Factor XIII Deficiency Questions & Answers

1. Shanbhag S, Ghosh K, Shetty S. Genetic basis of severe factor XIII deficiency in a large cohort of Indian patients: Identification of fourteen novel mutations. Blood Cells Mol Dis. 2016 Mar. 57:81-4. [QxMD MEDLINE Link].

2. Muszbek L, Pénzes K, Katona É. Auto- and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences. J Thromb Haemost. 2018 Feb 20. [QxMD MEDLINE Link].

3. Bouttefroy S, Meunier S, Milien V, Boucekine M, Chamouni P, Desprez D, et al. Congenital factor XIII deficiency: comprehensive overview of the FranceCoag cohort. Br J Haematol. 2020 Jan. 188 (2):317-320. [QxMD MEDLINE Link].

4. Chuliber FA, Penchasky D, Santoro DM, Viñuales S, Otero V, Villagra Iturre M, et al. Acquired factor XIII deficiency in patients under therapeutic plasma exchange: A poorly explored etiology. J Clin Apher. 2021 Feb. 36 (1):59-66. [QxMD MEDLINE Link].

5. Thakker S, McGehee W, Quismorio FP Jr. Arthropathy associated with factor XIII deficiency. Arthritis Rheum. 1986 Jun. 29(6):808-11. [QxMD MEDLINE Link].

6. McKenna R. Abnormal coagulation in the postoperative period contributing to excessive bleeding. Med Clin North Am. 2001 Sep. 85(5):1277-310, viii. [QxMD MEDLINE Link].

7. Wilmer M, Rudin K, Kolde H, et al. Evaluation of a sensitive colorimetric FXIII incorporation assay. Effects of FXIII Val34Leu, plasma fibrinogen concentration and congenital FXIII deficiency. Thromb Res. 2001 Apr 1. 102(1):81-91. [QxMD MEDLINE Link].

8. Laki K, Lóránd L. On the Solubility of Fibrin Clots. Science. 1948 Sep 10. 108(2802):280. [QxMD MEDLINE Link].

9. Duckert F, Jung E, Sherling DH. An undescribed congenital haemorrhagic diathesis probably due to fibrin stabilizing factor deficiency. Thromb Diath Haemorrh. 1960. 5:179.

10. Duckert F. Documentation of the plasma factor XIII deficiency in man. Ann N Y Acad Sci. 1972 Dec 8. 202:190-9. [QxMD MEDLINE Link].

11. Laki K. Our ancient heritage in blood clotting and some of its consequences. Ann N Y Acad Sci. 1972 Dec 8. 202:297-307. [QxMD MEDLINE Link].

12. Reitsma PH. Genetic principles underlying disorders of procoagulant and anticoagulant proteins. Coleman RW, Hirsh J, Marder VJ, et al, eds. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. Philadelphia, Pa: Lippincott Williams & Wilkins; 2001. 59-87.

13. Loewy AG, McDonagh J, Mikkola H, et al. Structure and function of F XIII. Coleman RW, Hirsh J, Marder VJ, et al, eds. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. Philadelphia, Pa: Lippincott Williams & Wilkins; 2001. 233-48.

14. Cushman M, O'Meara ES, Folsom AR, Heckbert SR. Coagulation factors IX through XIII and the risk of future venous thrombosis: the Longitudinal Investigation of Thromboembolism Etiology. Blood. 2009 Oct 1. 114(14):2878-83. [QxMD MEDLINE Link]. [Full Text].

15. Hoppe B, Tolou F, Dörner T, Kiesewetter H, Salama A. Gene polymorphisms implicated in influencing susceptibility to venous and arterial thromboembolism: frequency distribution in a healthy German population. Thromb Haemost. 2006 Oct. 96(4):465-70. [QxMD MEDLINE Link].

16. Wells PS, Anderson JL, Rodger MA, Carson N, Grimwood RL, Doucette SP. The factor XIII Val34Leu polymorphism: is it protective against idiopathic venous thromboembolism?. Blood Coagul Fibrinolysis. 2006 Oct. 17(7):533-8. [QxMD MEDLINE Link].

17. Shafey M, Anderson JL, Scarvelis D, Doucette SP, Gagnon F, Wells PS. Factor XIII Val34Leu variant and the risk of myocardial infarction: a meta-analysis. Thromb Haemost. 2007 Apr. 97(4):635-41. [QxMD MEDLINE Link].

18. Shin JI, Lee JS. Severe gastrointestinal vasculitis in Henoch-Schoenlein purpura: pathophysiologic mechanisms, the diagnostic value of factor XIII, and therapeutic options. Eur J Pediatr. 2007 Nov. 166(11):1199-200; author reply 1201. [QxMD MEDLINE Link].

