Clinicai and laboratory findings in jewish and bedouin patients in southern israel who were diagnosed with factor vii deficiency

Background: Congenital factor VII deficiency is a rare recessive autosomal bleeding disorder with a wide spectrum of clinicai manifestations. Objectives: To compare the clinicai and laboratory findings in Jewish and Bedouin patients with factor VII deficiency. Methods: The clinicai and laboratory findings of patients with factor VII deficiency treated at Soroka Medicai Center, a tertiary hospital in Israel, from 2005 to 2015 were analyzed regarding blood factor leveis, illness severity, treatment administration, and disease outcome. Results: Seventy-eight patients were enrolled (1:13,000 of the population in southem Israel) of whom 26 were diagnosed with severe factor VII deficiency (1:40,000). Sixty (76.9%) patients were Jewish and 18 (23.1%) were Bedouin. In uni- variable analysis, Bedouin patients exhibited a more severe illness, with significantly higher complication and fatality rates, and required more preventive treatment than the Jewish patients. Conclusions: The prevalence of congenital factor VII deficiency (including severe deficiency) in the Jewish and Bedouin populations of Southern Israel is higher than previously reported. The clinicai spectrum of the disease was found to be more severe in the Bedouin population.