Arterial fibromuscular dysplasia associated with severe α1-antitrypsin deficiency

W. I. Schievink, J. Bjornsson, Joseph E Parisi, U. B S Prakash

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Abstract

Objective: To elucidate the putative arteriopathy associated with α1- antitrypsin (α1-AT) deficiency. Design: We retrospectively studied the frequency of occurrence of fibromuscular dysplasia (FMD) in patients with α1-AT deficiency in whom a postmortem examination had been done during a 10-year period at the Mayo Clinic. Material and Methods: The medical records of all patients in whom an autopsy was done at the Mayo Clinic between 1983 and 1992 were reviewed to identify all those with a diagnosis of α1-AT deficiency or FMD. Results: Arterial FMD was found in 2 of 6 patients with α1-AT deficiency (33.3%; 95% confidence interval, 4.3 to 77.7%) in comparison with 23 of 6,690 patients without α1-AT deficiency (0.3%; 95% confidence interval, 0.2 to 0.5%). In both patients with α1-AT deficiency and FMD, the arterial media was thickened and composed of irregular arrays of muscular and connective tissue fibers in a background of mucoid ground substance. Conclusion: These findings provide further evidence for an underlying arteriopathy in patients with α1-AT deficiency and suggest that FMD may be a nonspecific disorder.

Original languageEnglish (US)
Pages (from-to)1040-1043
Number of pages4
JournalMayo Clinic Proceedings
Volume69
Issue number11
StatePublished - 1994

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Fibromuscular Dysplasia
Autopsy
Confidence Intervals
Tunica Media
Connective Tissue
Medical Records

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Schievink, W. I., Bjornsson, J., Parisi, J. E., & Prakash, U. B. S. (1994). Arterial fibromuscular dysplasia associated with severe α1-antitrypsin deficiency. Mayo Clinic Proceedings, 69(11), 1040-1043.

Arterial fibromuscular dysplasia associated with severe α1-antitrypsin deficiency. / Schievink, W. I.; Bjornsson, J.; Parisi, Joseph E; Prakash, U. B S.

In: Mayo Clinic Proceedings, Vol. 69, No. 11, 1994, p. 1040-1043.

Research output: Contribution to journalArticle

Schievink, WI, Bjornsson, J, Parisi, JE & Prakash, UBS 1994, 'Arterial fibromuscular dysplasia associated with severe α1-antitrypsin deficiency', Mayo Clinic Proceedings, vol. 69, no. 11, pp. 1040-1043.
Schievink WI, Bjornsson J, Parisi JE, Prakash UBS. Arterial fibromuscular dysplasia associated with severe α1-antitrypsin deficiency. Mayo Clinic Proceedings. 1994;69(11):1040-1043.
Schievink, W. I. ; Bjornsson, J. ; Parisi, Joseph E ; Prakash, U. B S. / Arterial fibromuscular dysplasia associated with severe α1-antitrypsin deficiency. In: Mayo Clinic Proceedings. 1994 ; Vol. 69, No. 11. pp. 1040-1043.
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