19. Shin JI, Lee JS. Could measurement of factor XIII level detect the vasculitic process of Henoch-Schönlein purpura without skin rash?. Acta Paediatr. 2008 Apr. 97(4):395. [QxMD MEDLINE Link].

20. Kuroda K, Tajima S. Proliferation of HSP47-positive skin fibroblasts in dermatofibroma. J Cutan Pathol. 2008 Jan. 35(1):21-6. [QxMD MEDLINE Link].

21. Dorgalaleh A, Tabibian S, Shams M, Majid G, Naderi M, Casini A, et al. A Unique Factor XIII Mutation in Southeastern Iran with an Unexpectedly High Prevalence: Khash Factor XIII. Semin Thromb Hemost. 2019 Feb. 45 (1):43-49. [QxMD MEDLINE Link].

22. Ichinose A. Factor XIII is a key molecule at the intersection of coagulation and fibrinolysis as well as inflammation and infection control. Int J Hematol. 2012 Apr. 95(4):362-70. [QxMD MEDLINE Link].

23. Schroeder V, Kohler HP. Factor XIII Deficiency: An Update. Semin Thromb Hemost. 2013 Sep. 39(6):632-41. [QxMD MEDLINE Link].

24. Lorand L. Factor XIII: structure, activation, and interactions with fibrinogen and fibrin. Ann N Y Acad Sci. 2001. 936:291-311. [QxMD MEDLINE Link].

25. Siebenlist KR, Meh DA, Mosesson MW. Plasma factor XIII binds specifically to fibrinogen molecules containing gamma chains. Biochemistry. 1996 Aug 13. 35(32):10448-53. [QxMD MEDLINE Link].

26. Greenberg CS, Shuman MA. The zymogen forms of blood coagulation factor XIII bind specifically to fibrinogen. J Biol Chem. 1982 Jun 10. 257(11):6096-101. [QxMD MEDLINE Link].

27. Mosesson M. Hereditary abnormalities of fibrinogen. Beutler E, Lichtman M, Coller BS, et al, eds. Williams Hematology. New York, NY: McGraw-Hill; 2001. 1659-71.

28. Jenner L, Husted L, Thirup S, Sottrup-Jensen L, Nyborg J. Crystal structure of the receptor-binding domain of alpha 2-macroglobulin. Structure. 1998 May 15. 6(5):595-604. [QxMD MEDLINE Link].

29. Nagy B Jr, Simon Z, Bagoly Z, Muszbek L, Kappelmayer J. Binding of plasma factor XIII to thrombin-receptor activated human platelets. Thromb Haemost. 2009 Jul. 102(1):83-9. [QxMD MEDLINE Link].

30. Monteiro MR, Murphy EE, Galaria NA, Whitaker-Menezes D, Murphy GF. Cytological alterations in dermal dendrocytes in vitro: evidence for transformation to a non-dendritic phenotype. Br J Dermatol. 2000 Jul. 143(1):84-90. [QxMD MEDLINE Link].

31. Lorand L. Sol Sherry Lecture in Thrombosis : research on clot stabilization provides clues for improving thrombolytic therapies. Arterioscler Thromb Vasc Biol. 2000 Jan. 20(1):2-9. [QxMD MEDLINE Link].

32. Noguchi K, Ishikawa K, Yokoyama Ki, Ohtsuka T, Nio N, Suzuki E. Crystal structure of red sea bream transglutaminase. J Biol Chem. 2001 Apr 13. 276(15):12055-9. [QxMD MEDLINE Link].

33. Andersen MD, Kjalke M, Bang S, et al. Coagulation factor XIII variants with altered thrombin activation rates. Biol Chem. 2009 Dec. 390(12):1279-83. [QxMD MEDLINE Link].

34. Fox BA, Yee VC, Pedersen LC, et al. Identification of the calcium binding site and a novel ytterbium site in blood coagulation factor XIII by x-ray crystallography. J Biol Chem. 1999 Feb 19. 274(8):4917-23. [QxMD MEDLINE Link].

35. Casadio R, Polverini E, Mariani P, et al. The structural basis for the regulation of tissue transglutaminase by calcium ions. Eur J Biochem. 1999 Jun. 262(3):672-9. [QxMD MEDLINE Link].

36. Hevessy Z, Haramura G, Boda Z, Udvardy M, Muszbek L. Promotion of the crosslinking of fibrin and alpha 2-antiplasmin by platelets. Thromb Haemost. 1996 Jan. 75(1):161-7. [QxMD MEDLINE Link].

