Which factors affect the level and activity of factor XIII (FXIII) in the pathophysiology of factor XIII (FXIII) deficiency?

Updated: Aug 01, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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Answer

When quantitative amine incorporation assays became available, healthy people were found to have an 8-fold spread in FXIIIa activity. [38] In recent studies of FXIII antigen and activity in humans, no correlation was found between these two parameters. During the search for an explanation, 23 unique FXIIIa genotypes were found. The Leu34 and Leu564 variants gave rise to increased specific activity; the Phe204 variant lowered specific activity. Other mutations gave rise to low, high, or median FXIII-specific activity, and some variants had no effect. [39, 40]

In a study of the variability of FXIII levels in racial groups, FXIII activity was found to be higher in Asian Indians (male and female) than in their Chinese counterparts, accounting for approximately one fourth of the variability. Common genetic polymorphisms in the A and B chains appeared to contribute to the differences. [41] An influence exerted by acquired factors was evident in the higher FXIII levels found in women who smoked 20 or more cigarettes per day during a normal pregnancy than was found in nonsmokers, with a lesser drop in the second half of pregnancy. [42]


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