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

Updated: Apr 02, 2018
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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Answer

Overall estimated frequency of the autosomal recessive disorder involving a severe deficiency of subunit A is approximately 1 case per 2 million population. Previously, consanguinity was believed to be necessary, but the detection of compound heterozygotes by the application of molecular techniques is changing that perception. Approximately 200 cases of FXIII deficiency have been described thus far. [68] See Other Problems to be Considered for a discussion of acquired FXIII deficiency related to diseases or inhibitors.


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