37. Moaddel M, Falls LA, Farrell DH. The role of gamma A/gamma ' fibrinogen in plasma factor XIII activation. J Biol Chem. 2000 Oct 13. 275(41):32135-40. [QxMD MEDLINE Link].

38. Weiss MS, Metzner HJ, Hilgenfeld R. Two non-proline cis peptide bonds may be important for factor XIII function. FEBS Lett. 1998 Feb 27. 423(3):291-6. [QxMD MEDLINE Link].

39. Jámbor C, Reul V, Schnider TW, Degiacomi P, Metzner H, Korte WC. In vitro inhibition of factor XIII retards clot formation, reduces clot firmness, and increases fibrinolytic effects in whole blood. Anesth Analg. 2009 Oct. 109(4):1023-8. [QxMD MEDLINE Link].

40. Lorand L, Urayama T, Atencio AC, Hsia DY. Inheritance of deficiency of fibrin-stabilizing factor (factor 13). Am J Hum Genet. 1970 Jan. 22(1):89-95. [QxMD MEDLINE Link]. [Full Text].

41. Anwar R, Gallivan L, Edmonds SD, Markham AF. Genotype/phenotype correlations for coagulation factor XIII: specific normal polymorphisms are associated with high or low factor XIII specific activity. Blood. 1999 Feb 1. 93(3):897-905. [QxMD MEDLINE Link].

42. Kangsadalampai S, Board PG. The Val34Leu polymorphism in the A subunit of coagulation factor XIII contributes to the large normal range in activity and demonstrates that the activation peptide plays a role in catalytic activity. Blood. 1998 Oct 15. 92(8):2766-70. [QxMD MEDLINE Link].

43. Saha N, Aston CE, Low PS, Kamboh MI. Racial and genetic determinants of plasma factor XIII activity. Genet Epidemiol. 2000 Dec. 19(4):440-55. [QxMD MEDLINE Link].

44. van Wersch JW, Vooijs ME, Ubachs JM. Coagulation factor XIII in pregnant smokers and non-smokers. Int J Clin Lab Res. 1997. 27(1):68-71. [QxMD MEDLINE Link].

45. Asahina T, Kobayashi T, Okada Y, et al. Studies on the role of adhesive proteins in maintaining pregnancy. Horm Res. 1998. 50 Suppl 2:37-45. [QxMD MEDLINE Link].

46. Asahina T, Kobayashi T, Okada Y, Goto J, Terao T. Maternal blood coagulation factor XIII is associated with the development of cytotrophoblastic shell. Placenta. 2000 May. 21(4):388-93. [QxMD MEDLINE Link].

47. Anwar R, Miloszewski KJ. Factor XIII deficiency. Br J Haematol. 1999 Dec. 107(3):468-84. [QxMD MEDLINE Link].

48. Noll T, Wozniak G, McCarson K, et al. Effect of factor XIII on endothelial barrier function. J Exp Med. 1999 May 3. 189(9):1373-82. [QxMD MEDLINE Link]. [Full Text].

49. Cario E, Goebell H, Dignass AU. Factor XIII modulates intestinal epithelial wound healing in vitro. Scand J Gastroenterol. 1999 May. 34(5):485-90. [QxMD MEDLINE Link].

50. Naito M, Nomura H, Iguchi A, Thompson WD, Smith EB. Effect of crosslinking by factor XIIIa on the migration of vascular smooth muscle cells into fibrin gels. Thromb Res. 1998 May 1. 90(3):111-6. [QxMD MEDLINE Link].

51. Wozniak G, Noll T, Brunner U, Hehrlein FW. Topical treatment of venous ulcer with fibrin stabilizing factor: experimental investigation of effects on vascular permeability. Vasa. 1999 Aug. 28(3):160-3. [QxMD MEDLINE Link].

52. Catani MV, Bernassola F, Rossi A, Melino G. Inhibition of clotting factor XIII activity by nitric oxide. Biochem Biophys Res Commun. 1998 Aug 10. 249(1):275-8. [QxMD MEDLINE Link].

53. Bernassola F, Rossi A, Melino G. Regulation of transglutaminases by nitric oxide. Ann N Y Acad Sci. 1999. 887:83-91. [QxMD MEDLINE Link].

54. Huang QQ, Teng MK, Niu LW. Purification and characterization of two fibrinogen-clotting enzymes from five-pace snake (Agkistrodon acutus) venom. Toxicon. 1999 Jul. 37(7):999-1013. [QxMD MEDLINE Link].

