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Hämophilie

Chapter
Part of the Handbuch der Inneren Medizin book series (INNEREN, volume 2 / 9)

Zusammenfassung

Die Hämophilie ist die häufigste angeborene hämorrhagische Diathese. Die Inzidenz dürfte generell bei 100/106 Männern für die Hämophilie A und 25/106 Männer für die Hämophilie B liegen (Aledort u.Goodnight 1981). Einzelne Untersucher fanden jedoch Abweichungen nach oben und unten. So schätzteBiggs (1977) die Häufigkeit der Hämophilie auf 50/106 Geburten.Stevenson u.Kerr (1967) nahmen eine Inzidenz von 100–120 Patienten mit Hämophilie A und 20–30 Patienten mit Hämophilie B auf 106 Geburten an. In einer amerikanischen Studie (National Blood Resource Program 1972) wurde hingegen eine Prävalenz von 258 Fällen von Hämophilie auf 106 Männer gefunden (205/106 Hämophilie A und 53/106 Männer Hämophilie B). In Deutschland wurde die Inzidenz auf 100 (Bitter et al. 1963) bzw. 110 (Landbeck u.Kurme 1970) Hämophile auf 106 Männer geschätzt. Die beobachteten Unterschiede dürften zum Teil auf den unterschiedlichen Erfassungsgrad in den untersuchten Populationen zurückzuführen sein.

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Literatur

  1. Aach RD, Lander JJ, Sherman LA et al. (1978) Transfusion transmitted viruses: interim analysis of hepatitis among transfused and nontransfused patients. In: Girish, Vyas, Cohen, Schmid (eds) Viral hepatitis. Franklin, Philadelphia, pp 383–396Google Scholar
  2. Abe T, Kazama M (1982) An international survey on the appropriate dosage of hemophilias and related congenital coagulopathies. In: Abe T (ed) Hemophilic treatment, Proc 3rd Int Symp. Kyoritsu Printings, Tokio, pp 273–304Google Scholar
  3. Abildgaard CF (1981) Hazards of prothrombin-complex concentrates in treatment of hemophilia. N Engl J Med 304: 670–671PubMedGoogle Scholar
  4. Abildgaard CF, Simone JV, Schulman I (1965) Steroid treatment of hemophilic hematuria. J Pediatr 66: 117PubMedGoogle Scholar
  5. Abildgaard CF, Britton M, Harrison J (1976) Prothrombin complex concentrate ( Konyne) in the treatment of hemophilic patients with factor VIII inhibitor. J Pediatr 88: 200Google Scholar
  6. Abildgaard CF, Penner J, Watson-Williams E (1980) Anti-inhibitor coagulant complex (autoplex) for treatment of factor VIII inhibitors in hemophilia. Blood 56: 978PubMedGoogle Scholar
  7. Adachihara A (1983) Oral treatment of hemophilia A using traditional kanpo medicine, Huang-lienchiehtutang (plant extract). Haemostasis 132: 78Google Scholar
  8. Addis T (1910) Hereditary haemophilia: deficiency in the coagulability of the blood the only immediate cause of the condition. Q J Med 4: 14Google Scholar
  9. Adelman MI, Gishen P, Dubbins P et al. (1979) Localized intramesenteric hemorrhage-recognizable syndrome in hemophilia ( Technical Note ). Br Med J 2: 642–643PubMedGoogle Scholar
  10. Aggeier PM, White SG, Glendening MB et al. (1952) Plasma thromboplastin component (PCT) deficiency: a new disease resembling hemophilia. Proc Soc Exp Biol Med 79: 692Google Scholar
  11. Agrawal BL, Zelkowitz L, Hletko P (1981) Acute myocardial infarction in a young hemophilic patient during therapy with factor IX concentrate and epsilon aminocaproic acid. J Pediatr 98: 931PubMedGoogle Scholar
  12. Ahlberg AKM (1975) On the natural history of hemophilic pseudotumor. J Bone Joint Surg [Am] 57: 1133Google Scholar
  13. Ahlberg AKM, Pettersson H (1981) Radiologic method for follow-up of hemophilic arthropathy. In: Seligsohn U, Rimon A, Horoszowski H (eds) Hemophilia. Castle House Publ, London, p 175Google Scholar
  14. Akhmeteli MA, Aledort LM, Alexaniants S et al. (1977) Methods for the detection of haemophilia carriers: a memorandum. Bull WHO 55: 675Google Scholar
  15. Aledort LM (1982) Lessons from Hemophilia. N Engl J Med 306: 605–608Google Scholar
  16. Aledort LM, Goodnight SH (1981) Hemophilia treatment: Its relationship to blood products. Prog Hematol 12: 125–141PubMedGoogle Scholar
  17. Allain JP (1977) Home treatment of hemophiliacs in France. Scand J Haematol 31: 5–8Google Scholar
  18. Allain JP (1979) Dose requirements for replacement therapy in haemophilia A. Thromb Haemost 42: 825–831PubMedGoogle Scholar
  19. Allain JP, Frommel D (1973) Antibodies to factor VIII. I. Variations in stability of antigen-antibody complexes in hemophilia A. Blood 42: 437–444PubMedGoogle Scholar
  20. Allain JP, Frommel D (1974) Antibodies to factor VIII: specificity and kinetics of iso and hetero-antibodies in hemophilia A. Blood 44: 313–322PubMedGoogle Scholar
  21. Allain JP, Frommel D (1976) Antibodies to factor VIII. V. Patterns of immune response to factor VIII in hemophilia A. Blood 47: 973–982Google Scholar
  22. Allain JP, Verroust F, Soulier JP (1980) In vitro and in vivo characterization of factor VIII preparations. Vox Sang 38: 68–80PubMedGoogle Scholar
  23. Allain JP, Gaillandre A, Lee H (1981) Immunochemical characterization of antibodies to factor VIII in hemophilic and non-hemophilic patients. J Lab Clin Med 97: 791PubMedGoogle Scholar
  24. Andes WA (1983) Factor VIII concentrate-hepatitis. JAMA 249: 2331PubMedGoogle Scholar
  25. Andes WA, Edmunds JO, Walker PD et al. (1983) Hemophilic arthropathyan immunologic study of the synovium. In: Blomback (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 127Google Scholar
  26. Andrade JR de, Grant C, Dixon AS J (1965) Joint distension and reflex muscle inhibition in the knee. J Bone Joint Surg [Am] 47: 313–322Google Scholar
  27. Arnold WD, Hilgartner MW (1977) Hemophilic arthropathy. Current concepts of pathogenesis and management. J Bone Joint Surg [Am] 59: 287Google Scholar
  28. Aronson DL (1979) Factor IX complex. Sem Thromb Hemost 6: 28Google Scholar
  29. Aronson DL, Bagley J (1981) Preliminary characterisation of factor VIII bypassing coagulant. Ann NY Acad Sci 370: 291PubMedGoogle Scholar
  30. Aronstam A, Kirk JP, McHardy J et al. (1977) Twice weekly prophylactic therapy in haemophilia A. J Clin Pathol 30: 65–67PubMedGoogle Scholar
  31. Aronstam A, McLellan DS, Turk P (1979 a) Transfusion requirements of adolescents with severe haemophilia A. J Clin Pathol 32: 927–930Google Scholar
  32. Aronstam A, Rainsford SG, Painter MJ (1979 b) Patterns of bleeding in adolescents with severe haemophilia A. Br Med J [Clin Res] 1: 469–470Google Scholar
  33. Aronstam A, Aston L, Wassef M et al. (1980 a) New activated factor IX product in haemophilia. Lancet 1294Google Scholar
  34. Aronstam A, Wassef M, Choudhury DP (1980b) Double-blind controlled trial of three dosage regiments in treatment of haemarthroses in haemophilia. A. Lancet 169–171Google Scholar
  35. Aronstam A, Wassef M, Hamad Z et al. (1981) The identification of high-risk elbow hemorrhages in adolescents with severe hemophilia A. J Pediatr 99: 776–778Google Scholar
  36. Aronstam A, Wassef M, Hamad Z (1982 a) Low doses of factor VIII for selected ankle bleeds in severe haemophilia A. Br Med [Clin Res] 284: 790Google Scholar
  37. Aronstam A, McLellan DS, Wassef M et al. (1982b) Effect of height and weight in the in vivo recovery of transfused factor VIII C. J Clin Pathol 35: 289–291PubMedGoogle Scholar
  38. Ashenhorst JB, Langehennig PL, Seeler RA et al. (1976) Hemolytic anemia due to anti-B in antihemophiliac factor concentrates. J Pediatr 88: 257Google Scholar
  39. Astrup T, Sjolin K (1958) Thromboplastic and fibrinolytic activity of human synovial membrane and fibrous capsular tissue. Proc Soc Exp Biol Med 97: 852PubMedGoogle Scholar
  40. Austen DEG, Rhymes IL (1975) A laboratory manual of blood coagulation. Blackwell Scientific, OxfordGoogle Scholar
  41. Austen DEG, Rizza CR (1981) Oral treatment of hemophilia. Lancet 11: 359Google Scholar
  42. Austen DEG, Rhymes IL, Rizza CR (1981) Factor VIII concentrates: what the label says. Lancet 11: 1167Google Scholar
  43. Austen DEG, Lechner K, Rizza CR et al. (1982) A comparison of the Bethesda and New Oxford methods of factor VIII antibody assay. Thromb Haemost 471: 72–75Google Scholar
  44. Aznar JA, Jorquera JL, Peiro A (1983) A protocol for the eradication of F-VIII antibodies applied to 6 hemophilia a patients with inhibitors. In: Blomback M (ed) Proc Congr WFH, Stockholm. Carger, Basel, p 45Google Scholar
  45. Baehner RL, Strauss HS (1966) Hemophilia in the first year of life. N Engl J Med 275: 524PubMedGoogle Scholar
  46. Bajaj SP, Fierer DS, Epstein DJ (1983) Activated protein C (APC) in factor IX concentrates: An inhibitory effect of APC on the nonactivated partial thromboplastin time ( NAPTT) of the concentrates. Thromb Hemostas 50: 349Google Scholar
  47. Bamber M, Murray A, Kernoff PBA et al. (1981) Short incubation non-A, non-B hepatitis transmitted by factor VIII concentrates in patients with congenital coagulation disorders: a preliminary report of an antigen/antibody system. Med Lab Sci 38: 373–378PubMedGoogle Scholar
  48. Bark CJ, Orloff MJ (1972) The partial thromboplastin time and factor VIII therapy. Am J Clin Pathol 57: 478PubMedGoogle Scholar
  49. Baron JM et al. (1970) Splenectomy in a hemophiliac. Diagnosis of occult rupture. Preoperative evaluation, and postoperative support. Surg Clin North Am 50: 205–211PubMedGoogle Scholar
  50. Barrett KE, Israels MCG (1965) Haemothorax in haemophilia. Thorax 20: 416Google Scholar
  51. Barrow EM, Graham JB (1974) Blood coagulation factor VIII (antihemophilic factor): with comments on von Willebrands disease and Christmas disease. Physiol Rev 54: 23–74PubMedGoogle Scholar
  52. Barrowcliffe TW, Kirkwood TBL (1978) An international collaborative assay of factor VIII clotting activity. Thromb Hemost 40: 260–271Google Scholar
  53. Barrowcliffe TW, Stableforth R, Dormandy KM (1973) Small scale preparation and clinical use of factor IX prothrombin complex. Vox Sang 25: 426–441PubMedGoogle Scholar
  54. Barrowcliffe TW, Kemball-Cook G, Gray E (1981a) Factor VIII inhibitor bypassing activity: A suggested mechanism of action. Thromb Res 21: 181Google Scholar
  55. Barrowcliffe TW, Kemball-Cook G, Morris G (1981 b) Factor VHI-related activities in therapeutic concentrates. J Lab Clin Med 97: 429–438Google Scholar
  56. Bartheis M (1981) Hämophilie: abdominelle Blutung, Blutungsort und lokale Ursachen. In: Landbeck G, Marx R (Hrsg) 10. Hämophilie-Symp, Hamburg 1979. Pharm Verlagsgesellschaft, München, S 124Google Scholar
  57. Bartheis M, Poliwoda H (1978) Ambulante Früh-und Selbstbehandlung nach Bedarf. In: Landbeck G, Marx R (eds) 8. Hämophiliesymposien. Global, HeidelbergGoogle Scholar
  58. Bartheis M, Sens B (1982) Die nordwestdeutsche Therapieverlaufsstudie zur Ermittlung einer rationalen Hämophilietherapie. Hämophilieblätter 16: 20Google Scholar
  59. Bartholome K, Geiger H, Schimpf K (1976) Therapie von Hemmkörperhämophilie mit isoagglutinfreiem Faktor VIII Konzentrat. Dtsch Med Wochenschr 101: 1252–1254PubMedGoogle Scholar
  60. Bern JL, Painter MJ, Aronstam A et al. (1979) The frequency of bleeding and height of adolescent haemophiliacs. Thromb Hemost 41: 286–290Google Scholar
  61. Bertina RM (1981) Genetic variants of factor IX. In: Seligsohn U, Rimon A, Horoszowski H (eds) Haemophilia. Castle House Publ, London, p 19Google Scholar
