title: Comparison of 2 Methods of Clot Solubility Testing in Detection of Factor XIII Deficiency.
abstract: BACKGROUND
  Deficiency of factor XIII (FXIII) is a rare bleeding disorder (RBD) affecting approximately 1 person per 2 million worldwide. Its life-threatening diatheses-umbilical cord bleeding, intracranial hemorrhage and central nervous system bleeding-present a significant challenge in both diagnosis and treatment. Confirming FXIII deficiency (FXIIID) is difficult and has been most commonly performed with the clot solubility test. Iran has limited resources and a rate of FXIIID 12-fold higher than the rest of the world, suggesting that in this country optimization of the clot solubility test is crucial. For this reason, we compared clot solubility test methods traditionally used in Iran.
  
  
  METHODS
  In this study, we assessed patients suspected to have FXIIID with routine coagulation tests and 2 different methods of clot solubility testing including 5M urea and monochloroacetic acid (MCA) as solubilizing agents, and thrombin and calcium chloride as clotting agents. Finally, we analyzed the data obtained with SPSS software.
  
  
  RESULTS
  During the study period, we were referred 83 patients with normal routine coagulation tests, of which 29 patients had abnormal clot solubility test results with 5M urea, and 21 had abnormal results with MCA (P = .03); 19 cases had abnormal results with both methods; 2 patients had abnormal results with MCA but normal results with urea. Ten patients had positive results with urea but normal results with MCA.
  
  
  CONCLUSIONS
  The clot solubility test with 5M urea as solubilizing method and thrombin as clotting agent is more sensitive in the detection of FXIIID, but simultaneous use of the 2 methods can prevent misdiagnosis of a considerable number of patients with FXIIID.
authors: Departments of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran.
authors: Akbar
authors: A
authors: Dorgalaleh
authors: Departments of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran.
authors: Shadi
authors: S
authors: Tabibian
authors: Student Research Committee, Kurdistan University of Medical Sciences, Sanandaj, Iran.
authors: Vahideh
authors: V
authors: Assadollahi
authors: School of Nursing and Midwifery, Shahroud University of Medical Sciences, Shahroud, Iran.
authors: Morteza
authors: M
authors: Shamsizadeh
authors: Health Promotion Research Center, Zahedan University of Medical Sciences, Zahedan, Iran hematology.1390@gmail.com.
authors: Iraj
authors: I
authors: Zareban
authors: Departments of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran.
authors: Shahrzad
authors: S
authors: Soori
authors: Departments of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran.
authors: Maryam
authors: M
authors: Daneshi
mesh_terms: D019411:Clinical Laboratory Techniques / Q000379:methods*; D003955:Diagnostic Tests, Routine / Q000379:methods*; D005177:Factor XIII Deficiency / Q000175:diagnosis*; D006801:Humans; D007492:Iran; D012680:Sensitivity and Specificity; D012995:Solubility; D013927:Thrombosis
keywords: clot solubility test; diagnosis; factor XIII deficiency