55. Arocha-Pinango CL, Marval E, Guerrero B. Lonomia genus caterpillar toxins: biochemical aspects. Biochimie. 2000 Sep-Oct. 82(9-10):937-42. [QxMD MEDLINE Link].

56. Zavalova L, Lukyanov S, Baskova I, et al. Genes from the medicinal leech (Hirudo medicinalis) coding for unusual enzymes that specifically cleave endo-epsilon (gamma-Glu)-Lys isopeptide bonds and help to dissolve blood clots. Mol Gen Genet. 1996 Nov 27. 253(1-2):20-5. [QxMD MEDLINE Link].

57. Undas A, Brummel KE, Musial J, Mann KG, Szczeklik A. Simvastatin depresses blood clotting by inhibiting activation of prothrombin, factor V, and factor XIII and by enhancing factor Va inactivation. Circulation. 2001 May 8. 103(18):2248-53. [QxMD MEDLINE Link].

58. Finney S, Seale L, Sawyer RT, Wallis RB. Tridegin, a new peptidic inhibitor of factor XIIIa, from the blood- sucking leech Haementeria ghilianii. Biochem J. 1997 Jun 15. 324 ( Pt 3):797-805. [QxMD MEDLINE Link].

59. Lee SY, Chang SK, Lee IH, Kim YM, Chung SI. Depletion of plasma factor XIII prevents disseminated intravascular coagulation-induced organ damage. Thromb Haemost. 2001 Mar. 85(3):464-9. [QxMD MEDLINE Link].

60. Sidelmann JJ, Gram J, Jespersen J, Kluft C. Fibrin clot formation and lysis: basic mechanisms. Semin Thromb Hemost. 2000. 26(6):605-18. [QxMD MEDLINE Link].

61. Takahashi H, Isobe T, Horibe S, et al. Tissue transglutaminase, coagulation factor XIII, and the pro-polypeptide of von Willebrand factor are all ligands for the integrins alpha 9beta 1 and alpha 4beta 1. J Biol Chem. 2000 Aug 4. 275(31):23589-95. [QxMD MEDLINE Link].

62. Salge U, Daubner E, Heiden M, Sietz R. Factor XIII does not stimulate growth of human cultured tumor cells. Blood Coagul Fibrinolysis. 2000 Mar. 11(2):217-8. [QxMD MEDLINE Link].

63. Molnar P, Nemes Z. Hemangiopericytoma of the cerebello-pontine angle. Diagnostic pitfalls and the diagnostic value of the subunit A of factor XIII as a tumor marker. Clin Neuropathol. 1995 Jan-Feb. 14(1):19-24. [QxMD MEDLINE Link].

64. Adany R, Bardos H, Antal M, et al. Factor XIII of blood coagulation as a nuclear crosslinking enzyme. Thromb Haemost. 2001 May. 85(5):845-51. [QxMD MEDLINE Link].

65. Kallberg Y, Gustafsson M, Persson B, Thyberg J, Johansson J. Prediction of amyloid fibril-forming proteins. J Biol Chem. 2001 Apr 20. 276(16):12945-50. [QxMD MEDLINE Link].

66. Bajzar L, Manuel R, Nesheim ME. Purification and characterization of TAFI, a thrombin-activable fibrinolysis inhibitor. J Biol Chem. 1995 Jun 16. 270(24):14477-84. [QxMD MEDLINE Link].

67. Redlitz A, Tan AK, Eaton DL, Plow EF. Plasma carboxypeptidases as regulators of the plasminogen system. J Clin Invest. 1995 Nov. 96(5):2534-8. [QxMD MEDLINE Link].

68. Bajzar L, Nesheim ME, Tracy PB. The profibrinolytic effect of activated protein C in clots formed from plasma is TAFI-dependent. Blood. 1996 Sep 15. 88(6):2093-100. [QxMD MEDLINE Link].

69. Hoffman M, Monroe DM 3rd. A cell-based model of hemostasis. Thromb Haemost. 2001 Jun. 85(6):958-65. [QxMD MEDLINE Link].

70. HGMD. Human Gene Mutation Database at the Institute of Medical Genetics in Cardiff. Available at: http://www.uwcm.ac.uk//uwcm/mg/hgmd0.html. Accessed April 9, 2002. [Full Text].

71. Dorgalaleh A, Assadollahi V, Tabibian S, Shamsizadeh M. Molecular Basis of Congenital Factor XIII Deficiency in Iran. Clin Appl Thromb Hemost. 2016 Nov 21. [QxMD MEDLINE Link].