  62. Bertina RM, Linden IK van der (1982 a) Detection and classification of molecular variants of factor IX. Methods in Hematology 5: 137–155Google Scholar
  63. Bertina RM, Linden IK van der (1982b) Factor IX Zutphen. A genetic variant of blood coagulation factor IX with an abnormally high molecular weight. J Lab Clin Med 100: 695Google Scholar
  64. Bertina R, Linden IK van der ( 1982 c) Factor IX Deventer. Evidence for the heterogeneity of hemophilia Bm. Thromb Hemost 47: 136Google Scholar
  65. Bertina RM, Veitkamp J J (1978) The abnormal factor IX of hemophilia B+ variants. Thromb Hemost 40: 335–349Google Scholar
  66. Bertina RM, Veitkamp JJ (1979) A genetic variant of factor IX with decreased capacity for Ca2+ binding. Br J Haematol 42: 623PubMedGoogle Scholar
  67. Biggs R (1977) Haemophilia treatment in the United Kingdom from 1969 to 1974. Br J Haematol 35: 481–504Google Scholar
  68. Biggs R, MacFarlane RG (1966) Treatment of haemophilia and other coagulation disorders. Black-well, Oxford, pp 49–50Google Scholar
  69. Biggs R, Matthews JM (1966) Treatment of haemophilia and other coagulation disorders. Blackwell, Oxford, pp 107–128Google Scholar
  70. Biggs R, Rizza CR (1976) The sporadic case of haemophilia A. Lancet 11: 431Google Scholar
  71. Biggs R, Douglas AS, MacFarlane RG et al. (1952) Christmas disease: a condition previously mistaken for hemophilia. Br Med J 2: 1378PubMedGoogle Scholar
  72. Biggs R, Austen DEG, Denson KWE et al. ( 1972 a) The mode of action of antibodies which destroy factor VIII. I. Antibodies which have second-order concentration graphs. Br J Haematol 23: 125PubMedGoogle Scholar
  73. Biggs R, Austen DEG, Denson KWE et al. (1972b) The mode of action of antibodies which destroy factor VIII. II. Antibodies which give complex concentration graphs. Br J Haematol 23: 137–155PubMedGoogle Scholar
  74. Bithell TC, Pizzaro A, MacDiarmid WD (1970) Variant of factor IX deficiency in female with 45, X Turner Syndrome. Blood 36: 169–179Google Scholar
  75. Bitter K, Goedeke L, Landbeck G et al. (1963) Die Vererbung der Hämophilie A. Der Internist 4: 397–400PubMedGoogle Scholar
  76. Bittscheid W, Hofmann P, Schumpe G (1978) Elektromyographische Untersuchungen an der Oberschenkelmuskulatur des Hämophilen beim Kniegelenkserguß. Z Orthop 116: 56–60Google Scholar
  77. Blatt PM, Lundblad RL, Kingdon HS et al. (1974) Thrombogenic materials in prothrombin complex concentrates. Ann Intern Med 81: 766–770PubMedGoogle Scholar
  78. Blatt PM, Menache D, Roberts HR (1980) A survey of the effectiveness of prothrombin complex concentrates in controlling hemorrhage in patients with hemophilia and anti-factor VIII antibodies. Thromb Hemost 44: 39Google Scholar
  79. Blättler W, Jacky E, Müller M et al. (1979) Der Einfluß von l-Desamino-D-Arginine-Vasopressin ( DDAVP) auf Blutgerinnung bei Patienten mit Hämophilie A und gesunden Männern. Schweiz Med Wochenschr 109: 1367PubMedGoogle Scholar
  80. Blombäck M (1977) Home treatment of hemophilias in Sweden. Scand J Haematol 31: 9Google Scholar
  81. Blombäck M, Larsson A, Wallinder U et al. (1983) Current situation of home care therapy in Sweden. In: Abe T (ed) Proc 3rd Int Symp Kyoritsu Printings, Tokyo, pp 177–186Google Scholar
  82. Bloom AL (1978) Clotting factor concentrates for resistant haemophilia. J Haematol 40: 21–27Google Scholar
  83. Bloom AL (1981) Factor VIII inhibitors revisited. Br J Haematol 49: 319PubMedGoogle Scholar
  84. Bosch P, Nowotny C, Schwägerl W (1979) Zur orthopädischen Anwendung des Fibrinklebesystems bei Hämophilen. Blut 38: 68Google Scholar
  85. Boese EC, Tantum KR, Eyster ME (1979) Pulmonary function abnormalities after infusion of antihemophilic factor ( AHF) concentrates. Am J Med 67: 474–476Google Scholar
  86. Bolkan B, Sawitsky A (1976) Factor IX deficiency in Gaucher’s disease. Arch Intern Med 136: 489–492Google Scholar
  87. Boulton FE, Smith A (1979) DDAVP and cryoprecipitate in mild hemophilia ( Letter ). Lancet 2: 535Google Scholar
  88. Bouma BN, Klaauw MM van der, Veitkamp JJ et al. (1975) Evaluation of the detection rate of hemophilia carriers. Thromb Res 7: 339–350PubMedGoogle Scholar
  89. Boyer SH, Graham JB (1965) Linkage between the X-chromosome loci for glucose-6-phosphate dehydrogenase electrophoretic variation and hemophilia A. Am J Hum Genet 17: 320–324PubMedGoogle Scholar
  90. Brackmann HH (1977) Hemophilia home treatment in West Germany. Scand J Haematol 31: 11–15Google Scholar
  91. Brackmann HH (1982) The treatment of inhibitors against factor VIII by continuous treatment of factor VIII and activated prothrombin complex concentrates. In: Mariani G, Russo MA, Mandelli F (eds) Activated prothrombin complex concentrates. Praeger Publ, New York, p 194Google Scholar
  92. Brackmann HH, Egli H (1981) Treatment of haemophilia patients with inhibitors. In: Seligsohn U, Rimon A, Horoszowski H (eds) Haemophilia. Castle House Publ, London, pp 113–119Google Scholar
  93. Brackmann HH, Gormsen J (1977) Massive factor VIII infusion in hemophiliac with factor VIII inhibitor, high responser (letter). Lancet 2: 933PubMedGoogle Scholar
  94. Brackmann HH, Hofmann P, Egli H (1980) Die kontrollierte Selbstbehandlung bei Hämophilen. Die gelben Hefte 20: 151Google Scholar
  95. Bradley DW, Cook EH, Maynard JE et al. (1979) Experimental infection of chimpanzees with antihemophilic ( Factor VIII) materials-recovery of virus-like particles associated with non-A, non-B hepatitis. J Med Virol 3: 253–269PubMedGoogle Scholar
  96. Braunstein KM, Griffith MJ, Briet E etal. (1981) The molecular defect of factor IX Chapel Hill. Thromb Haemost 46: 256Google Scholar
  97. Breederveld C (1983) Quantitative T-lymphocyte subpopulations in 190 Dutch hemophiliacs: a comparative study. In: Blomböck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 264Google Scholar
  98. Breen FA, Tullis JL (1969) Use of chromatographic prothrombin complex as an hemostatic agent. JAMA 208: 1848PubMedGoogle Scholar
  99. Briet E, Tilburg N van, Veltkamp JJ (1978) Oral contraception and the detection of carriers in haemophilia B. Thromb Res 13: 379–388PubMedGoogle Scholar
  100. Briet E, Bertina RM, Tilburg N van et al. ( 1982 a) Hemophilia B. Leyden: A sex-linked hereditary disorder that improves after puberty. N Engl J Med 306: 788PubMedGoogle Scholar
  101. Briet E, Reisner HM, Blatt PM (1982 b) Factor IX levels during pregnancy in a woman with hemophilia B. Haemostasis 11: 87–89Google Scholar
  102. Brinkhous KM (1947) Clotting defect in hemophilia deficiency is a plasma factor required for platelet utilization. Proc Soc Exp Biol Med 66: 117–120PubMedGoogle Scholar
  103. Brinkhous KM (1982) Faktor-VIII-Konzenträte. Ihre Verwendung bei Hämophilie A und von Willebrandscher Erkrankung. Hämostaseologie 2: 91Google Scholar
  104. Brinkhous KM, Shanbrom E, Roberts HR et al. (1968) A new high-potency glycine-precipitated antihemophilic factor (AHF) concentrate. Treatment of classical hemophilia and hemophilia with inhibitors. JAMA 205: 613–617PubMedGoogle Scholar
  105. Brinkhous KM, Roberts HR, Weiss AE (1972) Prevalence of inhibitors in hemophilia A and B. Thromb Diath Haemorrh [Suppl] 51: 315–320Google Scholar
  106. Broder S, Gallo RC (1984) A pathogenic retrovirus ( HTLV III) linked to AIDS. N Engl J Med 311: 1292Google Scholar
  107. Brook J, Newnam PE (1965) Spontaneous rupture of the spleen in hemophilia. Arch Intern Med 115: 595–597PubMedGoogle Scholar
  108. Brunswig D (1981) Diskussionsbemerkung. In: Landbeck G, Marx R (eds) 10. Hämophilie Symposion, Hamburg 1979. Pharm Verlagsgesellschaft, München, S 127Google Scholar
  109. Buchanan G, Kevy S (1978) Use of prothrombin complex concentrates in hemophiliacs with inhibitors: Clinical and laboratory studies. Pediatrics 62: 767Google Scholar
  110. Bütler R (1978) L’hepatite virale chez les hémophiles. Ned et Hyg (Geneve) 36: 1289Google Scholar
  111. Bütler R, Stampfli K (1979) Hepatitis epidemiology in Swiss hemophiliacs. Schweiz Med Wochenschr 109: 1449–1453PubMedGoogle Scholar
  112. Carron DB, Boon TH, Walker FC (1965) Peptic ulcer in hemophiliacs and its relation to gastrointestinal bleeding. Lancet II: 1036Google Scholar
  113. Cederbaum AL (1982) Abnormal serum transaminase levels in patients with hemophilia A. Arch Intern Med 142: 337Google Scholar
  114. Chandra S, Wickerhauser M (1979) Contact factors are responsible for the thrombogenicity of prothrombin complex. Thromb Res 14: 189PubMedGoogle Scholar
  115. Chantarangkul V, Ingram GIC, Thorn MB et al. (1978) An artificial „haemophiliac“plasma for one-stage factor VIII assay. Br J Haematol 40: 471–488PubMedGoogle Scholar
  116. Chediak J, Telfer MC, Joajaroenkul T et al. (1980) Lower factor VIII coagulant activity in daughters of subjects with hemophilia as compared to other obligate carriers. Blood 55: 552PubMedGoogle Scholar
  117. Chung KS et al. (1978 a) Purification and characterization of an abnormal factor IX (Christmas factor) molecule. J Clin Invest 62:1078–1086PubMedGoogle Scholar
  118. Chung KS et al. ( 1978 b) Purification and characterisation of an abnormal factor IX Alabama (F IXAla). Abstr. XVII. Congr Int Soc Hematology, Paris, International Society of Blood Transfusion, Book of Abstracts, p 859Google Scholar
  119. Cotter SM et al. (1978) Hemophilia A in three unrelated cats. J Am Vet Med Assoc 172 /2: 166–168PubMedGoogle Scholar
  120. Craske J, Dilling N, Stern D (1975) An outbreak of hepatitis associated with intravenous injection of factor VIII concentrate. Lancet 2: 221–223PubMedGoogle Scholar
  121. Crobcroft R, Tamagnini G, Dormandy KM (1977) Serial plasmapheresis in a haemophiliac with antibodies to factor VIII. J Clin Pathol 30: 763–765Google Scholar
  122. Cromwell LD, Kerber L, Ferry PC (1977) Spinal cord compression and hematoma: an unusual complication in a hemophiliac infant. Am J Roentgenol 128 /5: 847–849Google Scholar
  123. Dalinka MK, Lally JF, Rancier LF et al. (1975) Nephromegaly in haemophilia. Radiology 115: 337–340PubMedGoogle Scholar
  124. Danza FM, Falappa P, Uncini A et al. (1981) Iliac hematoma with femoral neuropathy: Computer-ized tomography demonstration. Thromb Haemost 45: 99PubMedGoogle Scholar
  125. Davis KC, Horsburgh CR, Hasiba U et al. (1983) Acquired immunodeficiency syndrome in a patient with hemophilia. Ann Intern Med 98: 284–286PubMedGoogle Scholar
  126. De La Fuente B, Hoyer LW (1984) The idiotypic characteristics of human antibodies to factor VIII. Blood 64: 672PubMedGoogle Scholar
  127. Denson KWE, Biggs R, Mannucci PM (1968) An investigation of three patients with Christmas disease due to an abnormal type of factor IX. J Clin Pathol 21: 160PubMedGoogle Scholar
  128. Denson KWE, Biggs R, Haddon ME (1969) Two types of haemophilia (A+ and A-): A study of 48 cases. Br J Haematol 17: 163PubMedGoogle Scholar
  129. Didisheim P, Loeb J, Blatrix C et al. (1959) Preparation of a human plasma fraction rich in prothrombin, proconvertin, Stuart factor and PTC and a study of its activity and toxicity in rabbits and in man. J Lab Clin Med 53: 322PubMedGoogle Scholar
