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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. CONCLUSIONS 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.
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
December 1997,
Developmental medicine and child neurology,
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
January 1982,
The Journal of cardiovascular surgery,
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
March 1999,
Neurology India,
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
April 1980,
The American surgeon,
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
July 1984,
RoFo : Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin,
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
January 1986,
Acta chirurgica Belgica,
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
February 1983,
Neurologic clinics,
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
January 1991,
Angiologia,
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
July 1979,
World journal of surgery,
W I Schievink, and
F B Meyer, and
J E Parisi, and
E F Wijdicks
November 1974,
Archives of surgery (Chicago, Ill. : 1960),
