PubMed1975
The author has carried out a clinical and genetic investigation of a large family who lives in the south of the Algerian Sahara and comprises 213 individuals belonging to 8 generations. Among the examined members of this family, 105 (49.2%) showed repeated nose bleeding. However, no anomalies of the blood or the coagulation could be found in these patients. From the physical point of view they were all normally developed and vigorous. The distribution of the patients according to age showed no pedilection for any age group (affected individuals between 7 months and 85 years). The condition affected both sexes with a slight predominance in favour of females (53.4% of women against 46.6% of men). The inheritance is autosomal dominant wigh sometimes skipping of one or several generations. In one sibship all 13 members were affected owing to the fact that the father was an affected homozygote, offspring of a marriage between two affected cousins. The author proposes for this new noslological and genetic entity the term hereditary recurrent epistaxis. This capillaropathy has to be distinguished in particular from hereditary hemorrhagic telangiectasia of Rendu-Osler.