  130. Dike GWR, Bidwell E, Rizza CR (1972) The preparation and clinical use of a new concentrate containing factor IX, prothrombin and factor X and of a separate concentrate containing factor VII. Br J Haematol 22: 469–489PubMedGoogle Scholar
  131. Donaldson MH, Clark RE (1968) Successful operation for intussusception in the classic hemophiliac. Ann Surg 168: 1043–1047PubMedGoogle Scholar
  132. Dormandy KM (1975) Immunosuppression in the treatment of hemophiliacs with antibodies to factor VIII. Proceedings of the IXth congress of the World Federation of hemophilia (Istanbul). Excerpta Med Int Congr Series 356: 225Google Scholar
  133. Dormandy KM, Sultan Y (1975) The suppression of factor VIII antibodies in haemophilia. Pathol Biol (Paris) 23: 17–23Google Scholar
  134. Dormandy KM, Hawkey C, Churchill WGL et al. (1971) Immunosuppression of inhibitors to human factors VIII in the patas monkey. Thrombos Diath Haemorr 43: 355–364Google Scholar
  135. Dvilansky A, Nathan I, Eldor A (1979) Classical haemophilia in a girl. Blut 39: 47–50Google Scholar
  136. Edson JR, McArthur JR, Branda RF et al. (1973) Successful management of a subdural hematoma in a hemophiliac with an anti-factor 8 antibody. Blood 41: 113–122PubMedGoogle Scholar
  137. Egberg N, Blombäck M (1981) High frequency of low plasma haptoglobin values found in hemophilia A patients on prophylactic treatment with factor VIII concentrates-A sign of hemolysis? Thromb Haemost 46: 554–557PubMedGoogle Scholar
  138. Egli H (1983) Diskussionsbemerkungen. In: Abe T (ed) Proc 3rd Int Symp. Kyoritsu Printings, Tokyo, p 306Google Scholar
  139. Ehrmann L, Lechner K, Mamoli B et al. (1981) Peripheral nerve lesions in haemophilia. J Neurol 225: 175–182PubMedGoogle Scholar
  140. Elödi S (1975) Factor IX activity and factor IX antigen in haemophilia B carriers. Thromb Res 6: 39PubMedGoogle Scholar
  141. Elödi S, Puskas E (1972) Variants of haemophilia B. Thromb Diath Haemorr 28: 489Google Scholar
  142. Elödi S, Varadi K (1978) Activation of clotting factors in prothrombin complex concentrates as demonstrated by clotting assays for factors IXa and Xa. Thromb Res 12: 797–807PubMedGoogle Scholar
  143. Elliot JL, Hoppes WL, Piatt MS et al. (1983) The acquired immunodeficiency syndrome and myco-bacterium avium intracellulare bacteremia in a patient with hemophilia. Ann Intern Med 98: 290–293Google Scholar
  144. Eisinger F (1982) Laboratory tests of activated prothrombin complex preparations. In: Mariani G, Russo MA, Mandelli F (eds) Activated prothrombin complex concentrates. Managing hemophilia with factor VIII inhibitor. Praeger Pubi, New York, p 77Google Scholar
  145. Elston RC, Graham JB, Miller CH et al. (1976) Probabilistic classification of hemophilia A carriers by disdriminant analysis. Thromb Res 8: 683PubMedGoogle Scholar
  146. English PJ, Sheppard EM, Wensley RT (1976) Traumatic rupture of the liver in a haemophiliac patient with factor VIII inhibitors. Lancet 1: 1299PubMedGoogle Scholar
  147. Erskine JG, Davidson JF (1981) Anaphylactic reaction to low-molecular weight porcine factor VIII concentrates. Br Med J 282: 2011Google Scholar
  148. Evenson SA, Thaule R, Groan K (1979) Selftherapy for haemophilia in Norway. Acta Med Scand 205: 395–399Google Scholar
  149. Ewing NP, Dietrich SL, Kasper CK (1983) In vivo factor VIII survival after infusion of cryoprecipitate or lyophilized concentrate in hemophilia A. Thromb Haemost 50: 209Google Scholar
  150. Eyster ME, Ladda RL, Bowman HS (1977) Carriers with excessively low factor VIII procoagulant activity ( VIII AHF ): A study of two unrelated families with mild hemophilia A. Blood 49: 607Google Scholar
  151. Eyster ME, Gill FM, Blatt PM et al. (1978) Central nervous system bleeding in hemophiliacs. Blood 51: 1179PubMedGoogle Scholar
  152. Eyster ME, Koch KL, Abt AB et al. (1982) Cryptosporidiosis in a hemophiliac with acquired immunodeficiency. Blood [Suppl] 60:1:211 aGoogle Scholar
  153. Fareed J, Mariani G, Parvez Z et al. (1983) Quantitation of protein C in anti-inhibitor complexes. Implication in the management of hemophiliacs. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Larger, Basel, p 175Google Scholar
  154. Fasching I, Lechner K, Niessner H et al. (1982) Klinische Erstmanifestation der schweren Hämophilia A und B. 13. Hamburger Hämophilie Symposium (im Druck) Fasching I, Lechner K, Bettelheim P et al. (1983) Variable response to high dose factor VIII treatment in hemophiliacs with antibody to factor V III. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, BaselGoogle Scholar
  155. Feinstein D, Chong MNY, Kasper CK et al. (1969) Haemophilia A: polymorphism detectable by a factor VIII antibody. Science 163: 1071PubMedGoogle Scholar
  156. Fekete LF, Hoist SL, Peetom F et al. (1972) Auto factor IX concentrate: a new therapeutic approach to treatment of haemophilia A patients with inhibitors. 14th Int Congr of Hematology, Brazil, Abstr 295Google Scholar
  157. Firshein SI, Hoyer LW, Lazarchick J et al. (1979) Prenatal diagnosis of classic hemophilia. N Engl J Med 300: 937–941PubMedGoogle Scholar
  158. Fishman DJ, Jones PK, Menitove JE et al. (1982) Detection of the carrier state for classic hemophilia using an enzyme-linked immunosorbent assay ( ELISA ). Blood 59: 1163PubMedGoogle Scholar
  159. Forbes CD, Prentice CRM (1977) Renal disorders in haemophilie A and B. Scand J Haematol 30: 43–50Google Scholar
  160. Forbes CD, Barr RD, McNicol GP et al. (1972) Aggregation of human platelets by commercial preparations of bovine and porcine AHF. J Clin Pathol 25: 210–217PubMedGoogle Scholar
  161. Forbes CD, Hughes JT, Oppenheimer DR (1973) Gastrointestinal bleeding in haemophilia. Q J Med 42: 503PubMedGoogle Scholar
  162. Francesconi M, Korninger C, Thaler E et al. (1982) Plasmapheresis: Its value in the management of patients with antibodies to factor VIII. Haemostasis 11: 79–86PubMedGoogle Scholar
  163. Franklin FI (1971) Trends in the treatment of hemophilia A: patient-administered clotting factor program. Thromb Diath Haemorr [Suppl] 43: 467–470Google Scholar
  164. Friedman-Kien AE, Laubenstein LJ, Rubinstein P et al. (1982) Disseminated Kaposi’s sarcoma in homosexual men. Ann Intern Med 96: 693–699PubMedGoogle Scholar
  165. Fripp RR et al. (1977) Intussusception in haemophilia: a case report. S Afr Med J 52: 52 /15: 617Google Scholar
  166. Frommel D (1979) Development of factor VIII antibody in hemophilic monozygotic twins. Scand J Haematol 23: 64–68Google Scholar
  167. Frommel D, Allain JP (1977) Genetic predisposition to develop factor VIII antibody in classic hemophilia. Clin Immunol Immunopathol 8: 34–38PubMedGoogle Scholar
  168. Frommel D, Muller JY, Prou-Wartelle O et al. (1977) Possible linkage between the major histocompatibility complex and the immune response to factor VIII in classic hemophilia. Vox Sang 33: 270–272PubMedGoogle Scholar
  169. Frommel D, Allain JP, Saint-Paul E et al. (1981) HLA Antigens and factor VIII antibody in classic hemophilia. Thromb Haemost 46: 687–689PubMedGoogle Scholar
  170. Fuerth JH, Mahrer P (1981) Myocardial infarction as a complication of therapy with factor IX concentrate. JAMA zit nach AbildgaardGoogle Scholar
  171. Fukui H, Fujimura Y, Takamashi Y et al. (1981) Laboratory evidence of DIC unter FEIBA treatment of a haemophilia patient with intracranial bleeding and high titre factor VIII inhibitor. Thromb Res 22: 177PubMedGoogle Scholar
  172. Furlan M, Feliz R, Beck EA (1979) Preparation of factor VIII deficient plasma by immunoadsorption. Vox Sang 36: 342PubMedGoogle Scholar
  173. Furlong RA, Peake IR, Bloom AL (1981) Factor VIII clotting antigen (VIIICAg) in haemophilia measured by two immunoradiometric assays (IRMA) using different antibodies, and the measurement of inhibitors to procoagulant factor VIII ( VIIIC) by IRMA. Br J Haematol 48: 643–650Google Scholar
  174. Gadner H, Odenwald E, Jarofke R et al. (1977) Thrombozytenfunktionsstörung während der hochdosierten Substitutionstherapie bei Hämophilie A. Klin Wochenschr 55: 1165PubMedGoogle Scholar
  175. Gatti L, Mannucci PM (1983) Dose-response relationship and immunogenicity of porcine factor VIII. In: Blombäck M (ed) Proc Congr WFH, Stockholm. [Karger, Basel], p 160 (abstr)Google Scholar
  176. Gawryl MS, Hoyer LW (1982) Inactivation of factor VIII coagulant activity by two different types of human antibodies. Blood 60: 1103–1109PubMedGoogle Scholar
  177. Gazengel C, Torchet MF, Bare L et al. ( 1983 a) Use of activated PCC in surgery for 5 hemophiliacs A with inhibitor. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 174Google Scholar
  178. Gazengel C, Torchet MF, Courrouce AM et al. ( 1983 b) Results of hepatitis B surface antigen vaccine associated to seroprophylaxis in hemophiliacs and patients with other constitutional coagulation defects. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 208Google Scholar
  179. Gerety RJ, Hoofnagle JH, Barker LF (1980) Hepatitis associated with haemophilia treatment. In: Mammen EF, Barnhart MJ, Lusher JM, Walsh RT (eds) Treatment of bleeding disorders, PJD Publications Limited, Westbury/NY, pp 199–216Google Scholar
  180. Gianelli F, Choo KH, Rees DJG et al. (1983) Gene delections in patients with haemophilia B and anti-factor IX antibodies. Nature 303: 181Google Scholar
  181. Giddings JC, Seligsohn U, Bloom AL (1977) Immunological studies in combined factor V and factor VIII deficiency. Br J Haematol 37: 257–264PubMedGoogle Scholar
  182. Giddings JC, Sugrue A, Bloom AL (1982) Quantitation of coagulant antigens and inhibition of activated protein C in combined factor V and VIII deficiency. Br J Haematol 52: 495–502PubMedGoogle Scholar
  183. Gilbert M (1975) Hemophilic pseudo-tumor. In: Brinkhous KM, Hemker HC (eds) Handbook of Hemophilia. Excerpta Medica, AmsterdamGoogle Scholar
  184. Gilchrist GS, Hammond D, Mecnyk J (1965) Hemophilia A in a phenotypically normal female with XX/XO mosaicism. N Engl J Med 273: 1402–1406PubMedGoogle Scholar
  185. Giles AR, Johnston M, Hoogendoorn H et al. (1980) The thrombogenicity of prothrombin complex concentrates. The relationship between in vitro characteristics and in vivo thrombogenicity in rabbits. Thromb Res 17: 353–366PubMedGoogle Scholar
  186. Girma JP, Lavergne JM, Meyer D et al. (1981) Immunoradiometric assay of factor VIII: coagulant antigen using four human antibodies. Study of 26 cases of hemophilia A. Br J Haematol 47: 269PubMedGoogle Scholar
  187. Girolami A, Venturelli R, Cella G et al. (1976) Combined hereditary deficiency of factors VII and VIII. A distinct coagulation disorder due to the „lack“of an autosomal gene controlling factor VII and VIII activation? Acta Haematol 55: 181–191PubMedGoogle Scholar
  188. Girolami A, Sticci A, Burul A et al. (1977) An immunological investigation of hemophilia B with a tentative classification of the disease into five variants. Vox Sang 32: 230PubMedGoogle Scholar
  189. Girolami A, Dal Bo Zanon R, Saltarin P et al. (1982) Incidence, significance and subtypes of hemophilia Bm in a large population of hemophilia B patients. Blut 44: 41–49PubMedGoogle Scholar
