[4]

Beighton P, 1998, AM J MED GENET, V77, P31, DOI 10.1002/(SICI)1096-8628(19980428)77:1<31::AID-AJMG8>3.0.CO

[5]

2-O

[6] Contemporary management of vascular complications associated with Ehlers-Danlos syndrome [J].

Brooke, Benjamin S. ;

Arnaoutakis, George ;

McDonnell, Nazli B. ;

Black, James H., III .

JOURNAL OF VASCULAR SURGERY, 2010, 51 (01) :131-139

[7]

Byers PH, 2007, EMERY RIMOINS PRINCI, P3625

[8]

Dalgleish R, 2011, EHLERS DANLOS SYNDRO

[9] Endovascular repair of multiple infrageniculate aneurysms in a patient with vascular type Ehlers-Danlos syndrome [J].

Domenick, Natalie ;

Cho, Jae S. ;

Abu Hamad, Ghassan ;

Makaroun, Michel S. ;

Chaer, Rabih A. .

JOURNAL OF VASCULAR SURGERY, 2011, 54 (03) :848-850

[10] Ehlers-Danlos syndrome type IV [J].

Germain, Dominique P. .

ORPHANET JOURNAL OF RARE DISEASES, 2007, 2 (1)

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