What is the prevalence of pediatric factor XIII (FXIII) deficiency?

Updated: Mar 15, 2019
  • Author: Helge Dirk Hartung, MD; Chief Editor: Cameron K Tebbi, MD  more...
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Answer

The prevalence of factor XIII deficiency is about 1 case per 2 million in the general population. Worldwide, the prevalence is highest in the Sistan and Baluchistan province of Iran. [3] In addition to Iran, population-specific mutations due to founder effects have been reported in Pakistan, India, Tunisia, and Finland.

A study by Gebhart et al of Austrian patients with mild to moderate bleeding disorders found that out of 418 individuals with these conditions, just one (0.2%) had factor XIII deficiency. Factor VIII, IX, and XI deficiencies occurred in 11 (2.6%), 3 (0.7%), and 3 (0.7%) patients, respectively. [4]


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