What is the role of quantitative testing in the workup 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

If the 5M urea solubility test demonstrates positive results, this finding should be confirmed by quantitating FXIII activity using a monodansylcadaverine or putrescine incorporation assay.

A new sensitive assay used to quantitate FXIII activity is based on monitoring the amount of ammonia (NH3) released by using glutamate dehydrogenase and nicotinamide adenine dinucleotide phosphate during the transamidation reaction (cross-linking) by FXIII. Another new and sensitive colorimetric assay is based on incorporation of 5-(biotinamido) pentylamine into fibrin/fibrinogen. [5]

In addition, a2PI and plasminogen activator inhibitor-1 assays should be performed to exclude abnormalities in the fibrinolytic pathway, which accelerate clot lysis. Sodium dodecylsulfate polyacrylamide gel electrophoresis under reducing conditions has been used to assess the presence of cross-linked g or a chains of fibrin, which is a reflection of FXIII activity. The studies must be performed by laboratory personnel with special expertise.


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