Clinical and Laboratory Findings in Jewish and Bedouin Patients in Southern Israel Who Were Diagnosed with Factor VII Deficiency

Congenital factor VII deficiency is a rare recessive autosomal bleeding disorder with a wide spectrum of clinical manifestations. To compare the clinical and laboratory findings in Jewish and Bedouin patients with factor VII deficiency. The clinical and laboratory findings of patients with factor VI...

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Bibliographic Details
Published in:	The Israel Medical Association journal Vol. 21; no. 5; pp. 318 - 321
Main Authors:	Blechman, Shahar, Fruchtman, Yariv, Perry, Zvi H, Mazar, Julia, Ben Harosh, Miriam, Abed, Abuquidar, Rozenberg, Nurit, Kenet, Gila, Leibovitz, Eugene
Format:	Journal Article
Language:	English
Published:	Israel 01-05-2019
Subjects:	Adolescent Adult Arabs - statistics & numerical data Child, Preschool Factor VII Deficiency - congenital Factor VII Deficiency - diagnosis Factor VII Deficiency - ethnology Factor VII Deficiency - mortality Female Hematologic Tests - methods Hematologic Tests - statistics & numerical data Humans Infant Israel - epidemiology Jews - statistics & numerical data Male Middle Aged Mortality Patient Care Management - methods Prevalence Severity of Illness Index Israel
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Summary:	Congenital factor VII deficiency is a rare recessive autosomal bleeding disorder with a wide spectrum of clinical manifestations. To compare the clinical and laboratory findings in Jewish and Bedouin patients with factor VII deficiency. The 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. Seventy-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. 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 clinical spectrum of the disease was found to be more severe in the Bedouin population.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	1565-1088