Characterization of @entity1 with XXXX without wheals: the importance of @entity6343 gene screening.

@entity1659 @entity28444 @entity1 @entity6343 @entity74 @entity113 @entity7058
Sporadic and familiar forms of non-histaminergic @entity113 and normal C1 inhibitor encompass a group of disorders possibly caused by bradikinin. We aimed to study the subgroups of @entity1659 with FXII mutation (FXII- @entity7058 ), unknown @entity74 ( @entity7058 ) and idiopathic non-histaminergic acquired @entity113 (InH-AAE). We screened the @entity6343 locus in our cohort and delineated the clinical, laboratory and genetic features. Four families carried the @entity28444 mutation in @entity6343 gene. Haplotyping confirmed the hypothesis of a common founder. Six families were affected by @entity7058 and 13 @entity1 by sporadic InH-AAE. C4 levels were significantly lower in FXII- @entity7058 than in InH-AAE. In the FXII- @entity7058 group, none had attacks exclusively in high estrogenic states; acute attacks were treated with icatibant. Prophylaxis with tranexamic acid reduced the attack frequency in most @entity1 . Our study provides new data on the diagnosis, clinical features and treatment of non-histaminergic @entity113 , underlying the role of the screening for @entity6343 mutations.