72. Doncarli A, Demiguel V, Guseva Canu I, Goulet V, Bayart S, Calvez T, et al. FranceCoag: a 22-year prospective follow-up of the national French cohort of patients with inherited bleeding disorders. Eur J Epidemiol. 2019 May. 34 (5):521-532. [QxMD MEDLINE Link].

73. Attié-Castro FA, Zago MA, Lavinha J, et al. Ethnic heterogeneity of the factor XIII Val34Leu polymorphism. Thromb Haemost. 2000 Oct. 84(4):601-3. [QxMD MEDLINE Link].

74. FDA approves Tretten to treat rare genetic clotting disorder. U.S. Food & Drug Administration. Available at https://www.fda.gov/newsevents/newsroom/pressannouncements/ucm379696.htm. December 23, 2013; Accessed: March 4, 2017.

75. Sharief LA, Kadir RA. Congenital factor XIII deficiency in women: a systematic review of literature. Haemophilia. 2013 Aug 28. [QxMD MEDLINE Link].

76. Mikkola H, Muszbek L, Laiho E, et al. Molecular mechanism of a mild phenotype in coagulation factor XIII (FXIII) deficiency: a splicing mutation permitting partial correct splicing of FXIII A-subunit mRNA. Blood. 1997 Feb 15. 89(4):1279-87. [QxMD MEDLINE Link].

77. Greenberg DL, Davie EW. Blood coagulation factors: their complementary DNAs, genes, and expression. Coleman RW, Hirsh J, Marder VJ, et al, eds. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. Philadelphia, Pa: Lippincott Williams & Wilkins; 2001. 21-57.

78. Kangsadalampai S, Yenchitsomanus P, Chelvanayagam G, Sawasdee N, Laosombat V, Board P. Identification of a new mutation (Gly420Ser), distal to the active site, that leads to factor XIII deficiency. Eur J Haematol. 2000 Oct. 65(4):279-84. [QxMD MEDLINE Link].

79. Koseki S, Souri M, Koga S, et al. Truncated mutant B subunit for factor XIII causes its deficiency due to impaired intracellular transportation. Blood. 2001 May 1. 97(9):2667-72. [QxMD MEDLINE Link].

80. Gomez Garcia EB, Poort SR, Stibbe J. Two novel and one recurrent missense mutation in the factor XIII A gene in two Dutch patients with factor XIII deficiency. Br J Haematol. 2001 Feb. 112(2):513-8. [QxMD MEDLINE Link].

81. Anwar R, Gallivan L, Trinh C, Hill F, Markham A. Identification of a new Leu354Pro mutation responsible for factor XIII deficiency. Eur J Haematol. 2001 Feb. 66(2):133-6. [QxMD MEDLINE Link].

82. Warner D, Mansfield MW, Grant PJ. Coagulation factor XIII and cardiovascular disease in UK Asian patients undergoing coronary angiography. Thromb Haemost. 2001 Mar. 85(3):408-11. [QxMD MEDLINE Link].

83. Canavy I, Henry M, Morange PE, et al. Genetic polymorphisms and coronary artery disease in the south of France. Thromb Haemost. 2000 Feb. 83(2):212-6. [QxMD MEDLINE Link].

84. Franco RF, Middeldorp S, Meinardi JR, van Pampus EC, Reitsma PH. Factor XIII Val34Leu and the risk of venous thromboembolism in factor V Leiden carriers. Br J Haematol. 2000 Oct. 111(1):118-21. [QxMD MEDLINE Link].

85. Catto AJ, Kohler HP, Coore J, Mansfield MW, Stickland MH, Grant PJ. Association of a common polymorphism in the factor XIII gene with venous thrombosis. Blood. 1999 Feb 1. 93(3):906-8. [QxMD MEDLINE Link].

86. Kohler HP. Role of blood coagulation factor XIII in vascular diseases. Swiss Med Wkly. 2001 Jan 27. 131(3-4):31-4. [QxMD MEDLINE Link].

87. Prata MJ, Miranda C, Rocha J, Amorim A. Allelic affinities between the F13A common gene products inferred by the analysis of an (AAAG)n STR polymorphism within the 5' untranslated region. Hum Hered. 2000 May-Jun. 50(3):189-93. [QxMD MEDLINE Link].

88. Moret A, Zúñiga Á, Ayala JM, Liquori A, Cid AR, Haya S, et al. Factor XIII deficiency in two Spanish families with a novel variant in gene F13A1 detected by next-generation sequencing; symptoms and clinical management. J Thromb Thrombolysis. 2020 Feb 15. [QxMD MEDLINE Link].

