Document Detail


Fibromuscular dysplasia of the internal carotid artery associated with alpha1-antitrypsin deficiency.
MedLine Citation:
PMID:  9696074     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: A deficiency of alpha1-antitrypsin has been implicated in the development of various disorders affecting medium-sized arteries, including intracranial aneurysms, cervicocephalic arterial dissections, and fibromuscular dysplasia (FMD). We performed alpha1-antitrypsin phenotyping in three consecutive patients who underwent bypass surgery for FMD of the extracranial internal carotid artery to test the hypothesis that alpha1-antitrypsin deficiency is a genetic risk factor for the development of FMD. METHODS: The study population consisted of three women (aged 37, 49, and 53 years, respectively) who had bilateral internal carotid artery stenosis caused by FMD. The indications for surgery included ocular or cerebral ischemic symptoms in two patients and progressive stenosis in one patient. The diagnosis of FMD was confirmed by histological examination of the resected segment of artery. The alpha1-antitrypsin phenotype was determined by isoelectric focusing in polyacrylamide gels. RESULTS: Two of the three patients had a heterozygous alpha1-antitrypsin deficiency (PiMZ phenotype). Pathological examination of the resected arterial segment showed typical medial FMD with focal intimal fibroplasia in both patients with the PiMZ phenotype. CONCLUSION: These findings suggest that a heterozygous alpha1-antitrypsin deficiency may be a genetic risk factor for the development of FMD of the internal carotid artery.
Authors:
W I Schievink; F B Meyer; J E Parisi; E F Wijdicks
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Neurosurgery     Volume:  43     ISSN:  0148-396X     ISO Abbreviation:  Neurosurgery     Publication Date:  1998 Aug 
Date Detail:
Created Date:  1998-11-24     Completed Date:  1998-11-24     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7802914     Medline TA:  Neurosurgery     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  229-33; discussion 233-4     Citation Subset:  IM    
Affiliation:
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA.
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MeSH Terms
Descriptor/Qualifier:
Adult
Carotid Artery, Internal / radiography,  surgery
Carotid Stenosis / genetics*,  radiography,  surgery
Cerebral Angiography
Female
Fibromuscular Dysplasia / genetics*,  radiography,  surgery
Humans
Middle Aged
Phenotype*
Risk Factors
alpha 1-Antitrypsin Deficiency / genetics*,  radiography,  surgery

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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