  190. Gitschier J, Wood WI, Goralka TM et al. (1984) Characterization of the human factor VIII gene. Nature (Lond) 312: 326Google Scholar
  191. Goldfleld M (1977) quoted by Parker L: How frequent is posttransfusion hepatitis after the introduction of 3rd generation donor screening for hepatitis B? What is its probable nature? Vox Sang 32: 346Google Scholar
  192. Goldsmith JC, Moseley PL, Monick M et al. (1983) T-lymphocyte subpopulation abnormalities in apparently healthy patients with hemophilia. Ann Intern Med 98: 294–296PubMedGoogle Scholar
  193. Gomperts ED, Mohammed H, Malekzadeh MH, Fine RN (1981a) Dialysis and renal transplant in a hemophiliac. Thromb Haemost 46 /3: 626–628PubMedGoogle Scholar
  194. Gomperts ED, Jordan S, Berg D et al. (1981b) Circulating immune complexes pre and post clotting factor infusion in hemophilia. Thromb Haemost 46 /4: 694–698PubMedGoogle Scholar
  195. Gomperts ED, Jordan S, Church JA et al. (1983) High dose factor VIII infusion in a child with high titer inhibitor: Immune tolerance induction. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 267 (abstr)Google Scholar
  196. Goodfellow J, Fearn CB da, Matthews JM (1967) Iliacus hematoma. A common complication of hemophilia. J Bone Joint Surg [Br] 49:748Google Scholar
  197. Goodnight SH, Britell CW, Wuepper KD et al. (1979) Circulating factor IX antigen-inhibitor complexes in hemophilia B following infusion of a factor IX concentrate. Blood 53: 93PubMedGoogle Scholar
  198. Gordon RA, Avignon MB, Storch AE et al. (1981) Intramural gastric hematoma in a hemophiliac with an inhibitor. Pediatr 67: 417Google Scholar
  199. Gore RM, Weinberg PE, Anandappa E (1981) Intracranial complications of pediatric hematologic disorders: computed tomographic assessment. Invest Radiol 16 /3: 175PubMedGoogle Scholar
  200. Gottlieb MS, Schroff R, Schanker HM et al. (1981) Pneumocystis carinii pneumonia and mucosal candidiasis in previously healthy homosexual men. N Engl J Med 305: 1425–1431PubMedGoogle Scholar
  201. Gourdeau R, Denton RL (1968) Steroids and hemophilia in the hemophiliac and his world. Proc 5th Congr World Federation of Hemophilia, Montreal. Bibl Haematologica 34: 65Google Scholar
  202. Gowda M, Vietti T, Ternberg JL (1968) The use of cryoprecipitate in the surgical treatment of spontaneous rupture of the spleen in a hemophilia patient. Surg 64: 1119Google Scholar
  203. Graham JB (1979) Genotype assignment (carrier detection) in the haemophilias. Clin Haematol 8: 115–145PubMedGoogle Scholar
  204. Graham JB, Buckwalter JA, Hartley LJ et al. (1949) Canine hemophilia. J Exp Med 90: 97PubMedGoogle Scholar
  205. Graham JB, Barrow ES, Roberts HR et al. (1975) Dominant inheritance of hemophilia A in three generations of women. Blood 46: 175–188PubMedGoogle Scholar
  206. Graham JB, Flyer P, Eiston RC et al. (1979) Statistical study of genotype assignment (carrier detection) in hemophilia B. Thromb Res 15: 69–78PubMedGoogle Scholar
  207. Graham JB, Barrow ES, Flyer P et al. (1980) Identifying carriers of mild haemophilia. Br J Haematol 44: 671PubMedGoogle Scholar
  208. Gralnik HR, Rick ME (1983) Danazol increases factor VIII and factor IX in classic hemophilia and Christmas disease. N Engl J Med 308: 1393–1395Google Scholar
  209. Green D (1975) Factor VIII antibodies. Immunosuppressive therapy. Recent Advances in Hemophilia 240: 389Google Scholar
  210. Green D, Lechner K (1981) A survey of 215 non-hemophilic patients with inhibitors to factor VIII. Thromb Haemost 45: 200PubMedGoogle Scholar
  211. Green D, Rana N, Spies S (1979) Hemophilic bleeding evaluated by blood pool scanning. Abstract XIII Intern Congr WFH, Tel Aviv, U. Seligsohn, Tel Aviv, p 61Google Scholar
  212. Grignani G, Gamba G, Ascari E (1979) In vivo platelet aggregates after replacement therapy in patients with hemophilia A ( Letter ). Thromb Haemost 42: 813–814Google Scholar
  213. Gunning AJ (1966) The surgery of haemophilic cysts. In: Biggs R, McFariane (eds) Treatment of haemophilia and other coagulation disorders. Blackwell Scientific Pubi, Oxford, p 262Google Scholar
  214. Hadziyannis SJ (1981) Chronic sequelae of hepatitis. In: Seligsohn U, Rimon A, Horoszowski H (eds) Hemophilia. Castle House Pubi, London, p 141Google Scholar
  215. Hanna WT, Madigan RR, Miles MA et al. (1981) Activated factor IX complex in treatment of surgical cases of hemophilia A with inhibitor. Thromb Haemost 46 /3: 638–641PubMedGoogle Scholar
  216. Harris PJ, Kessler CM, Lessin LS (1983) Acquired hemolytic anemia and thrombocytopenia ( Evans syndrome) in hemophilia. N Engl J Med 309: 50Google Scholar
  217. Harris RI, Stuart J (1979) Treatment at home. Low-dose factor VIII in adults with haemophilic arthropathy. Lancet 1: 93–94Google Scholar
  218. Harrison HC et al. (1972) Spontaneous intramural hematoma in the sigmoid colon of a haemophiliac. Aust J Surg 42: 69Google Scholar
  219. Hartmann JR (1965) More on steroid treatment of hemophilic hematuria. J Pediatr 66: 1107Google Scholar
  220. Hartmann W, Henning J (1982) Die Qualität von Faktor VIII-Konzentraten auf dem deutschen Arzneimittelmarkt. Die Ortskrankenkasse 64: 1–13Google Scholar
  221. Harvie A, Lowe DO, Forbes CD et al. (1977) Intraspinal bleeding in haemophilia Successful treatment with factor VIII concentrate. J Neurol Neurosurg Psychiatry 40/12:1220 Hasiba VW, Spero JA, Lewis JH (1977) Chronic liver dysfunction in multitransfused hemophiliacs. Transfusion 17: 490Google Scholar
  222. Hasiba U, Seranton PE, Lewis JH et al. (1980) Efficacy and safety of ibuprofen for hemophilic arthropathy. Arch Intern Med 140 /12: 1583PubMedGoogle Scholar
  223. Hassan HJ, Orlando M, Leonardi A et al. (1985) Intragenic factor IX restriction site polymorphism in hemophilia B variants. Blood 65: 441PubMedGoogle Scholar
  224. Hathaway WE, Mahasandana C, Clarke S et al. (1973) Paradoxical bleeding in intensively transfused hemophiliacs. Alteration of platelet function. Transfusion 13: 6PubMedGoogle Scholar
  225. Hathaway WE, Assmus SL, Montgomery RR et al. (1979) Activated partial thromboplastin time and minor coagulopathies. Am J Clin Pathol 71: 22PubMedGoogle Scholar
  226. Hathaway WE, Christian MJ, Jacobson LJ (1983) Variant mild hemophilia. Discrepancy in one stage and two stage factor VIII assays. Thromb Haemost 50: 357Google Scholar
  227. Heath D (1981) Highly purified porcine factor VIII in hemophilia A with inhibitors to factor VIII. Br Med J 282: 654Google Scholar
  228. Hedner U, Kisiel W (1983) Hemostatic effect of factor Vila in two patients with hemophilia A with anti-VIII:C. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 157Google Scholar
  229. Hedner U, Nilsson IM, Bergentz SE (1976) Various prothrombin complex concentrates and their effects on coagulation and fibrinolysis in vivo. Thromb Haemost 35: 386PubMedGoogle Scholar
  230. Hedner U, Nilsson IM, Bergentz SE (1979) Studies on the thrombogenic activities in two prothrombin complex concentrates. Thromb Haemost 42: 1022PubMedGoogle Scholar
  231. Hedner U, Sundqvist SB, Nilsson IM (1982) Immunosuppressive treatment in hemophiliacs with inhibitors. In: Mariani G, Russo MA, Mandelli F (eds) Activated prothrombin complex concentrates. Praeger Pubi, New York, p 45Google Scholar
  232. Hedner U, Hansson BG, Vermylen J et al. (1983) Hepatitis B vaccination in hemophiliacs. A joint study between the hemophilia center in Leuven, Belgium and Malmö, Sweden. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 207Google Scholar
  233. Heijnen L, de Groot C, Prevo A et al. (1983) Arthropathy in haemophiliacs, comparing long-term prophylaxis with treatment on demand. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 194Google Scholar
  234. Heim M, Horoszowski H, Seligsohn U et al. (1982) Ilio-psoas hematoma-its detection, and treatment with special reference to hemophilia. Arch Orthop Trauma Surg 99: 195PubMedGoogle Scholar
  235. Heimburger N (1982) Fortschritte in der Behandlung der Haemophilie A: ein hepatitissicheres Faktor VIII-Konzentrat. Blut 44: 249–251PubMedGoogle Scholar
  236. Heimburger N, Schwinn H, Gratz P et al. (1981) Faktor VIII-Konzentrat, hochgereinigt und in Lösung erhitzt. Arzneimittelforsch 31 /1: 619PubMedGoogle Scholar
  237. Heinrich D, Kotitschke R, Berthold H (1982) Clinical evaluation of the hepatitis safety of a ß-propiolactone/ultraviolet treated factor IX concentrate ( PPSB ). Thromb Res 28: 75Google Scholar
  238. Helmer RE, Alperin JA, Yunginger JW et al. (1980) Anaphylactic reactions following infusion of factor VIII in a patient with classic hemophilia. Am J Med 69 /6: 953PubMedGoogle Scholar
  239. Helske T, Ikkala E, Myllylä G et al. (1982) Joint involvement in patients with severe haemophilia A in 1957–59 and 1978–79. Br J Haematol 51: 643PubMedGoogle Scholar
  240. Hemker HC et al. (1980) Oral treatment of haemophilia A by gastrointestinal absorption of factor VIII entrapped in liposomes. Lancet 1: 70PubMedGoogle Scholar
  241. Herde J, Weller P (1979) Hemophilia-from the point of view of the ophthalmologist. Deut Gesund 34: 1091Google Scholar
  242. Hewitt P, Mackie IJ, Machin SJ (1982) Highly purified porcine factor VIII in haemophilia A. Lancet 1: 741PubMedGoogle Scholar
  243. Hilgartner MW, Arnold WD (1975) Hemophilic pseudotumor treated with replacement therapy and radiation. J Bone Joint Surg [Am] 57: 1145Google Scholar
  244. Hilgartner MW, Giardina P (1977) Liver dysfunction in patients with hemophilia Scand J Hematol [Suppl] 30: 6–1Google Scholar
  245. Hilgartner MW, Sergis E (1977) Current therapy for hemophiliacs: home care and therapeutic complications. Mt Sinai J Med (NY) 44: 316Google Scholar
  246. Hilgartner MW, Knatterud GL, FEIBA study group (1983) The use of factor VIII inhibitor bypassing activity ( FEIBA Immuno) product for treatment of bleeding episodes in hemophiliacs with inhibitors. Blood 61: 36Google Scholar
  247. Hill FGH, Gidding JC, Williams CE et al. (1983) Combined deficiency of factors V and VIII: Study of a family and response to cryoprecipitate and DD A VP infusions including protein C inhibitor measurement. Thromb Haemost 50: 210Google Scholar
  248. Hoag MS, Johnsson FF, Robinson JA et al. (1969) Treatment of hemophilia B with a new clotting-factor concentrate. N Engl J Med 280: 581PubMedGoogle Scholar
  249. Hoffmann JJ, Meulendijk PN (1978) Comparison of reagents for determining the activated partial thromboplastin time. Thromb Haemost 39: 640PubMedGoogle Scholar
  250. Hofmann P, Schumpe G, Brackmann HH et al. (1981) Clinical and radiological evaluation of hemophilic arthropathy in 494 patients. In: Seligsohn U, Rimon A, Horoszowski H (eds) Hemophilia. Castle House Publ, London, p 167Google Scholar
  251. Hofmann P, Döhring S, Schumpe G et al. (1982a) Hämophile Pseudotumoren. Z Orthop 120: 91–21Google Scholar
  252. Hofmann P, Schumpe G, Brackmann HH et al. (1982b) Die hämophile Arthropathie als Beispiel einer chronisch rezidivierenden Kniegelenksarthritis. Therapiewoche 32: 201Google Scholar
  253. Hollinger FB, Aach RD, Gitnick GL et al. (1973) Limitations of solid-phase radioimmunoassay for HB Ag in reducing frequency of post-transfusion hepatitis. N Engl J Med 289: 385PubMedGoogle Scholar
