What is the prevalence of congenital fibrinogen disorders?

Updated: Oct 06, 2020
  • Author: Irene S Pakos, DO; Chief Editor: Perumal Thiagarajan, MD  more...
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Answer

Answer

The true prevalence of congenital fibrinogen disorders is unknown. The condition appears to be rare, however, with only 400 families reported as of 2012. [6, 7] Of these patients, 55% were asymptomatic (their cases detected by chance), 25% had bleeding symptoms, and 20% presented with thrombosis. The prevalence of dysfibrinogenemia in patients with a history of venous thrombosis is low (0.8%), as deduced from 9 studies in 7 countries on 2376 subjects. [7] No variation by race, age, or sex is known. Mortality is related to the severity of bleeding and/or to thrombotic complications at presentation.


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