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

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

FXIII deficiency has been reported in many ethnic groups around the world, including persons from Canada, Europe, India, Israel, Japan, Kuala Lumpur, Pakistan, Papua New Guinea, South America, Thailand, Turkey, and the United States. A high rate of consanguinity was documented in affected persons in Iran. [69]

Diagnosis of disorders of FXIII inhibitors, which may have been missed in the past, is increasing as more laboratory support becomes available around the world. Increasing use of isoniazid (INH) to combat a worldwide rise in incidence of tuberculosis could contribute to an increased incidence of FXIII inhibitors in patients.

Variability in the distribution of mutations is exemplified by existing data. For example, significant ethnic heterogeneity was found in a Brazilian population in which the Val34Leu mutation was present in 51.2% of American Indians, 44% of whites, and 28.9% of Africans, but in only 2.5% of Japanese Asians. [70]


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