  254. Holmberg L (1972) Genetic studies in a family with testicular feminization, haemophilia and colour blindness. Clin Genet 3: 253PubMedGoogle Scholar
  255. Holmberg L, Henriksson P, Ekelund H et al. (1974) Coagulation in the human fetus. J Pediatr 85: 860PubMedGoogle Scholar
  256. Holmberg L, Nilsson IM, Henriksson P et al. (1978) Homozygous expression of haemophilia B in a heterozygote. Acta Med Scand 204: 231PubMedGoogle Scholar
  257. Holmberg L, Borge L, Ljung R et al. (1979) Measurement of antihaemophilic factor A antigen (VIII: CAg) with a solid phase immunoradiometric method based on homologous non-haemophilic antibodies. Scand J Haematol 23: 17PubMedGoogle Scholar
  258. Holmberg L, Gustavii B, Cordesius E et al. (1980) Prenatal diagnosis of hemophilia B by an immunoradiometric assay of factor IX. Blood 56: 397PubMedGoogle Scholar
  259. Holmberg L, Borge L, Nilsson IM (1981) Factor VIII:C and VIII CAg response in a patient with haemophilia A and von Willebrands disease after administration of different factor VIII concentrates or plasma. Br J Haematol 47: 587PubMedGoogle Scholar
  260. Hoofnagle JH, Aronson D, Roberts H (1975) Serologic evidence for hepatitis B virus infection in patients with hemophilia B. Thromb Diath Haemorr 33: 606Google Scholar
  261. Hopff F (1928) Cited by united states surgeon generals catalogue, 1st series. HaemophiliaGoogle Scholar
  262. Hougie C, Twomey J J (1967) Hemophilia BM: A new type of factor IX deficiency. Lancet 1: 198Google Scholar
  263. Hoyer LW, Mahoney MJ (1981) Prenatal diagnosis of classic hemophilia. X IV Intern Congr. of the World Federation of Hemophilia, Costa Rica. In: Hemophilia care in developing countries, Oficina de Publicaciones de la Universidad de Costa RicaGoogle Scholar
  264. Hoyer LW, Rick ME (1975) Implications of immunologic methods for measuring antihemophilic factor (factor VIII). Ann NY Acad Sei 240: 97–10Google Scholar
  265. Hoyer LW, Carta CA, Mahoney MJ (1982) Detection of hemophilia carriers during pregnancy. Blood 60: 1407PubMedGoogle Scholar
  266. Hruby MA, Schauf V (1978) Transfusion related short-incubation hepatitis in hemophilic patients. J Am Med Worn Assoc 240: 1355Google Scholar
  267. Hultin M (1979) Activated clotting factors in factor IX concentrates. Blood 54: 1028PubMedGoogle Scholar
  268. Hultin M (1981) Management of inhibitors by immunosuppression. In: Seligsohn U, Rimon A, Horoszowski H (eds) Hemophilia. Castle House Publ., London, p 107Google Scholar
  269. Hultin MB, Shapiro SS, Bowman HS et al. (1976) Immunosuppressive therapy of factor VIII inhibitors. Blood 48: 95PubMedGoogle Scholar
  270. Hultin MB, London FS, Shapiro SS et al. (1977) Heterogeneity of factor VIII antibodies: further immunochemical and biologic studies. Blood 49: 807PubMedGoogle Scholar
  271. Iizuka A, Nagao T (1983) Analysis of IgG heavy chain sublcasses of alloantibodies to factor IX by crossed immunoelectrophoresis of factor IX using the intermediate gel technique. Br J Haematol 53: 687PubMedGoogle Scholar
  272. Ikkala E, Simonen O (1971) Factor VIII inhibitors and the use of blood products in patients with haemophilia A. Scand J Haematol 8: 16PubMedGoogle Scholar
  273. Ikkala E, Helske T, Myllylä G et al. (1982) Changes in the life expectancy of patients with severe haemophilia A in Finland in 1930–. Br J Haematol 52: 7PubMedGoogle Scholar
  274. Imhof H (1951) Über das Vorkommen von haemophilen Blutungen am und im Zentralnervensystem. Z menschl Vererb-und Konstitlehre 30: 466Google Scholar
  275. Ingram GIC (1976) The history of haemophilia. J Clin Pathol 29: 3–1Google Scholar
  276. Ingram GIC (1981) Calculating the dose of factor VIII in the management of haemophilia. Br J Haematol 48: 355Google Scholar
  277. Inwood MJ, Vaugh E (1982) The current status of home care for hemophiliacs in Canada. In: Abe T (ed) Proc 3rd Int Symp HT. Kyoritsu Printers, Tokyo, p 171Google Scholar
  278. Italian CISMEL study group (1980) Activated partial thromboplastin time: a multicenter evaluation of commercial reagents in the diagnosis of mild hemophilia A and other coagulation defects. Scand J Haematol 25: 308Google Scholar
  279. Iwarson S, Kjellman H, Teger-Nilsson AC (1976) Incidence of viral hepatitis after administration of factor IX concentrates. Vox Sang 31: 136PubMedGoogle Scholar
  280. Jarczok K, Krawczyk-Kulis M, Molowiedin J et al. (1981) Hemophilia A in a female with gonadal dysgenesis and XX karyotype. Folia Haematol 108 /1: 136Google Scholar
  281. Johnson AJ, MacDonald VE, Semar M et al. (1978) Preparation of the major plasma fractions by solid-phase polyelectrolytes. J Lab Clin Med 92: 194PubMedGoogle Scholar
  282. Johnson AJ, MacDonald VE, Brind J (1979) Enhanced yield of antihemophilic factor and von Willebrand factor by cryoprecipitation with polyethylene glycol. Vox Sang 36: 72PubMedGoogle Scholar
  283. Jones P (1982) Faktor VIII:C aus Tiermaterial. Hämostaseologie 2: 105Google Scholar
  284. Jones P, Fearns M, Forbes C et al. (1978) Haemophilia A-home therapy in the United Kingdom 1975–. Br Med J 1: 1447PubMedGoogle Scholar
  285. Jones P, Proctor S, Dickinson A et al. (1983) Altered immunology in haemophilia. Lancet 1: 120PubMedGoogle Scholar
  286. Jones PK, Ratnoff OD (1981) Sources of variability in antihemophilic factor (factor VIII) procoagu-lant titers and precipitating antigen levels among obligate carriers of classic hemophilia. Blood 57: 928PubMedGoogle Scholar
  287. Kahn A, Van den Bogaert N, Cremer N et al. (1977) Intramural hematoma of the alimentary tract in the hemophilic children. Helv Paediatr Acta 31 /6: 503PubMedGoogle Scholar
  288. Kamiya T, Koie K, Ismiguro J et al. (1977) Classical haemophilia in girl with 46 XX karyotype and no family history of bleeding disorders (letter). Lancet 1 /8014: 756PubMedGoogle Scholar
  289. Kaneda T, Nagayama M, Ohmori M et al. (1979) Hemarthrosis of the temporomandibular-joint in a patient with hemophilia B-report of case. J Oral Surg 37: 513PubMedGoogle Scholar
  290. Kaneshiro M, Mielke C, Kasper C et al. (1969) Bleeding time after aspirin in disorders of intrinsic clotting. N Engl J Med 281: 1039PubMedGoogle Scholar
  291. Kasper CK (1972) Hepatitis and clotting-factor concentrates. JAMA 221: 5Google Scholar
  292. Kasper CK (1973) Postoperative thrombosis in hemophilia B. N Engl J Med 289: 160PubMedGoogle Scholar
  293. Kasper CK (1975a) Self-infusion treatment program. In: Brinkhous KM, Hemker HC (eds) Handbook of Hemophilia. Excerpta Medica, Amsterdam, pp 597–603Google Scholar
  294. Kasper CK (1975b) Thromboembolic complications. Thromb Diath Haemorr 33: 640Google Scholar
  295. Kasper CK (1975c) Thromboembolic complications following the use of prothrombin complex concentrates. Thromb Diath Haemorr 33: 640Google Scholar
  296. Kasper CK (1981 a) Management of inhibitors to factor VIII. Prog Haematol 12:143 Kasper CK (1981 b) Problems with the potency of factor VIII concentrate. N Engl J Med 50–51Google Scholar
  297. Kasper CK, Rapaport S (1972) Bleeding times and platelet aggregation after analgesics in hemophilia. Ann Intern Med 77: 189PubMedGoogle Scholar
  298. Kasper CK, Kipnis SA (1972) Hepatitis and clotting-factor concentrates. JAMA 221: 510PubMedGoogle Scholar
  299. Kasper CK, Dietrich SL, Rapaport SI (1970) Hemophilia prophylaxis with factor VIII concentrate. Arch Intern Med 125: 1004PubMedGoogle Scholar
  300. Kasper CK, Aledort LM, Counts RB et al. (1975 a) A more uniform measurement of factor VIII inhibitors. Thromb Diath Haemorr 34: 869Google Scholar
  301. Kasper CK et al. (1975b) Determinants of factor VIII recovery in cryoprecipitate. Transfusion 15: 312PubMedGoogle Scholar
  302. Kasper CK, Boylen AL (1985) Poor response to danazol in hemophilia. Blood 65: 211–213PubMedGoogle Scholar
  303. Kasper CK, Osterud B, Minami JY et al. (1977) Hemophilia B: characterization of genetic variants and detection of carriers. Blood 50: 351PubMedGoogle Scholar
  304. Kasper CK and the Hemophilia study group (1979) Effect of prothrombin complex concentrates on factor VIII inhibitors levels. Blood 54: 1358Google Scholar
  305. Kazama M, Abe T (1981) Analysis of the corrective effect of activated prothrombin complexes. Haemostasis 10: 215Google Scholar
  306. Kernoff PBA, Tuddenham EGD (1981) Reactions to low-molecular-weight porcine factor VIII concentrates. Br Med J 283: 281Google Scholar
  307. Kernoff PBA, Thomas ND, Lilley PA et al. (1981) Clinical experience with polyelectrolyte-fractiona-ted porcine factor VIII concentrate in the treatment of haemophiliacs with antibodies to factor VIII. Br J Haematol 49: 131Google Scholar
  308. Kernoff PBA, Thomas ND, Lilley PA et al. (1984) Clinical experience with polyelectrocyte-fraction-ated porcine factor VIII concentrate in the treatment of hemophiliacs with antibodies to factor VIII. Blood 63: 31PubMedGoogle Scholar
  309. Kerr CB (1964) Intracranial haemorrhage in Haemophilia. J Neurol Neurosurg Psychiatry 27: 166PubMedGoogle Scholar
  310. Kerr CB (1965) Genetics of human blood coagulation. J Med Genet 2:254 Kingdon HS, Hassel TM (1980) An animal model for evaluating the haemostatic effectiveness of factor VIII inhibitor bypassing materials. Clin Res 28: 769Google Scholar
  311. Kingdon HS, Lundblad RL, Veitkamp JJ et al. (1975) Potentially thrombogenetic material in factor IX concentrates. Thromb Diath Haemorr 33: 617Google Scholar
  312. Kinney TR, Zimmermann RA, Butler RB et al. (1977) Computerized tomography in the management of intracranial bleeding in hemophilia. J Pediatr 91 /1: 31PubMedGoogle Scholar
  313. Kirkwood TB, Rizza CR, Snape TJ et al. (1977) Identification of sources of inter-laboratory variation in factor VIII assay. Br J Haematol 37: 559PubMedGoogle Scholar
  314. Kisker CT, Burke C (1970) Double-blind studies on the use of steroids in the treatment of acute hemarthrosis in patients with hemophilia. N Engl J Med 282: 639PubMedGoogle Scholar
  315. Kitchens CS (1982) Computed tomography in two cases of hemophilic pseudotumors. Am J Haematol 12: 277Google Scholar
  316. Klein HG, Aledort LM, Bouma BN et al. (1977) A cooperative study for the detection of the carrier state of classic hemophilia. N Engl J Med 296: 959PubMedGoogle Scholar
  317. Klose HJ (1982) Hepatitis als Schicksal des Hämophiliepatienten. In: Frösner G, Lasch HJ, Lechler E (Hrsg) Plasmaproteine und Virushepatitis. Springer, Berlin Heidelberg New York, S 24Google Scholar
  318. Klose HJ, Goetz O, Peller P et al. (1980) Cytomegalic-, Epstein-Barr-, Hepatitis A-, Hepatitis B-Virus-Antikörper und Hepatitis B-Antigene bei kindlichen und jugendlichen Hämophilie-Patienten. In: Schimpf K (Hrsg) Fibrinogen, Fibrin und Fibrinkleber. Schattauer, Stuttgart New York, S 309Google Scholar
  319. Kluge A, Schimpf KL (1978) Immunhämolyse durch Anti-A/B als Nebenwirkung von Gerinnungs-präparaten aus Humanplasma. Forschungsergebnisse Transfusionsmed. Immunhämatol 4: 38Google Scholar
  320. Kluge A, Schimpf KL (1980) Immunhämatologische Nebenwirkungen von Gerinnungspräparaten aus Humanplasma. In: Schimpf K (Hrsg) Fibrinogen, Fibrin und Fibrinkleber. Schattauer, Stuttgart New York, S 327Google Scholar
