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An Acquired Inhibitor to Factor XIII

An Acquired Inhibitor to Factor XIII Abstract Factor XIII (fibrin stabilizing factor [FSF]) is a coagulation factor necessary for the formation of stable fibrin clots. In the presence of calcium ions1,2 and thrombin,3 factor XIII promotes the formation of ε(γglutamyl)lysine covalent bonds between fibrin monomers.4,5 Such bonds render fibrin clots stable in 5M urea or dilute solutions of monochloroacetic acid. The first patient with a congenital deficiency of factor XIII was described by Duckert et al in 1960.6 Subsequently, many other cases have been reported.7 Absence of factor XIII may result in severe bleeding. Umbilical cord bleeding is usually the earliest manifestation and, characteristically, patients bruise easily and experience excessive bleeding following trauma. Wound healing may be delayed, and disfiguring scar formation has been reported.6 Hemarthroses also occur but are rare.8 Diminished factor XIII activity has been reported in patients with liver disease, leukemia, lymphoma, and some other diseases.9-11 References 1. Robbins KC: A study on the conversion of fibrinogen to fibrin. Amer J Physiol 142:581-588, 1944. 2. Laki K, Lorand L: On the solubility of fibrin clots. Science 108:280, 1948.Crossref 3. Lorand L, Konishi K: Activation of fibrin stabilizing factor of plasma by thrombin. Arch Biochem 105:58-67, 1954.Crossref 4. Pisano JJ, Finlayson JS, Peyton MP: Cross-link in fibrin polymerized by factor XIII: ε(γglutamyl)lysine. Science 160:892-893, 1968.Crossref 5. Matac̈ić S, Loewy AG: The identification of isopeptide cross-links in insoluble fibrin. Biochem Biophys Res Commun 30:356-362, 1968.Crossref 6. Duckert F, Jung E, Shmerling DH: A hitherto undescribed congenital hemorrhagic diathesis probably due to fibrin-stabilizing factor deficiency. Thromb Diath Haemorrh 5:179-186, 1960. 7. Ratnoff OD, Steinberg AG: Inheritance of fibrin-stabilizing factor deficiency. Lancet 1:25-26, 1968.Crossref 8. Britten AFH: Congenital deficiency of factor XIII (fibrin stabilizing factor). Amer J Med 43:751-761, 1967.Crossref 9. Nussbaum M, Morse BS: Plasma fibrin stabilizing factor activity in various diseases. Blood 23:669-678, 1964. 10. Mandel EE, Gerhold WM: Plasma fibrin stabilizing factor: acquired deficiency in various diseases. Amer J Clin Path 52:547-556, 1969. 11. Walls WD, Losowsky MS: Plasma fibrin stabilizing factor activity in normal subjects and patients with chronic liver disease. Thromb Diath Haemorrh 21:134-143, 1969. 12. Lorand L, Urayama T, deKiewiet JWC, et al: Diagnostic and genetic studies on fibrin-stabilizing factor with a new assay based on amine incorporation. J Clin Invest 48:1054-1064, 1969.Crossref 13. Lewis JH, Szeto ILF, Ellis LD, et al: An acquired inhibitor to coagulation factor XIII. Johns Hopkins Med J 120:401-407, 1967. 14. Godal HC: An inhibitor to fibrin-stabilizing factor (FSF, factor XIII). Scand J Haemat 7:43-48, 1970.Crossref 15. Lorand L, Jacobsen A, Brunner-Lorand J: A pathologic inhibitor of fibrin cross-linking. J Clin Invest 47:268-273, 1968.Crossref 16. Moretz WH, Voyles WR, Thomas CB: Value of physiologic amputation. Ann Surg 154:851-858, 1961.Crossref 17. Still JM, Wray CH, Moretz WH: Selective physiologic amputation: A valuable adjunct in preparation for surgical operation. Ann Surg 171:143-151, 1970.Crossref 18. Quick AJ, Stanley-Brown M, Bancroft FW: A study of the coagulation defect in hemophilia and in jaundice. Amer J Med Sci 190:501-511, 1935.Crossref 19. Langdell RD, Wagner RH, Brinkhous KM: Effect of antihemophilic factor on one-stage clotting tests. J Lab Clin Med 41:637-647, 1953. 20. Ingram GIC, Norris RP: Acute coagulation disorders at partuition. J Obstet Gynaec Brit Commun 67:367-383, 1960.Crossref 21. Biggs R, Douglas AS: The thromboplastin generation test. J Clin Path 6:23-29, 1953.Crossref 22. Blix S: Studies on the fibrinolytic system in the euglobulin fraction of human plasma. Scand J Clin Lab Invest 13( (suppl 58) ):3-19, 1961. 23. Ivy AC, Shapiro PF, Melnick P: The bleeding tendency in jaundice. Surg Gynec Obstet 60:781-784, 1935. 24. Lee RI, White PD: A clinical study of the coagulation time of the blood. Amer J Med Sci 145:495-503, 1913.Crossref 25. Webster WP, Roberts HR, Penick GD: Hemostasis in factor V deficiency. Amer J Med Sci 248:194-202, 1964.Crossref 26. Karpatkin S, Ingram GIC, Graham JB: Severe isolated prothrombin deficiency: An acquired state with complete recovery. Thromb Diath Haemorrh 8:222-234, 1962. 27. Barrow EM, Bullock WR, Graham JB: A study of the carrier state for plasma thromboplastin antecedent (PTC, Christmas factor) deficiency, utilizing a new assay procedure. J Lab Clin Med 55:936-945, 1960. 28. Hougie C, Barrow EM, Graham JB: Stuart clotting defect: I. Segregation of an hereditary hemorrhagic state from the heterogenous group heretofore called stable factor (SPCA, proconvertin, factor VII deficiency). J Clin Invest 36:485-496, 1957.Crossref 29. Roberts HR, Scales MB, Madison JT, et al: A clinical and experimental study of acquired inhibitors to factor VIII. Blood 26:805-818, 1965. 30. Jones JF, Smith HP: The fibrinogen level in hepatectomized dogs and an outline of a method for the quantitative determination of fibrin. Amer J Physiol 94:144-161, 1930. 31. Smith HP, Warner ED, Brinkhous KM: Prothrombin deficiency and the bleeding tendency in liver injury (chloroform intoxication). J Exp Med 66:801-811, 1937.Crossref 32. McDonagh J, McDonagh RP, Delâge JM, et al: Factor XIII in human plasma and platelets. J Clin Invest 48:940-946, 1969.Crossref 33. Barry A, Delâge JM: Congenital deficiency of fibrin stabilizing factor. New Eng J Med 272:943-946, 1965.Crossref 34. Marshall FN, Massad EN: Interference with fibrin stabilization by polymyxin B sulfate. Proc Soc Exp Biol Med 125:420-423, 1967.Crossref 35. Lorand L, Rule NG, Ong HH, et al: Amine specificity in transpeptidation: Inhibitor of fibrin cross-linking. Biochemistry 7:1214-1223, 1968.Crossref 36. Roberts HR, Gross GP, Webster WP, et al: Acquired inhibitors of plasma factor IX: A study of their induction properties and neutralization. Amer J Med Sci 251:43-50, 1966.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1972 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1972.03650050090019
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Abstract

Abstract Factor XIII (fibrin stabilizing factor [FSF]) is a coagulation factor necessary for the formation of stable fibrin clots. In the presence of calcium ions1,2 and thrombin,3 factor XIII promotes the formation of ε(γglutamyl)lysine covalent bonds between fibrin monomers.4,5 Such bonds render fibrin clots stable in 5M urea or dilute solutions of monochloroacetic acid. The first patient with a congenital deficiency of factor XIII was described by Duckert et al in 1960.6 Subsequently, many other cases have been reported.7 Absence of factor XIII may result in severe bleeding. Umbilical cord bleeding is usually the earliest manifestation and, characteristically, patients bruise easily and experience excessive bleeding following trauma. Wound healing may be delayed, and disfiguring scar formation has been reported.6 Hemarthroses also occur but are rare.8 Diminished factor XIII activity has been reported in patients with liver disease, leukemia, lymphoma, and some other diseases.9-11 References 1. Robbins KC: A study on the conversion of fibrinogen to fibrin. Amer J Physiol 142:581-588, 1944. 2. Laki K, Lorand L: On the solubility of fibrin clots. Science 108:280, 1948.Crossref 3. Lorand L, Konishi K: Activation of fibrin stabilizing factor of plasma by thrombin. Arch Biochem 105:58-67, 1954.Crossref 4. Pisano JJ, Finlayson JS, Peyton MP: Cross-link in fibrin polymerized by factor XIII: ε(γglutamyl)lysine. Science 160:892-893, 1968.Crossref 5. Matac̈ić S, Loewy AG: The identification of isopeptide cross-links in insoluble fibrin. Biochem Biophys Res Commun 30:356-362, 1968.Crossref 6. Duckert F, Jung E, Shmerling DH: A hitherto undescribed congenital hemorrhagic diathesis probably due to fibrin-stabilizing factor deficiency. Thromb Diath Haemorrh 5:179-186, 1960. 7. Ratnoff OD, Steinberg AG: Inheritance of fibrin-stabilizing factor deficiency. Lancet 1:25-26, 1968.Crossref 8. Britten AFH: Congenital deficiency of factor XIII (fibrin stabilizing factor). Amer J Med 43:751-761, 1967.Crossref 9. Nussbaum M, Morse BS: Plasma fibrin stabilizing factor activity in various diseases. Blood 23:669-678, 1964. 10. Mandel EE, Gerhold WM: Plasma fibrin stabilizing factor: acquired deficiency in various diseases. Amer J Clin Path 52:547-556, 1969. 11. Walls WD, Losowsky MS: Plasma fibrin stabilizing factor activity in normal subjects and patients with chronic liver disease. Thromb Diath Haemorrh 21:134-143, 1969. 12. Lorand L, Urayama T, deKiewiet JWC, et al: Diagnostic and genetic studies on fibrin-stabilizing factor with a new assay based on amine incorporation. J Clin Invest 48:1054-1064, 1969.Crossref 13. Lewis JH, Szeto ILF, Ellis LD, et al: An acquired inhibitor to coagulation factor XIII. Johns Hopkins Med J 120:401-407, 1967. 14. Godal HC: An inhibitor to fibrin-stabilizing factor (FSF, factor XIII). Scand J Haemat 7:43-48, 1970.Crossref 15. Lorand L, Jacobsen A, Brunner-Lorand J: A pathologic inhibitor of fibrin cross-linking. J Clin Invest 47:268-273, 1968.Crossref 16. Moretz WH, Voyles WR, Thomas CB: Value of physiologic amputation. Ann Surg 154:851-858, 1961.Crossref 17. Still JM, Wray CH, Moretz WH: Selective physiologic amputation: A valuable adjunct in preparation for surgical operation. Ann Surg 171:143-151, 1970.Crossref 18. Quick AJ, Stanley-Brown M, Bancroft FW: A study of the coagulation defect in hemophilia and in jaundice. Amer J Med Sci 190:501-511, 1935.Crossref 19. Langdell RD, Wagner RH, Brinkhous KM: Effect of antihemophilic factor on one-stage clotting tests. J Lab Clin Med 41:637-647, 1953. 20. Ingram GIC, Norris RP: Acute coagulation disorders at partuition. J Obstet Gynaec Brit Commun 67:367-383, 1960.Crossref 21. Biggs R, Douglas AS: The thromboplastin generation test. J Clin Path 6:23-29, 1953.Crossref 22. Blix S: Studies on the fibrinolytic system in the euglobulin fraction of human plasma. Scand J Clin Lab Invest 13( (suppl 58) ):3-19, 1961. 23. Ivy AC, Shapiro PF, Melnick P: The bleeding tendency in jaundice. Surg Gynec Obstet 60:781-784, 1935. 24. Lee RI, White PD: A clinical study of the coagulation time of the blood. Amer J Med Sci 145:495-503, 1913.Crossref 25. Webster WP, Roberts HR, Penick GD: Hemostasis in factor V deficiency. Amer J Med Sci 248:194-202, 1964.Crossref 26. Karpatkin S, Ingram GIC, Graham JB: Severe isolated prothrombin deficiency: An acquired state with complete recovery. Thromb Diath Haemorrh 8:222-234, 1962. 27. Barrow EM, Bullock WR, Graham JB: A study of the carrier state for plasma thromboplastin antecedent (PTC, Christmas factor) deficiency, utilizing a new assay procedure. J Lab Clin Med 55:936-945, 1960. 28. Hougie C, Barrow EM, Graham JB: Stuart clotting defect: I. Segregation of an hereditary hemorrhagic state from the heterogenous group heretofore called stable factor (SPCA, proconvertin, factor VII deficiency). J Clin Invest 36:485-496, 1957.Crossref 29. Roberts HR, Scales MB, Madison JT, et al: A clinical and experimental study of acquired inhibitors to factor VIII. Blood 26:805-818, 1965. 30. Jones JF, Smith HP: The fibrinogen level in hepatectomized dogs and an outline of a method for the quantitative determination of fibrin. Amer J Physiol 94:144-161, 1930. 31. Smith HP, Warner ED, Brinkhous KM: Prothrombin deficiency and the bleeding tendency in liver injury (chloroform intoxication). J Exp Med 66:801-811, 1937.Crossref 32. McDonagh J, McDonagh RP, Delâge JM, et al: Factor XIII in human plasma and platelets. J Clin Invest 48:940-946, 1969.Crossref 33. Barry A, Delâge JM: Congenital deficiency of fibrin stabilizing factor. New Eng J Med 272:943-946, 1965.Crossref 34. Marshall FN, Massad EN: Interference with fibrin stabilization by polymyxin B sulfate. Proc Soc Exp Biol Med 125:420-423, 1967.Crossref 35. Lorand L, Rule NG, Ong HH, et al: Amine specificity in transpeptidation: Inhibitor of fibrin cross-linking. Biochemistry 7:1214-1223, 1968.Crossref 36. Roberts HR, Gross GP, Webster WP, et al: Acquired inhibitors of plasma factor IX: A study of their induction properties and neutralization. Amer J Med Sci 251:43-50, 1966.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Nov 1, 1972

References