89. Chandler WL, Patel MA, Gravelle L, et al. Factor XIIIA and clot strength after cardiopulmonary bypass. Blood Coagul Fibrinolysis. 2001 Mar. 12(2):101-8. [QxMD MEDLINE Link].

90. Holst FG, Hemmer CJ, Foth C, Seitz R, Egbring R, Dietrich M. Low levels of fibrin-stabilizing factor (factor XIII) in human Plasmodium falciparum malaria: correlation with clinical severity. Am J Trop Med Hyg. 1999 Jan. 60(1):99-104. [QxMD MEDLINE Link].

91. Matayoshi T, Omi T, Sakai N, Kawana S. Clinical Significance of Blood Coagulation Factor XIII Activity in Adult Henoch-Schönlein Purpura. J Nippon Med Sch. 2013. 80(4):268-78. [QxMD MEDLINE Link].

92. Lorand L, Velasco PT, Murthy SN, Lefebvre P, Green D. Autoimmune antibody in a hemorrhagic patient interacts with thrombin-activated factor XIII in a unique manner. Blood. 1999 Feb 1. 93(3):909-17. [QxMD MEDLINE Link].

93. Feinstein DI. Immune coagulation disorders. Coleman RW, Hirsh J, Marder VJ, et al, eds. Hemostasis and Thrombosis. Basic Principles and Clinical Practice. Philadelphia, Pa: Lippincott Williams & Wilkins; 2001. 1003-20.

94. Ahmad F, Solymoss S, Poon MC, Berube C, Sullivan AK. Characterization of an acquired IgG inhibitor of coagulation factor XIII in a patient with systemic lupus erythematosus. Br J Haematol. 1996 Jun. 93(3):700-3. [QxMD MEDLINE Link].

95. Tosetto A, Rodeghiero F, Gatto E, Manotti C, Poli T. An acquired hemorrhagic disorder of fibrin crosslinking due to IgG antibodies to FXIII, successfully treated with FXIII replacement and cyclophosphamide. Am J Hematol. 1995 Jan. 48(1):34-9. [QxMD MEDLINE Link].

96. Krumdieck R, Shaw DR, Huang ST, Poon MC, Rustagi PK. Hemorrhagic disorder due to an isoniazid-associated acquired factor XIII inhibitor in a patient with Waldenstrom's macroglobulinemia. Am J Med. 1991 May. 90(5):639-45. [QxMD MEDLINE Link].

97. Heinle K, Adam O, Rauh G. Factor XIII insufficiency in a patient with severe psoriasis vulgaris, arthritis, and infirmity. Clin Rheumatol. 1998. 17(4):346-8. [QxMD MEDLINE Link].

98. Lorand L. Acquired inhibitors of fibrin stabilization: a class of hemorrhagic disorders of diverse origins. Anticoagulants: Physiologic, Pathologic and Pharmacologic. CRC Press; 1994. 169-91.

99. Dorgalaleh A, Kazemi A, Zaker F, Shamsizadeh M, Rashidpanah J, Mollaei M. Laboratory Diagnosis of Factor XIII Deficiency, Routine Coagulation Tests with Quantitative and Qualitative Methods. Clin Lab. 2016. 62 (4):491-8. [QxMD MEDLINE Link].

100. Glidden PF, Malaska C, Herring SW. Thromboelastograph assay for measuring the mechanical strength of fibrin sealant clots. Clin Appl Thromb Hemost. 2000 Oct. 6(4):226-33. [QxMD MEDLINE Link].

101. Karpati L, Penke B, Katona E, Balogh I, Vamosi G, Muszbek L. A modified, optimized kinetic photometric assay for the determination of blood coagulation factor XIII activity in plasma. Clin Chem. 2000 Dec. 46(12):1946-55. [QxMD MEDLINE Link].

102. Katona E, Haramura G, Karpati L, Fachet J, Muszbek L. A simple, quick one-step ELISA assay for the determination of complex plasma factor XIII (A2B2). Thromb Haemost. 2000 Feb. 83(2):268-73. [QxMD MEDLINE Link].

103. Killick CJ, Barton CJ, Aslam S, Standen G. Prenatal diagnosis in factor XIII-A deficiency. Arch Dis Child Fetal Neonatal Ed. 1999 May. 80(3):F238-9. [QxMD MEDLINE Link]. [Full Text].

104. Orosz ZZ, Katona E, Facsko A, Berta A, Muszbek L. A highly sensitive chemiluminescence immunoassay for the measurement of coagulation factor XIII subunits and their complex in tears. J Immunol Methods. 2010 Feb 28. 353(1-2):87-92. [QxMD MEDLINE Link].