  321. Koene RAP, Gerlag PGG, Jansen JLF et al. (1977) Successful haemodialysis and renal transplanta-tion in a patient with haemophilia A. Proc Eur Dial Transplant Assoc 14: 401PubMedGoogle Scholar
  322. König F (1892) Die Gelenkserkrankungen bei Blutern, mit besonderer Berücksichtigung der Diagnose. Klin Vorträge 36: 233Google Scholar
  323. Korninger C, Niessner H, Lechner K (1981) Impaired fibrinolytic response to DDAVP and venous occlusion in a sub-group of patients with von Willebrand disease. Thromb Res 23: 365PubMedGoogle Scholar
  324. Kotitschke R, Stephan W, Prince AM et al. (1983) Evaluation of thrombogenicity of /?-propiolactone/ ultraviolet (ß-PL/UV) treated PPSB in chimpanzees. Thromb Res 30: 235PubMedGoogle Scholar
  325. Koutts J, Meyer D, Rickard K et al. (1975) Heterogeneity in biological activity of human factor VIII antibodies. Br J Haematol 29: 99PubMedGoogle Scholar
  326. Kraus B, Klose HJ, Riegel K et al. (1982) Schwere Blutungen bei neugeborenen Kindern mit Hämophilie. In: Landbeck G, Marx R, Stolte HP (Hrsg) Hamburg. Pharmazeutische Verlagsges. mbH, München, Hämophilie Symposion, S 266Google Scholar
  327. Kumari S, Fulco JD, Karayalein G et al. (1979) Gray scale ultrasound: Evaluation of iliopsoas hematomas in hemophiliacs. AJR 133: 103PubMedGoogle Scholar
  328. Kurczynski EM, Penner (1974) Activated prothrombin concentrate for patients with factor VIII inhibitors. N Engl J Med 291: 164Google Scholar
  329. Lancourt JE, Gilbert MS, Posner MA (1977) Management of bleeding and associated complications of hemophilia in the hand and forearm. J Bone Joint Surg [Am] 59: 451Google Scholar
  330. Landbeck G, Kurme A (1970) Die hämophile Kniegelenksarthropathie. Monatsschr Kinderheilk 118: 29Google Scholar
  331. Landbeck G, Marsmann G (1976) Nebenwirkungen langzeitiger kontinuierlicher Substitutionspro-phylaxe bei Patienten mit schwerer Hämophilie A. In: Landbeck G, Marx R (Hrsg) 6. Häm Symp Hamburg 1975. Immuno, HeidelbergGoogle Scholar
  332. Lane S (1940) Successful transfusion of blood. Lancet 1: 185Google Scholar
  333. Larsson SA, Wiechel B (1983) Deaths in swedish hemophiliacs 1957–198. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger Basel, p 23Google Scholar
  334. Larsson SA, Nilsson IM, Blombäck M (1983) Vital statistics of swedish hemophiliacs 1830–198. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 260Google Scholar
  335. Laurian Y, Girma JP, Allain JP et al. (1982) Absence of anamnestic response after transfusion of washed red blood cells in haemophilia A patients with antibody to factor VIII. Scand J Haematol 28: 233PubMedGoogle Scholar
  336. Laurian Y, Girma JP, Lambert T et al. (1984) Incidence of immune responses following 102 infusions of autoplex in 18 hemophilic patients with antibody to factor VIII. Blood 63: 457PubMedGoogle Scholar
  337. Lavergne JM, Meyer D, Reisner H (1976) Characterization of human anti-factor VIII antibodies purified by immune complex formation. Blood 48: 931PubMedGoogle Scholar
  338. Lavergne JM, Meyer D, Koutts J et al. (1978) Isolation of human antibodies to factor VIII. Br J Haematol 40: 631PubMedGoogle Scholar
  339. Lavergne JM, Meyer D, Girma JP et al. (1982) Precipitating anti-VIII:C antibodies in two patients with haemophilia A. Br J Haematol 50: 135PubMedGoogle Scholar
  340. Lazarchick J, Hoyer LW (1977) The properties of immune complexes formed by human antibodies to factor VIII. J Clin Invest 60: 1070PubMedGoogle Scholar
  341. Lazarchick J, Hoyer LW (1978) Immunoradiometric measurement of the factor VIII procoagulant antigen. J Clin Invest 62: 1048PubMedGoogle Scholar
  342. Lechler E (1982) Prothrombinkomplexkonzentrate (Faktor II-VII-IX-X-Komplex). Eigenschaften und klinische Anwendung. Hämostaseologie 2: 116Google Scholar
  343. Lechner K (1971) Factor IX inhibitors: Report of two cases and a study of the biological, chemical and immunological properties of the inhibitor. Thromb Diath Haemorr 25: 447Google Scholar
  344. Lechner K (1972) Inactive factor VIII in haemophilia A and Willebrands disease. Acta Haematol 48: 257PubMedGoogle Scholar
  345. Lechner K (1973) Immunoreactive factor VIII in carriers of hemophilia A+ and A~. Thromb Diath Haemorr 29: 240Google Scholar
  346. Lechner K (1977) Collaborative study on factor VIII inhibitor assay. Workshop on inhibitors of *factors VIII and IX. Facultas, Wien, p 32Google Scholar
  347. Lechner K (1982a) Aktivierte Prothrombinkomplexkonzentrate. Hämostaseologie 2: 110Google Scholar
  348. Lechner K (1982b) Blutgerinnungsstörungen. Laboratoriumsdiagnose hämatologischer Erkrankungen. Springer, Berlin Heidelberg New YorkGoogle Scholar
  349. Lechner K, Korninger C (1981) Structure, characteristics and natural history of factor VIII-and IX-inhibitors in non-hemophiliacs. In: Seligsohn U, Rimon A, Horoszowski (eds) Haemophilia. Castle House Publ, London, p 87Google Scholar
  350. Lechner K, Ludwig E, Niessner H et al. (1972) Factor Vlll-inhibitor in a patient with mild hemophilia A. Haemostasis 1: 261PubMedGoogle Scholar
  351. Lechner K, Nowotny C, Krinninger B et al. (1978) The effect of treatment with activated prothrombin complex concentrate ( FEIBA) on factor VIII antibody level. Thromb Haemost 40: 478Google Scholar
  352. Lechner K, Korainger C, Niessner H et al. (1980) Suppression of hemophilic antibodies by continuous treatment with factor VIII concentrate and prothrombin complex preparation. In: 18th Congr Int Soc Hematology, Montreal, (Abstr. number 1047 ) International Society of Blood Transfusion, Book of AbstractsGoogle Scholar
  353. Lechner K, Bettelheim P, Nowotny C et al. (1982) Suppression of hemophilic factor VIII antibodies by treatment with intermediate doses of factor VIII combined with FEIBA. In: Mariani G, Russo MA, Mandelli F (eds) Activated prothrombin complex concentrates. Praeger Pubi, New York, p 223Google Scholar
  354. Lechner K, Bettelheim P, Deutsch E et al. (1983a) Acquired immune deficiency syndrom (AIDS): A serious complication of hemophilia treatment. Thromb Haemost 50: 410Google Scholar
  355. Lechner K, Fasching I, Niessner H et al. (1983b) Current situation of home care in Austria. In: Abe T (ed) Proc 3rd Int Symp H T. Kyoritsu Printings, Tokyo, p 205Google Scholar
  356. Lederman MM, Ratnoff OD, Scillian JJ et al. (1983) Impaired cell-mediated immunity in patients with classic hemophilia. N Engl J Med 308: 79PubMedGoogle Scholar
  357. Lee CA, Janossy G, Ashley D et al. (1983) Plasma fraction methods and T-cell subsets in haemophilia. Lancet 11: 158Google Scholar
  358. Leeuwen EF v, Geijlswijk JL v, Mauser-Bunschoten EP et al. (1983) Disappearance of factor VIII antibodies in hemophiliacs on factor VIII replacement therapy. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 42 (abstr)Google Scholar
  359. Lesesne HR, Morgan JE, Blatt PM et al. (1977) Liver biopsy in hemophilia A. Ann Intern Med 86: 703PubMedGoogle Scholar
  360. Levine PH (1974) Efficacy of self-therapy in hemophilia: a study of 72 patients with hemophiA and B. N Engl J Med 291: 1381PubMedGoogle Scholar
  361. Levine PH, Britten AFH (1973) Supervised patient-management of hemophilia. Ann Intern Med 78: 195PubMedGoogle Scholar
  362. Levine PH, McVerry BA, Attock B et al. (1977) Health of the intensively treated hemophiliac, with special reference to abnormal liver chemistries and splenomegaly. Blood 50: 1–9PubMedGoogle Scholar
  363. Lewis JH (1970) Causes of death in hemophilia. JAMA 214: 1707PubMedGoogle Scholar
  364. Lewis JH, Bontempo FA, Spero JA et al. (1985) Liver transplantation in a hemophiliac. New Engl J Med 312: 1189PubMedGoogle Scholar
  365. Lewis RM, Reisner HM, Chung KS et al. (1980) Detection of factor IX antibodies by radioimmunoassay: effect of calcium on antibody factor IX interaction. Blood 56: 608PubMedGoogle Scholar
  366. Lindner J (1981) Bindegewebs-Grundlagen der Synositis und Arthrose (unter besonderer Berücksichtigung der Gefäße bei posthämorrhagischer Arthritis und Arthrose. In: Landbeck G (Hrsg) 10. Hämophilie Symp, Hamburg 1979. Pharm. Verlagsgesellschaft, München, S 153Google Scholar
  367. Loeliger EA, Hensen A, Mattern MJ et al. (1967) Treatment of haemophilia B with purified factor IX ( PPSB ). Folia Medica Neerlandica 10: 4Google Scholar
  368. Ljung R, Holmberg L (1981) F VIII:CAg in haemophilia A comparison between IRMA:s using haemophilic and spontaneous antibodies. Thromb Res 24: 45PubMedGoogle Scholar
  369. Ljung R, Nilsson IM (1982) Hemophilia B Leyden and a similar variant of hemophilia A. N Engl J Med 14: 897Google Scholar
  370. Lo Grippo GA, Hayaski H (1973) Efficacy of netaprone with ultraviolet irradiation in hepatitis antigen in human plasma pools. Henry Ford Hosp Med J 21: 2Google Scholar
  371. Lorenz HM (1978) Therapieschema der akuten Blutung bei Patienten mit Hämophilie A. In: Landbeck G, Marx R (Hrsg) 8. Hämophilie Symp Hamburg 1977. Global, Heidelberg, S 63Google Scholar
  372. Lossing TS, Kaspar C, Feinstein DI (1977) Detection of factor VIII inhibitors with the partial thromboplastin time. Blood 49: 793PubMedGoogle Scholar
  373. Louizou C, Panaiotopoulou C, Anatopoulou A et al. (1977) Isoimmunization in haemophiliacs. Scand J Haematol 30: 51Google Scholar
  374. Lowe G, Pettigrew A, Middleton J et al. (1977) DDAVP in hemophilia (letter). Lancet 11: 614Google Scholar
  375. Luban NLC, Kelleher JF, Reaman GH (1983) Altered distribution of T-lymphocyte subpopulations in children and adolescents with haemophilia. Lancet 1: 503PubMedGoogle Scholar
  376. Ludlam CA, Peake IR, Allen N et al. (1980) Factor VIII and fibrinolytic response to deamine-D-argenine vasopressin in normal subjects and dissociate response in some patients with haemophilia and von Willebrand disease. Br J Haematol 45: 449Google Scholar
  377. Ludwig E, Lechner K (1974) Prophylaktische Behandlung bei schwerer Hämophilie B mit einem Faktor-IX-Konzentrat. Dtsch Med Wochenschr 99: 1355PubMedGoogle Scholar
  378. Lurie A (1972) The laboratory assessment of the efficacy of antihaemophilic therapy. S Afr Med J 46: 844PubMedGoogle Scholar
  379. Lusher JM, McMillan CW and the hemophilia study group (1978) Severe factor VIII and factor IX deficiency in females. Am J Med 65: 637Google Scholar
  380. Lusher JM, Shapiro SS, Palascak JP et al. (1980) Efficacy of prothrombin complex concentrates in hemophiliacs with antibodies to factor VIII: a multicenter therapeutic trial. N Engl J Med 303: 421PubMedGoogle Scholar
  381. Lusher JM, Shapiro SS, Palascak JE et al. (1981) Hazards of prothrombin complex concentrates in treatment of hemophilia. N Engl J Med 304: 671Google Scholar
  382. Lusher JM, Ofosu FA, Edson JR et al. (1983) North American study of factor VIII concentrate potency. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 27Google Scholar
  383. MacFarlane RG, Mallmam PC, Witts LJ et al. (1957) Surgery in haemophilia. The use of animal AHG and human plasma in 13 cases. Lancet 11: 251–25Google Scholar
  384. Machin SJ, Miller BR (1980) Congenital combined factor VII and factor VIII deficiency. Acta Haematol 63: 167–16PubMedGoogle Scholar
  385. Mackie IJ, Seghatchian MJ (1982) Analysis of the procoagulant activities of factor IX concentrates. Thromb Res 28: 499–507PubMedGoogle Scholar
  386. Mahoney DH (1974) Intramural gastric lesion with sudden abdominal pain. JAMA 230: 603Google Scholar
  387. Mainardi CL, Levine PH, Werb Z et al. (1978) Proliferative synositis in hemophilia: Biochemical and morphologic observations. Arthritis Rheum 21: 137–144PubMedGoogle Scholar
  388. Mamoli B, Sonneck G, Lechner K (1976) Intrakranielle und spinale Blutungen bei Hämophilie. J Neurol 211: 143–154PubMedGoogle Scholar
  389. Mannucci PM (1977) Home care of haemophilia in Italy. Scand J Haematol 30: 74–75Google Scholar
  390. Mannucci PM, Rota L (1980) Plasminogen activator response after DDAVP: A clinicopharmacologi-cal study. Thromb Res 20: 69Google Scholar
  391. Mannucci PM, Aaberg M, Nilsson IM et al. (1975 a) Mechanism of plasminogen activator and factor VIII increase after vasoactive drugs. Br J Haematol 30: 81–93PubMedGoogle Scholar
  392. Mannucci PM, Capitanio A, Del Ninno E et al. (1975 b) Asymptomatic liver disease in hemophiliacs. J Clin Pathol 28: 620–624PubMedGoogle Scholar
  393. Mannucci PM, Ruggeri ZM, Capitanio A et al. (1976) Clinical experience with a prothrombin complex concentrate in the management of factor VIII inhibitors. Workshop on inhibitor of factors VIII and IX, Jan. 26th and 27th, 1976, Vienna. Facultas, Wien, p 136Google Scholar
  394. Mannucci PM, Ruggeri ZM, Pareti FI et al. (1977) l-deamino–d-arginine vasopressin: A new pharmacological approach to the management of haemophilia and von Willebrand’s disease. Lancet 1: 869–872PubMedGoogle Scholar
  395. Mannucci PM, Coppaia R, Lombardi R et al. (1978 a) Direct proof of extreme lyonization as a cause of low factor VIII levels in females (letter). Thromb Haemost 39/2:544–545Google Scholar
  396. Mannucci PM, Ronchi G, Rota L et al. (1978 b) A clinicopathological study of liver disease in hemophiliacs. J Clin Pathol 31: 779–783PubMedGoogle Scholar
  397. Mannucci PM, Federici A, Vigano S et al. (1979) Multiple dental extractions with a new prothrombin complex concentrate in two patients with factor VIII inhibitors. Thromb Res 15: 359PubMedGoogle Scholar
  398. Mannucci PM, Canciani MT, Rota L et al. (1981) Response of factor VIII/von Willebrand factor to DDAVP in healthy subjects and patients with haemophilia A and von Willebrands disease. Br J Haematol 47: 283–293PubMedGoogle Scholar
  399. Mannucci PM, Colombo M, Rizzetto M (1982) Nonprogressive course of Non-A, Non-B chronic hepatitis in multitransfused hemophiliacs. Blood 60: 655–658Google Scholar
  400. Mariani G, Mazzucconi MG, Chistolini R et al. (1983) Preliminary immunological study in an inhibitor patient treated with long-term-high-dose-factor VIII concentrates. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 40 (abstr)Google Scholar
  401. Markowitz RI, Mendel JB (1981) Retropharyngeal bleeding in haemophilia. Br J Radiol 54 /642: 521–523PubMedGoogle Scholar
  402. Marlar RA, Griffin JH (1980) Deficiency of protein C inhibitor in combined factor V/VIII deficiency disease. J Clin Invest 66: 1186–1189PubMedGoogle Scholar
  403. Martinowitz U, Heim H, Horoszowsky H et al. (1983) Low affinity factor VIII inhibitor in a hemophiliac patient. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 214Google Scholar
  404. Marx F, Lechner K (1973) Zur Prognose der Haemophilie. Lebensversicherungsmedizin 25: 1–7Google Scholar
  405. Mason EC, Pepper DS, Griffin B (1981) Production of cryoprecipitate of intermediate purity in a closed system thaw-siphon process. Thromb Haemost 46 /2: 543–546PubMedGoogle Scholar
  406. Matsuoka M, Ito M, Takahashi K et al. (1976) An immunological method for detection of the carrier of hemophilia B. Thromb Haemost 36: 441–450PubMedGoogle Scholar
  407. Mayr W, Lechner K, Niessner H, Pabinger-Fasching J (1984) HLA-DR and factor Vlll-antibodies in hemophilia A. Thromb Haemost 51: 293PubMedGoogle Scholar
  408. Mazzucconi MG, Bertina RM, Romoli D et al. (1980) Factor VII activity and antigen in haemophilia B variants. Thromb Haemost 43: 16PubMedGoogle Scholar
  409. Mclntyre B, Philip R, Inwood M (1978) Effect of ibuprofen on platelet function in normal subjects and hemophiliac patients. Clin Pharmacol Ther 24: 616–621Google Scholar
  410. McLellan DS, Devlin JD, Groom P et al. (1981) A radial immunodiffusion method for the assay of factor VIII: C antigen (VIII: C Ag) in plasma. Br J Haematol 47: 295PubMedGoogle Scholar
  411. McLellan DS, Pelly C, McLellan HG (1982) The “in vivo” survival characteristics of factor VIII procoagulant antigen (VIII: V Ag) in haemophilia A subjects. Thromb Res 25: 33Google Scholar
  412. McMillan CW (1984) Clinical patterns of hemophilic patients who develop inhibitors. In: Hoyer LW (ed) Factor VIII inhibitors. Alan R Riss Inc, New YorkGoogle Scholar
  413. McVerry BA, Machin SJ (1979 a) Incidence of allo-immunization and allergic reactions to cryoprecipi-tate in haemophilia. Vox Sang 36/2:77–80Google Scholar
  414. McVerry BA, Ross MGR, Knowles WA et al. (1979b) Viral exposure and abnormal liver function in haemophilia. J Clin Pathol 32: 377–381PubMedGoogle Scholar
  415. McVerry BA, Voke J, Vicary FW et al. (1979c) Ultrasonography in the management of hemophilia. Lancet 11: 872–874Google Scholar
  416. Menache D, Aronson DL (1978) Heterogeneity of factor IX in therapeutic factor IX concentrates. Thromb Res 13: 821–828PubMedGoogle Scholar
  417. Menitove JE, Aster RH, Casper JT et al. (1983) T-Lymphocyte subpopulations in patients with classic hemophilia treated with cryoprecipitate and lyophilized concentrates. N Engl J Med 308: 83PubMedGoogle Scholar
  418. Mertens K, Cupers R, Linden IK van der et al. (1983) The functional defect of factor IX Eindhoven, a genetic variant of factor IX. Thromb Haemost 50: 249Google Scholar
  419. Meyer D, Larrieu MJ (1971) Factor VIII and IX variants. Relationship between haemophilia BM and haemophilia B~. Eur J Clin Invest 1: 425Google Scholar
  420. Meyer D, Bidwell E, Larrieu MJ (1972) Crossreacting material in genetic variants of haemophilia B.J Clin Pathol 25: 433Google Scholar
  421. Meyer D, Pías A, Allain JP et al. (1975) Problems in the detection of carriers of haemophilia A. J Clin Pathol 28: 690PubMedGoogle Scholar
  422. Mibashan RS, Rodeck CH, Thumpston JK et al. (1979) Plasma assay of fetal factors VIII and IX for prenatal diagnosis of hemophilia. Lancet 1: 1309–1311PubMedGoogle Scholar
  423. Mibashan RS, Peake IR, Rodeck CH et al. (1980) Dual diagnosis of prenatal hemophilia by measurement of fetal factor VIII C and VIII C antigen. Lancet 11: 994–997Google Scholar
  424. Mibashan RS, Rodeck CH, Holmberg L et al. (1981a) Prenatal diagnosis of hemophilia B by assay of fetal factor IXC and IXAg ( IRMA ). Thromb Haemost 46: 167Google Scholar
  425. Mibashan RS, Peake IR, Newcombe RG et al. (1981b) Carrier detection of hemophilia A in pregnancy by measurement of factor VIIIC/RAg and VIIICAg/RAg ratios. Thromb Haemost 46: 187Google Scholar
  426. Mibashan RS, Rodeck CH, Thumpston JK (1982) Prenatal diagnosis of the hemophilias. Methods in Haematology 5: 197Google Scholar
  427. Middleton SM, Robinson PN, Morton A et al. (1983) The use of a solid phase polyelectrolyte for the reduction of hepatitis B surface antigen and blood group isoagglutinins from a concentrate of human factor VIII. Thromb Haemost 50: 209Google Scholar
  428. Mitchell GA, Abdullahad CM, Ruiz JA et al. (1981) Fluorogenic substrate assays for factors VIII and IX: Introduction of a new solid phase fluorescent detection method. Thromb Res 21: 573–584PubMedGoogle Scholar
  429. Miyoshi K, Sasaki N, Shirakami A et al. (1978) Hereditary persistence of fetal hemoglobin and Xg blood group in hemophilia and von Willebrands disease. Jap J Hum Gen 23:268 Mori PG, Pasino M, Vadala CR et al. (1979) Hemophilia A in a 46,X,I ( XQ) female. Br J Haematol 43: 143Google Scholar
  430. Mörz R, Lechner K, Kundi M (1983) Low incidence of cardiovascular deaths in patients with mild and moderate hemophilia A. Thromb Haemost 50: 209Google Scholar
  431. Mozen MM (1980) The development and use of the coagulation concentrates factor IX (Konyne) and factor VIII (Koate). In: Mammen EF, Barnhart MJ, Lusher JM, Walsh RT (eds) Treatment of bleeding disorders. PJD Publications, Westbury/NYGoogle Scholar
  432. Muller HP, Tilburg NH van, Bertina RM et al. (1980) Immunologic studies on the relationship between FVIII related antigen and FVIII procoagulant activity. Thromb Res 20:85 Muller HP, Tilburg NH van, Derks J et al. (1981) A monoclonal antibody to VIII:C produced by a mouse hybridoma. Blood 58: 1000–1006Google Scholar
  433. Muller HP, Tilburg NH van, Bertina RM et al. (1982) Heterogeneity of haemophilia A: a study with three different antisera. Br J Haematol 52: 485–494PubMedGoogle Scholar
  434. Müller N, Brackmann HH (1983) Lymphocytotoxic antibodies in haemophiliacs. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 183Google Scholar
  435. Muntean W (1981) Hemorrhagic diathesis from transiently acquired factor VIII complex deficiency. J Pediatr 98: 262–264PubMedGoogle Scholar
  436. Myers TJ, Tenbrevilla Zubiri CL, Klatsky AU et al. (1980) Recurrent acute hepatitis following the use of factor VIII concentrates. Blood 55: 748–751PubMedGoogle Scholar
  437. Nagao T, Iizuka A, Hanada R (1983) Treatment with FEIBA of bleeding episodes in hemophilia B patients with inhibitor. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 179Google Scholar
  438. National Blood Resouce Program (1972) Pilot study of hemophilia in the U.S. National institutes of health, department of health, education and welfare. Bethesda, MdGoogle Scholar
  439. Niessner H, Korninger C (1983) DDAVP-an alternative in the management of mild haemophilia A and von Willebrands disease. Wien Klin Wochenschr 21: 753–757Google Scholar
  440. Nilsson IM, Hedner U (1977) Characteristics of various factor VIII concentrates used in treatment of hemophilia A. Br J Haematol 37: 543–557PubMedGoogle Scholar
  441. Nilsson IM, Blombäck M, Thilen A et al. (1959) Carriers of hemophilia A. A laboratory study. Acta Med Scand 165: 357–370PubMedGoogle Scholar
  442. Nilsson IM, Blombäck M, Ramgran O et al. (1962) Heamophilia in Sweden. II. Carriers of hemophilia A and B. Acta Med Scand 171: 223–235Google Scholar
  443. Nilsson IM, Hedner V, Björlin G (1973) Suppression of factor IX antibody in hemophilia B by factor IX and cyclophosphamide. Ann Intern Med 78: 91–95PubMedGoogle Scholar
  444. Nilsson IM, Hedner U, Ahlberg A et al. (1977) Surgery of hemophiliacs–20 years experience. World J Surg 1: 55–68PubMedGoogle Scholar
  445. Nilsson IM, Mikaelsson M, Vilhardt H et al. (1979 a) DDAVP factor VIII concentrate and its properties in vivo and in vitro. Thromb Res 15: 263–271PubMedGoogle Scholar
  446. Nilsson IM, Walter H, Mikaelsson M et al. (1979b) Factor VIII concentrate prepared from DDAVP stimulated blood donor plasma. Scand J Haematol 22: 42–46PubMedGoogle Scholar
  447. Nilsson IM, Kirkwood TBL, Barrowcliffe TW ( 1979 c) In vivo recovery of factor VIII: A comparison of one-stage and two-stage assay methods. Thromb Haemost 42: 1230–1239Google Scholar
  448. Nilsson IM, Holmberg L, Stenbjerg P et al. (1980a) Characteristics of factor VIII protein and factor XIII in various factor VIII concentrates. Scand J Haematol 24: 340PubMedGoogle Scholar
  449. Nilsson IM, Holmberg L, Aberg M et al. (1980b) The release of plasminogen activator and factor VIII after injection of DDAVP in healthy volunteers and in patients with von Willebrands disease. Scand J Haematol 24: 351–359Google Scholar
  450. Nilsson IM, Jonsson S, Sundquist SB (1981) A procedure for removing high titer antibodies by extracorporeal protein A. Sepharose adsorption in hemophilia: Substitution therapy and surgery in a patient with hemophilic B and antibodies. Blood 58: 38Google Scholar
  451. Nilsson IM, Mikaelsson M, Vilhardt H (1982) The effect of intranasal DDAVP on coagulation and fibrinolytic activity in normal persons. Scand J Haematol 29: 70–74PubMedGoogle Scholar
  452. Nordfang O, Ezban M, Dinesen B (1983) Development of a simple and sensitive ELISA for factor VIII: CAg. Thromb Haemost 50: 111Google Scholar
  453. Norkrans G, Widell A, Teger-Nilsson AC et al. (1981) Acute hepatitis Non-A, Non-B following administration of factor VIII concentrates. Vox Sang 41: 129–133PubMedGoogle Scholar
  454. Nossel HL, Archer RK, MacFarlane RG (1962) Equine haemophilia: report of a case and its response to multiple infusions of heterospecific AHG. Br J Haematol 8: 335PubMedGoogle Scholar
  455. Nossel HL, Lanzkowsky P, Levy S et al. (1966) A study of coagulation factor levels in women during labour and in their newborn infants. Thromb Diath Haemorr 16: 185–197Google Scholar
  456. Nowotny C, Wutka P (1980) Anwendung von Fibrinkleber zur Blutstillung nach Zahnextraktionen bei Patienten mit angeborenen und erworbenen Gerinnungsstörungen. In: Schimpf K (Hrsg) Fibrinogen, Fibrin und Fibrinkleber. Schattauer, Stuttgart New YorkGoogle Scholar
  457. Nowotny C, Niessner H, Thaler E et al. (1976) Sonography: A method for localization of hematomas in hemophiliacs. Haemostasis 5: 129PubMedGoogle Scholar
  458. Nowotny C, Lechner K, Niessner H et al. (1979) Effect of in vivo administration of FEIBA on clotting tests. Dose-response relationship. Thromb Haemost 42: 199Google Scholar
  459. Noyes CM, Griffith MJ, Roberts HR, Lundblod RL (1983) Identification of the molecular defect in factor IX Chapel Hill, substitution of histidine for arginine at position 145. Proc Natl Acad Sci USA 80: 4200PubMedGoogle Scholar
  460. O’Brien PF, North WRS, Ingram GIC (1981) The diagnosis of mild haemophilia by the partial thromboplastin time test. WFH/ICTH study of the Manchester method. Thromb Hemost 45 /2: 162–168Google Scholar
  461. Oldenburger D, Gundlach W (1977) Intramural esophageal hematoma in a hemophiliac. An unusual cause of gastrointestinal bleeding. JAMA 237 /8: 800PubMedGoogle Scholar
  462. Onder O, Hoyer LW (1979) Factor VIII coagulant antigen in factor IX complex concentrates. Thromb Res 15: 569–572PubMedGoogle Scholar
  463. Orringer EP, Koury MJ, Blatt PM et al. (1976) Hemolysis caused by factor VIII concentrates. Arch Intern Med 136: 1018–1020PubMedGoogle Scholar
  464. Orstavik KH (1979) Electroimmunoassay of factor IX antigen: Increased sensitivity by enzyme amplification of immunoprecipitates. Thromb Res 15: 721–726PubMedGoogle Scholar
  465. Orstavik KH (1981) Alloantibodies to factor IX in haemophilia B characterized by crossed immuno-electrophoresis and enzymeconjugated antisera to human immunoglobulins. Br J Haematol 48:15 Orstavik KH, Laake K (1978) Antiserum against factor IX shortens the bovine thromboplastin coagulation time of human plasma. Thromb Res 12: 455–466Google Scholar
  466. Orstavik KH, Nilsson IM (1978) A study of acquired inhibitors of factor IX by means of precipitating rabbit antisera against factor IX. Thromb Res 12: 863–874PubMedGoogle Scholar
  467. Orstavik KH, Osterud B, Prydz H et al. (1975) Electroimmunoassay of factor IX in hemophilia B. Thromb Res 7: 373PubMedGoogle Scholar
  468. Orstavik KH, Stormorken H (1983) Hemophilia B+ in a woman. In: Blomback M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 118Google Scholar
  469. Orstavik KH, Veltkamp JJ, Bertina RM et al. (1979) Detection of carriers of haemophilia B. Br J Haematol 42: 293PubMedGoogle Scholar
  470. Osterud B, Kasper CK, Prodanos C (1979) Factor IX variants of hemophilia B. The effect of activated factor XI and the reaction product of factor VII and tissue factor on the abnormal factor IX molecules. Thromb Res 15: 235–243Google Scholar
  471. Osterud B, Kasper CK, Lavine KK et al. (1981) Purification and properties of an abnormal blood coagulation factor IX (Factor IX BM)/Kinetics of its inhibition of factor X activation by factor VII and bovine tissue factor. Thromb Haemost 45 (1): 55–59PubMedGoogle Scholar
  472. Otto JC (1803) An account of an hemorrhagic disposition existing in certain families. Med Repos 6: 1Google Scholar
  473. Palma AF de, Cotler J (1956) Hemophilic arthropathy. Clin Orthop 8: 163–190Google Scholar
  474. Panicucci F, Sagripanti A, Conte B et al. (1981) Inhibitor to factor IX following activated prothrom-bin-complex concentrate treatment. Thromb Haemost 45: 96PubMedGoogle Scholar
  475. Parekh VR, Mannucci PM, Ruggeri ZM (1978) Immunological heterogeneity of haemophilia B: a multicentre study of 98 kindreds. Br J Haematol 40: 643–655PubMedGoogle Scholar
  476. Patek AJ, Taylor FHL (1937) Hemophilia. Some properties of a substance obtained from normal human plasma effective in accelerating the coagulation of hemophilic blood. J Clin Invest 16: 113Google Scholar
  477. Patriavin HJ (1980) Ureteric hemorrhage in hemophilia with rapid healthy. J Can Assoc Radiol 31: 265Google Scholar
  478. Paulssen MMP, Pelt BC van (1981) Oral treatment of haemophilia A by factor VIII bound to chylomicra. Lancet 1310Google Scholar
  479. Peake IR, Bloom AL (1978) Immunoradiometric assay of procoagulant factor VIII antigen in plasma and serum and its reduction in hemophilia. Preliminary studies on adult and fetal blood. Lancet 1: 473Google Scholar
  480. Peake IR, Bloom AL, Giddings JC et al. (1979) An immunoradiometric assay for procoagulant factor VIII antigen. Results in haemophilia, von Willebrands disease and fetal plasma and serum. Br J Haematol 42: 269–281PubMedGoogle Scholar
  481. Peake IR, Furlong BL, Bloom AL (1984) Carrier detection by direct gene analysis in a family with haemophilia B (factor IX deficiency). Lancet 1: 242PubMedGoogle Scholar
  482. Peake IR, Newcombe RG, Davies BL et al. (1981) Carrier detection in haemophilia A by immunological measurement of factor VIII Carrier detection in haemophilia A by immunological measurement of factor VIII related (VIIIRAg) and factor VIII clotting antigen ( VIIICAg ). Br J Haematol 48: 651–660PubMedGoogle Scholar
  483. Pechet L, Tiarks CY, Stevens J et al. (1978) Relationship of factor IX antigen and coagulant in hemophilia B patients and carriers. Thromb Haemost 40: 465–477Google Scholar
  484. Penner JA (1981) Therapeutic approaches to inhibitors: Vitamin K-dependent factor concentrates. In: Seligsohn U, Rimon A, Horoszowski H (eds) Haemophilia. Castle House Publ, London, p 97Google Scholar
  485. Penner JA, Abildgaard CF (1979) Ineffectiveness of certain commercial prothrombin complex concentrates in treatment of patients with inhibitors of factors VIII and IX. N Engl J Med 300: 565–566PubMedGoogle Scholar
  486. Pepper DS, Banhegyi D, Howie A et al. (1977) In vitro thrombogenicity tests of factor IX concentrates. Br J Haematol 36: 573–583PubMedGoogle Scholar
  487. Perez-Bianco BR, Schachter S, Elgue G et al. (1983) Successful effect of FEIBA plus factor IX in a patient with inhibitor to factor IX. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 180Google Scholar
  488. Pettersson H, Ahlberg A, Nilsson IM (1980) A radiologic classification of hemophilic arthropathy. Clin Orthop 149: 153–159PubMedGoogle Scholar
  489. Pike IM, Yount WJ, Puritz EM et al. (1972) Immunochemical characterization of a monoclonal yG4 human antibody to factor IX. Blood 40: 1–10PubMedGoogle Scholar
  490. Poller L, Thomson JM, Palmer MK (1976) Measuring partial thromboplastin time. An international collaborative study. Lancet II: 842–846Google Scholar
  491. Pollmann H, Sutor AH (1983) Anti-factor IX-antibody after treatment with FEIBA. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 181Google Scholar
  492. Pool JG, Robinson J (1959) Observations on plasma banking and transfusion procedures for haemo-philic patients. Using a quantitative assay for antihaemophilic globulin ( AHG ). Br J Haematol 5: 24–30Google Scholar
  493. Pool JG, Shannon AE (1965) Production of high purity concentrates of anti-hemophilic globulin in a closed bag system. N Engl J Med 273: 1443–1447PubMedGoogle Scholar
  494. Poon MC, Ratnoff OD (1977) Immunologic evidence that the antihemophilic plasma possesses a nonfunctional low molecular weight subcomponent. Blood 50: 367PubMedGoogle Scholar
  495. Poon MC, Landay A, Prasthofer EF et al. (1983) Acquired immunodeficiency syndrome with Pneumocystis carinii pneumonia and mycobacterium avium-intracellulare infection in a previously healthy patient with classic hemophilia. Ann Intern Med 98: 287–290PubMedGoogle Scholar
  496. Prager D, Djerassi I, Eyster E et al. (1979) Pennsylvania state-wide hemophilia program: Summary of immediate reactions with the use of factor VIII and factor IX concentrate. Blood 53: 1012–1013PubMedGoogle Scholar
  497. Prentice CRM, Lindsay RM, Barr RD et al. (1971) Renal complications in haemophilia and Christmas disease. Q J Med 40: 47PubMedGoogle Scholar
  498. Prentice CRM, Forbes CD, Morrice S et al. (1975) Calculation of predictive odds for possible carriers of haemophilia. Thromb Diath Haemorr 34: 740Google Scholar
  499. Preston FE, Malia RG, Lilleyman JS et al. (1977) Heparinised clotting factor concentrates in patients with Christmas disease and liver disease. Thromb Haemost 38: 504–509PubMedGoogle Scholar
  500. Preston FE, Triger DR, Underwood JCE et al. (1978) Percutaneous liver biopsy and chronic liver disease in hemophiliacs. Lancet 2: 592–594PubMedGoogle Scholar
  501. Prowse CV, Cash JD (1981) The use of factor IX concentrates in man: a 9-year experience of scottish concentrates in the southeast of Scotland. Br J Haematol 47: 91PubMedGoogle Scholar
  502. Prowse CV, Williams AE (1979) A non-stasis rabbit model for the detection of factor IX concentrate thrombogenicity. Thromb Haemost 42: 199Google Scholar
  503. Prowse CV, Williams AE (1980) A comparison of the in vitro and in vivo thrombogenic activity of factor IX concentrates using stasis ( Wessler) and non-stasis rabbit models. Thromb Haemost 44: 81Google Scholar
  504. Prowse CV, Sas G, Gader AMA et al. (1979) Specificity in the factor VIII response to vasopressin infusion in man. Br J Haematol 41: 437–447PubMedGoogle Scholar
  505. Quick AJ (1935) The prothrombin in hemophilia and in obstructive jaundice. J Biol Chem 109: 23Google Scholar
  506. Rabiner SF, Telfer MC (1970) Home transfusion for patients with hemophilia A. N Engl J Med 283: 1011–1015PubMedGoogle Scholar
  507. