LINKAGE OF FAMILIAL HIBERNIAN FEVER TO CHROMOSOME 12P13

Autosomal dominant periodic fevers are characterized by intermittent f ebrile attacks of unknown etiology and by recurrent abdominal pains. T he biochemical and molecular bases of all autosomal dominant periodic fevers are unknown, and only familial Hibernian fever (FHF) has been d escribed as a distinct clinical entity. FHF has been reported in three families-the original Irish-Scottish family and two Irish families wi th similar clinical features. We have undertaken a genomewide search i n these families and report significant multipoint LOD scores between the disease and markers on chromosome 12p13. Cumulative multipoint lin kage analyses indicate that an FHF gene is likely to be located in an 8-cM interval between D12S77 and D12S356, with a maximum LOD score (Z( max)) of 3.79. The two-point Z(max) was 3.11, for D12S77. There was no evidence of genetic heterogeneity in these three families; it is prop osed that these markers should be tested in other families, of differe nt background, that have autosomal dominant periodic fever, as a prelu de to identification of the FHF-susceptibility gene.