Intracranial aneurysm and hemorrhagic stroke in glucocorticoid-remediable aldosteronism

被引:134
作者
Litchfield, WR
Anderson, BF
Weiss, RJ
Lifton, RP
Dluhy, RG [1 ]
机构
[1] Brigham & Womens Hosp, Div Endocrine Hypertens, 221 Longwood Ave,RFB-2, Boston, MA 02115 USA
[2] Harvard Univ, Sch Med, Boston, MA 02115 USA
[3] Yale Univ, Sch Med, Howard Hughes Med Inst, Dept Med, New Haven, CT 06510 USA
[4] Yale Univ, Sch Med, Howard Hughes Med Inst, Dept Genet,Boyer Ctr Mol Med, New Haven, CT 06510 USA
关键词
glucocorticoid-remediable aldosteronism; hyperaldosteronism; cerebrovascular disease; intracranial aneurysm; hemorrhagic stroke;
D O I
10.1161/01.HYP.31.1.445
中图分类号
R6 [外科学];
学科分类号
1002 ; 100210 ;
摘要
There are anecdotal reports of early cerebrovascular complications occurring in patients with glucocorticoid-remediable aldosteronism (GRA). The issue has never been systematically evaluated. In this study, we retrospectively reviewed the International Registry for GRA to see if there was an association between cerebrovascular complications and GRA. We searched the records of 376 patients from 27 genetically proven GRA pedigrees for premature death or cerebrovascular complications. Each case was subsequently verified through the referring physician, or autopsy reports. The number of complications occurring in patients with proven GRA were compared to GRA negative subjects from the same pedigrees. There were 18 cerebrovascular events in 15 patients with proven GRA (n=167) and none in the GRA negative group (n=194; P<.001). There were an additional 15 events in 15 subjects that were suspected of having GRA based on clinical history. Seventy percent of events were hemorrhagic strokes; the overall case fatality rate was 61%. The mean (+/- SD) age at the time of the initial event was 31.7+/-11.3 years. In total, 48% of all GRA pedigrees and 18% of all GRA patients had cerebrovascular complications, which is similar to the frequency of aneurysm in adult polycystic kidney disease. GRA is associated with high morbidity and mortality from early onset of hemorrhagic stroke and ruptured intracranial aneurysms. Screening for intracranial aneurysm with magnetic resonance angiography is advised for patients with genetically proven GRA.
引用
收藏
页码:445 / 450
页数:6
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