Aneurysms of the abdominal aorta: familial and genetic aspects in three hundred thirteen pedigrees.

peer reviewed ; PURPOSE: Familial clustering of abdominal aortic aneurysm was first noticed in 1977. METHODS: Through questionnaire and phone inquiry, familial data on 324 probands with abdominal aortic aneurysms allowed the establishment of 313 multigenerational pedigrees including 39 with multiple affected patients. RESULTS: There were 276 sporadic cases (264 men, 12 women); 81 cases belonged to multiplex pedigrees (76 men; 5 women). We compared familial and sporadic male cases; the ages at diagnosis were 64.1 +/- 7.9 years and 66.0 +/- 7.3 years (p < 0.05), respectively, the ages at rupt... Mehr ...

Verfasser: VERLOES, Alain
SakalihasanN, Natzi
Koulischer, Lucien
Limet, Raymond
Dokumenttyp: journal article
Erscheinungsdatum: 1995
Verlag/Hrsg.: Mosby Year Book
Schlagwörter: Adult / Age Factors / Aged / 80 and over / Alleles / Aortic Aneurysm / Abdominal/epidemiology/genetics/mortality / Aortic Rupture/epidemiology/genetics / Belgium/epidemiology / Female / Genes / Dominant/genetics / Humans / Male / Middle Aged / Nuclear Family / Pedigree / Probability / Risk Factors / Sex Factors / Statistics as Topic / Survival Rate / Human health sciences / Surgery / Sciences de la santé humaine / Chirurgie
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-28940196
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://orbi.uliege.be/handle/2268/109710

peer reviewed ; PURPOSE: Familial clustering of abdominal aortic aneurysm was first noticed in 1977. METHODS: Through questionnaire and phone inquiry, familial data on 324 probands with abdominal aortic aneurysms allowed the establishment of 313 multigenerational pedigrees including 39 with multiple affected patients. RESULTS: There were 276 sporadic cases (264 men, 12 women); 81 cases belonged to multiplex pedigrees (76 men; 5 women). We compared familial and sporadic male cases; the ages at diagnosis were 64.1 +/- 7.9 years and 66.0 +/- 7.3 years (p < 0.05), respectively, the ages at rupture were 65.4 +/- 6.6 years and 75.2 +/- 8.6 years (p < 0.001), and the rupture rate was 32.4% and 8.7% (p < 0.001). Survival curves were computed. Relative risk for male siblings of a male patient was 18. We performed a segregation analysis with the mixed model, the most likely explanation for occurrence of abdominal aortic aneurysm in our families was a single gene effect showing dominant inheritance. The frequency of the morbid allele was 1:250, and its age-related penetrance was not higher than 0.4. CONCLUSION: This analysis indicates the preeminence of genetic factors on multifactorial/environmental effects of the pathogenesis of abdominal aortic aneurysm.