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CONGENITAL HYPOPROTHROMBINEMIC STATES

CONGENITAL HYPOPROTHROMBINEMIC STATES Abstract THE DISCOVERY of a case of congenital hypoprothrombinemia in 1941 by Rhoads and Fitz-Hugh 1 marked the beginning of an important advance in the study of hemorrhagic diseases. This case had been diagnosed and regarded as hemophilia for nine years. Only after the prothrombin-time test became available was it possible to differentiate such a condition readily from hemophilia. From the early studies * it became evident that cases of this type were congenital and likely familial. All were tacitly accepted as true hypoprothrombinemia. The discovery of the labile factor (proaccelerin, Factor V, or ac-globulin) in 1943 clearly demonstrated that the prothrombin time could be prolonged by a deficiency of this new factor, and the prediction that such a lack might cause a hemorrhagic disease 9 was borne out when, less than two years later, Owren 10 reported a patient with a bleeding tendency who lacked this agent. Shortly thereafter, members of References 1. References 2-8. 2. References 11 and 12. 3. References 13 and 14. 4. References 25-28. 5. References 29-31. 6. Rhoads, J. E., and Fitz-Hugh, T., Jr.: Idiopathic Hypoprothrombinemia—An Apparently Unrecorded Condition , Am. J. M. Sc. 202:662, 1941.Crossref 7. Giordano, A. S.: Idiopathic Hypoprothrombinemia , Am. J. Clin. Path. 13:285, 1943. 8. Plum, P.: Idiopathic Hypoprothrombinemia Refractory to Vitamin K , Acta med. scandinav. 113:262, 1943.Crossref 9. Murphy, F. D., and Clark, J. K.: Idiopathic Hypoprothrombinemia , Am. J. M. Sc. 207:77, 1944.Crossref 10. de Marval, L., and Bomchil, G.: Hipoprotombinemia idiopática , Semana méd. 1:1088, 1944. 11. de Marval, L.: Hipoprotombinemia idiopática , Semana méd. 1:476, 1945. 12. Hauser, F.: Familiäre, Vitamin-K-resistente Hypoprothrombinämie , Ann. pædiat. 165:142, 1945. 13. Lewis, J. H., and Bennett, I. J., Jr.: Clinical Hypoprothrombinemia: A Study of Factors A and B of Prothrombin , J. Clin. Invest. 26:1187, 1947. 14. Quick, A. J.: On the Constitution of Prothrombin , Am. J. Physiol. 140:212, 1943. 15. Owren, P. A.: Parahaemophilia: Haemorrhagic Diathesis Due to Absence of a Previously Unknown Clotting Factor , Lancet 1:446, 1947.Crossref 16. Quick, A. J.: Effect of Synthetic Vitamin K and of Quinine Sulfate on the Prothrombin Level , J. Lab. & Clin. Med. 31:79, 1946. 17. Quick, A. J.: Congenital Hypoprothrombinaemia and Pseudo-Hypoprothrombinaemia , Lancet 2:379, 1947. 18. Alexander, B.; deVries, A.; Goldstein, R., and Landwehr, G.: Prothrombin Conversion Accelerator in Serum , Science 109:545, 1949. 19. Alexander, B.; Goldstein, R.; Landwehr, G., and Cook, C. D.: Congenital SPCA Deficiency: A Hitherto Unrecognized Coagulation Defect with Hemorrhage Rectified by Serum and Serum Fractions , J. Clin. Invest. 30:596, 1951. 20. Jacox, R. F.: Studies on the Activation of a Serum "Prothrombin-Conversion Factor," J. Clin. Invest. 28:492, 1949. 21. Koller, F.; Loeliger, A., and Duckert, F.: Experiments on a New Clotting Factor (Factor VII) , Acta haemat. 6:1, 1951. 22. Owren, P. A.: Le complexe activateur de la prothrombine at son intérêt clinique , Rev. hémat. 6:135, 1951. 23. Mann, F. D.: Co-Thromboplastin , Am. J. Clin. Path. 19:861, 1949. 24. Quick, A. J.: The Physiology and Pathology of Hemostasis , Philadelphia, Lea & Febiger, 1951. 25. Quick, A. J.; Murat, L. G.; Hussey, C. V., and Burgess, G. F.: The Prothrombin in the Newborn , Surg., Gynec. & Obst. 95:671, 1952. 26. Duckert, F.; Koller, F., and Matter, M.: Purification and Physiological Properties of Factor VII from Plasma and Serum: Separation for Prothrombin , Proc. Soc. Exper. Biol. & Med. 82:259, 1953. 27. Hagen, P. S., and Watson, C. J.: Idiopathic (Familial) Hypoprothrombinemia , J. Lab. & Clin. Med. 33:542, 1948. 28. Frick, P. G., and Hagen, P. S.: Congenital Familial Deficiency of the Stable Prothrombin Conversion Factor: Restudy of Case Originally Reported as "Idiopathic Hypoprothrombinemia," J. Lab. & Clin. Med. 42:212, 1953. 29. Alexander, B., in Blood Clotting and Allied Problems, Transactions of the Fifth Conference, edited by J. E. Flynn, New York, Josiah Macy, Jr., Foundation, 1952, p. 119. 30. Quick, A. J., and Stefanini, M.: The State of Component A (Prothrombin) in Human Blood: Evidence That It Is Partly Free and Partly in an Inactive or Precursor Form , J. Lab. & Clin. Med. 34:1203, 1949. 31. Quick, A. J., and Hussey, C. V.: Influence of Concentration of Thromboplastin on Prothrombin Time of Human and Dog Plasma , Proc. Soc. Exper. Biol. & Med. 76:732, 1951. 32. Quick, A. J., and Hussey, C. V.: On the Comparative Prothrombin Activity of Human and Dog Blood , J. Clin. Invest. 30:903, 1951. 33. Quick, A. J., and Hussey, C. V.: On the Interpretation of the One-Stage Method for Determining Prothrombin Time , New England J. Med. 248:624, 1953. 34. Quick, A. J.: The Coagulation Defect in Sweet Clover Disease and in the Hemorrhagic Chick Disease of Dietary Origin: A Consideration of the Source of Prothrombin , Am. J. Physiol. 118:260, 1937. 35. Stefanini, M., and Pisciotta, A. V.: Rate of Disappearance of Prothrombin from the Circulation , Science 111:364, 1950. 36. Landwehr, G.; Lang, H., and Alexander, B.: Congenital Hypoprothrombinemia , Am. J. Med. 8:255, 1950. 37. Quick, A. J.; Hussey, C. V., and Collentine, G. E., Jr.: Vitamin K Requirements of Adult Dogs and the Influence of Bile on Its Absorption from the Intestine , Am. J. Physiol. 176:239, 1954. 38. Quick, A. J.; Flood, F. T., and Hussey, C. V.: Classification of the Hypoprothrombinemias , Am. J. Clin. Path. 23:951, 1953. 39. Quick, A. J.: The Nature of the Bleeding in Jaundice , J. A. M. A. 110:1658, 1938. 40. Owren, P. A.: Prothrombin and Accessory Factors , Am. J. Med. 14:201, 1953. 41. Desjardins, R. A., and Droulard, K. E.: Idiopathic Hypoprothrombinemia , J. Indiana M. A. 45:1153, 1952. 42. Brink, A. J., and Kingsley, C. S.: A Familial Disorder of Blood Coagulation Due to Deficiency of the Labile Factor , Quart. J. Med. 21:19, 1952. 43. Alexander, B., and Goldstein, R.: Parahemophilia in 3 Siblings (Owren's Disease) , Am. J. Med. 8:255, 1952. 44. Crockett, C. L., Jr.; Shotton, D.; Craddock, C. G., and Leavell, B. S.: Hypoprothrombinemia: Studies of a Case of the Idiopathic Type and the Effect of Serum Administration , Blood 4:1293, 1949. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Internal Medicine American Medical Association

CONGENITAL HYPOPROTHROMBINEMIC STATES

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References (41)

Publisher
American Medical Association
Copyright
Copyright © 1955 American Medical Association. All Rights Reserved.
ISSN
0888-2479
DOI
10.1001/archinte.1955.00250070018002
Publisher site
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Abstract

Abstract THE DISCOVERY of a case of congenital hypoprothrombinemia in 1941 by Rhoads and Fitz-Hugh 1 marked the beginning of an important advance in the study of hemorrhagic diseases. This case had been diagnosed and regarded as hemophilia for nine years. Only after the prothrombin-time test became available was it possible to differentiate such a condition readily from hemophilia. From the early studies * it became evident that cases of this type were congenital and likely familial. All were tacitly accepted as true hypoprothrombinemia. The discovery of the labile factor (proaccelerin, Factor V, or ac-globulin) in 1943 clearly demonstrated that the prothrombin time could be prolonged by a deficiency of this new factor, and the prediction that such a lack might cause a hemorrhagic disease 9 was borne out when, less than two years later, Owren 10 reported a patient with a bleeding tendency who lacked this agent. Shortly thereafter, members of References 1. References 2-8. 2. References 11 and 12. 3. References 13 and 14. 4. References 25-28. 5. References 29-31. 6. Rhoads, J. E., and Fitz-Hugh, T., Jr.: Idiopathic Hypoprothrombinemia—An Apparently Unrecorded Condition , Am. J. M. Sc. 202:662, 1941.Crossref 7. Giordano, A. S.: Idiopathic Hypoprothrombinemia , Am. J. Clin. Path. 13:285, 1943. 8. Plum, P.: Idiopathic Hypoprothrombinemia Refractory to Vitamin K , Acta med. scandinav. 113:262, 1943.Crossref 9. Murphy, F. D., and Clark, J. K.: Idiopathic Hypoprothrombinemia , Am. J. M. Sc. 207:77, 1944.Crossref 10. de Marval, L., and Bomchil, G.: Hipoprotombinemia idiopática , Semana méd. 1:1088, 1944. 11. de Marval, L.: Hipoprotombinemia idiopática , Semana méd. 1:476, 1945. 12. Hauser, F.: Familiäre, Vitamin-K-resistente Hypoprothrombinämie , Ann. pædiat. 165:142, 1945. 13. Lewis, J. H., and Bennett, I. J., Jr.: Clinical Hypoprothrombinemia: A Study of Factors A and B of Prothrombin , J. Clin. Invest. 26:1187, 1947. 14. Quick, A. J.: On the Constitution of Prothrombin , Am. J. Physiol. 140:212, 1943. 15. Owren, P. A.: Parahaemophilia: Haemorrhagic Diathesis Due to Absence of a Previously Unknown Clotting Factor , Lancet 1:446, 1947.Crossref 16. Quick, A. J.: Effect of Synthetic Vitamin K and of Quinine Sulfate on the Prothrombin Level , J. Lab. & Clin. Med. 31:79, 1946. 17. Quick, A. J.: Congenital Hypoprothrombinaemia and Pseudo-Hypoprothrombinaemia , Lancet 2:379, 1947. 18. Alexander, B.; deVries, A.; Goldstein, R., and Landwehr, G.: Prothrombin Conversion Accelerator in Serum , Science 109:545, 1949. 19. Alexander, B.; Goldstein, R.; Landwehr, G., and Cook, C. D.: Congenital SPCA Deficiency: A Hitherto Unrecognized Coagulation Defect with Hemorrhage Rectified by Serum and Serum Fractions , J. Clin. Invest. 30:596, 1951. 20. Jacox, R. F.: Studies on the Activation of a Serum "Prothrombin-Conversion Factor," J. Clin. Invest. 28:492, 1949. 21. Koller, F.; Loeliger, A., and Duckert, F.: Experiments on a New Clotting Factor (Factor VII) , Acta haemat. 6:1, 1951. 22. Owren, P. A.: Le complexe activateur de la prothrombine at son intérêt clinique , Rev. hémat. 6:135, 1951. 23. Mann, F. D.: Co-Thromboplastin , Am. J. Clin. Path. 19:861, 1949. 24. Quick, A. J.: The Physiology and Pathology of Hemostasis , Philadelphia, Lea & Febiger, 1951. 25. Quick, A. J.; Murat, L. G.; Hussey, C. V., and Burgess, G. F.: The Prothrombin in the Newborn , Surg., Gynec. & Obst. 95:671, 1952. 26. Duckert, F.; Koller, F., and Matter, M.: Purification and Physiological Properties of Factor VII from Plasma and Serum: Separation for Prothrombin , Proc. Soc. Exper. Biol. & Med. 82:259, 1953. 27. Hagen, P. S., and Watson, C. J.: Idiopathic (Familial) Hypoprothrombinemia , J. Lab. & Clin. Med. 33:542, 1948. 28. Frick, P. G., and Hagen, P. S.: Congenital Familial Deficiency of the Stable Prothrombin Conversion Factor: Restudy of Case Originally Reported as "Idiopathic Hypoprothrombinemia," J. Lab. & Clin. Med. 42:212, 1953. 29. Alexander, B., in Blood Clotting and Allied Problems, Transactions of the Fifth Conference, edited by J. E. Flynn, New York, Josiah Macy, Jr., Foundation, 1952, p. 119. 30. Quick, A. J., and Stefanini, M.: The State of Component A (Prothrombin) in Human Blood: Evidence That It Is Partly Free and Partly in an Inactive or Precursor Form , J. Lab. & Clin. Med. 34:1203, 1949. 31. Quick, A. J., and Hussey, C. V.: Influence of Concentration of Thromboplastin on Prothrombin Time of Human and Dog Plasma , Proc. Soc. Exper. Biol. & Med. 76:732, 1951. 32. Quick, A. J., and Hussey, C. V.: On the Comparative Prothrombin Activity of Human and Dog Blood , J. Clin. Invest. 30:903, 1951. 33. Quick, A. J., and Hussey, C. V.: On the Interpretation of the One-Stage Method for Determining Prothrombin Time , New England J. Med. 248:624, 1953. 34. Quick, A. J.: The Coagulation Defect in Sweet Clover Disease and in the Hemorrhagic Chick Disease of Dietary Origin: A Consideration of the Source of Prothrombin , Am. J. Physiol. 118:260, 1937. 35. Stefanini, M., and Pisciotta, A. V.: Rate of Disappearance of Prothrombin from the Circulation , Science 111:364, 1950. 36. Landwehr, G.; Lang, H., and Alexander, B.: Congenital Hypoprothrombinemia , Am. J. Med. 8:255, 1950. 37. Quick, A. J.; Hussey, C. V., and Collentine, G. E., Jr.: Vitamin K Requirements of Adult Dogs and the Influence of Bile on Its Absorption from the Intestine , Am. J. Physiol. 176:239, 1954. 38. Quick, A. J.; Flood, F. T., and Hussey, C. V.: Classification of the Hypoprothrombinemias , Am. J. Clin. Path. 23:951, 1953. 39. Quick, A. J.: The Nature of the Bleeding in Jaundice , J. A. M. A. 110:1658, 1938. 40. Owren, P. A.: Prothrombin and Accessory Factors , Am. J. Med. 14:201, 1953. 41. Desjardins, R. A., and Droulard, K. E.: Idiopathic Hypoprothrombinemia , J. Indiana M. A. 45:1153, 1952. 42. Brink, A. J., and Kingsley, C. S.: A Familial Disorder of Blood Coagulation Due to Deficiency of the Labile Factor , Quart. J. Med. 21:19, 1952. 43. Alexander, B., and Goldstein, R.: Parahemophilia in 3 Siblings (Owren's Disease) , Am. J. Med. 8:255, 1952. 44. Crockett, C. L., Jr.; Shotton, D.; Craddock, C. G., and Leavell, B. S.: Hypoprothrombinemia: Studies of a Case of the Idiopathic Type and the Effect of Serum Administration , Blood 4:1293, 1949.

Journal

A.M.A. Archives of Internal Medicine – American Medical Association

Published: Jan 1, 1955

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