105. Brooks M. FDA Clears Tretten for Coagulation Factor XIII Deficiency. Available at http://www.medscape.com/viewarticle/818264. Accessed: January 6, 2014.

106. Inbal A, Oldenburg J, Carcao M, Rosholm A, Tehranchi R, Nugent D. Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency. Blood. 2012 May 31. 119(22):5111-7. [QxMD MEDLINE Link].

107. Gootenberg JE. Factor concentrates for the treatment of factor XIII deficiency. Curr Opin Hematol. 1998 Nov. 5(6):372-5. [QxMD MEDLINE Link].

108. Green D. Spontaneous inhibitors to coagulation factors. Clin Lab Haematol. 2000 Oct. 22 Suppl 1:21-5; discussion 30-2. [QxMD MEDLINE Link].

109. Lorand L, Losowsky MS, Miloszewski KJM. Human factor XIII: fibrin stabilizing factor. Spaet T, ed. Progress in Hemostasis and Thrombosis. New York, NY: Grune & Stratton; 1980. Vol 5: 245-90.

110. Abbondanzo SL, Gootenberg JE, Lofts RS, McPherson RA. Intracranial hemorrhage in congenital deficiency of factor XIII. Am J Pediatr Hematol Oncol. 1988 Spring. 10(1):65-8. [QxMD MEDLINE Link].

111. Green D, Sanders J, Wong C, et al. Coronary revascularization in the presence of an inhibitory antibody to factor XIII. Bull Intensive Crit Care. 1996. 3(3):14-6.

112. Wiel E, Marciniak B, Wibaut B. [Recurrent hematomas and normal standard hemostasis tests]. Ann Fr Anesth Reanim. 1998. 17(1):61-4. [QxMD MEDLINE Link].

113. Kawamura A, Tamaki N, Yonezawa K, Nakamura M, Asada M. [Effect of factor XIII on intractable CSF leakage after a transpetrosal-approach operation: a case report]. No Shinkei Geka. 1997 Jan. 25(1):53-6. [QxMD MEDLINE Link].

114. Chamouard P, Grunebaum L, Wiesel ML, et al. Significance of diminished factor XIII in Crohn's disease. Am J Gastroenterol. 1998 Apr. 93(4):610-4. [QxMD MEDLINE Link].

115. Linskens RK, van Bodegraven AA, Schoorl M, Tuynman HA, Bartels P. Predictive value of inflammatory and coagulation parameters in the course of severe ulcerative colitis. Dig Dis Sci. 2001 Mar. 46(3):644-8. [QxMD MEDLINE Link].

116. Helio T, Wartiovaara U, Halme L, et al. Arg506Gln factor V mutation and Val34Leu factor XIII polymorphism in Finnish patients with inflammatory bowel disease. Scand J Gastroenterol. 1999 Feb. 34(2):170-4. [QxMD MEDLINE Link].

117. Burrows RF, Ray JG, Burrows EA. Bleeding risk and reproductive capacity among patients with factor XIII deficiency: a case presentation and review of the literature. Obstet Gynecol Surv. 2000 Feb. 55(2):103-8. [QxMD MEDLINE Link].

118. Kreilgaard L, Jones LS, Randolph TW, et al. Effect of Tween 20 on freeze-thawing- and agitation-induced aggregation of recombinant human factor XIII. J Pharm Sci. 1998 Dec. 87(12):1597-603. [QxMD MEDLINE Link].

119. Kreilgaard L, Frokjaer S, Flink JM, Randolph TW, Carpenter JF. Effects of additives on the stability of recombinant human factor XIII during freeze-drying and storage in the dried solid. Arch Biochem Biophys. 1998 Dec 1. 360(1):121-34. [QxMD MEDLINE Link].

120. Becker PS. Gene Therapy for Blood Disorders. The Hematologist: ASH News and Reports. Available at http://www.hematology.org/Thehematologist/Years-Best/3601.aspx. January 13, 2015; Accessed: March 4, 2017.

121. Dickneite G, Metzner H, Nicolay U. Prevention of suture hole bleeding using fibrin sealant: benefits of factor XIII. J Surg Res. 2000 Oct. 93(2):201-5. [QxMD MEDLINE Link].

122. ARC. PLAS+SD (pooled plasma, solvent-detergent treated). Monograph by the American Red Cross and V. I. Technologies, Inc. 1999. VIT-001A9/99:

123. MediView Express. Recombinant therapy enhances safety and quality of life for hemophilia patients. Paper presented at: 53rd Annual Meeting of the National Hemophilia Foundation. November 16, 2001: Nashville, Tennessee.

124. Rigas B, Hasan I, Rehman R, Donahue P, Wittkowski KM, Lebovics E. Effect on treatment outcome of coinfection with SEN viruses in patients with hepatitis C. Lancet. 2001 Dec 8. 358(9297):1961-2. [QxMD MEDLINE Link].

125. Azzi A, De Santis R, Morfini M, et al. TT virus contaminates first-generation recombinant factor VIII concentrates. Blood. 2001 Oct 15. 98(8):2571-3. [QxMD MEDLINE Link].

126. Bachmann F. Plasminogen-plasmin enzyme system. Colman RW, Hirsh J, George JN, et al, eds. Hemostasis and Thrombosis: Basic Principles and Clinical Practice. 4th ed. Lippincott Williams & Wilkins; 2001. 275-320.

127. Fergusson DA, Hebert PC, Mazer CD, et al. A comparison of aprotinin and lysine analogues in high-risk cardiac surgery. N Engl J Med. 2008 May 29. 358(22):2319-31. [QxMD MEDLINE Link].

128. Di Bisceglie AM. SEN and sensibility: interactions between newly discovered and other hepatitis viruses?. Lancet. 2001 Dec 8. 358(9297):1925-6. [QxMD MEDLINE Link].

129. Treisman GJ, Angelino AF, Hutton HE. Psychiatric issues in the management of patients with HIV infection. JAMA. 2001 Dec 12. 286(22):2857-64. [QxMD MEDLINE Link].

130. Carcao M, Altisent C, Castaman G, Fukutake K, Kerlin BA, Kessler C, et al. Recombinant FXIII (rFXIII-A2) Prophylaxis Prevents Bleeding and Allows for Surgery in Patients with Congenital FXIII A-Subunit Deficiency. Thromb Haemost. 2018 Mar. 118 (3):451-460. [QxMD MEDLINE Link]. [Full Text].

131. Urwin PJ, Mackenzie JM, Llewelyn CA, Will RG, Hewitt PE. Creutzfeldt-Jakob disease and blood transfusion: updated results of the UK Transfusion Medicine Epidemiology Review Study. Vox Sang. 2016 May. 110 (4):310-6. [QxMD MEDLINE Link].

132. Jackson GS, Burk-Rafel J, Edgeworth JA, Sicilia A, Abdilahi S, Korteweg J, et al. Population screening for variant Creutzfeldt-Jakob disease using a novel blood test: diagnostic accuracy and feasibility study. JAMA Neurol. 2014 Apr. 71 (4):421-8. [QxMD MEDLINE Link].

133. Ivaskevicius V, Biswas A, Loreth R, et al. Mutations affecting disulphide bonds contribute to a fairly common prevalence of F13B gene defects: results of a genetic study in 14 families with factor XIII B deficiency. Haemophilia. 2010 Mar 10. [QxMD MEDLINE Link].

134. Inbal A, Oldenburg J, Carcao M, Rosholm A, Tehranchi R, Nugent D. Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency. Blood. 2012 May 31. 119(22):5111-7. [QxMD MEDLINE Link].

135. ARC. FDA-approved product circular for Pooled Plasma, Solvent-Detergent-Treated (PLAS+SD) manufactured by the American Red Cross and V.I. Technologies, Inc. 2000.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Elzbieta Klujszo, MD Head of Department of Dermatology, Wojewodzki Szpital Zespolony, Kielce

Disclosure: Nothing to disclose.

Pere Gascon, MD, PhD Professor and Senior Consultant, Division of Medical Oncology, Institute of Hematology and Medical Oncology, Hospital Clinic; Director, Laboratory of Molecular and Translational Oncology, University of Barcelona Faculty of Biology, Spain

Pere Gascon, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, New York Academy of Medicine, New York Academy of Sciences, Royal College of Surgeons of Edinburgh, Royal European Academy of Doctors, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ronald A Sacher, MD, FRCPC, DTM&H Professor Emeritus of Internal Medicine and Hematology/Oncology, Emeritus Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MD, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Perumal Thiagarajan, MD Professor, Department of Pathology and Medicine, Baylor College of Medicine; Director, Transfusion Medicine and Hematology Laboratory, Michael E DeBakey Veterans Affairs Medical Center

Perumal Thiagarajan, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society for Biochemistry and Molecular Biology, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Royal College of Physicians

Disclosure: Nothing to disclose.

Paul Schick, MD † Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

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