• Shahar Blechman, Yariv Fruchtman, Zvi H Perry, Julia Mazar, Miriam Ben Harosh, Abuquidar Abed, Nurit Rozenberg, Gila Kenet, and Eugene Leibovitz.
• Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.
• Isr Med Assoc J. 2019 May 1; 21 (5): 318-321.

BackgroundCongenital factor VII deficiency is a rare recessive autosomal bleeding disorder with a wide spectrum of clinical manifestations.ObjectivesTo compare the clinical and laboratory findings in Jewish and Bedouin patients with factor VII deficiency.MethodsThe clinical and laboratory findings of patients with factor VII deficiency treated at Soroka Medical Center, a tertiary hospital in Israel, from 2005 to 2015 were analyzed regarding blood factor levels, illness severity, treatment administration, and disease outcome.ResultsSeventy-eight patients were enrolled (1:13,000 of the population in southern 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 univariable analysis, Bedouin patients exhibited a more severe illness, with significantly higher complication and fatality rates, and required more preventive treatment than the Jewish patients.ConclusionsThe prevalence of congenital factor VII deficiency (including severe deficiency) in the Jewish and Bedouin populations of southern Israel is higher than previously reported. The clinical spectrum of the disease was found to be more severe in the Bedouin population.

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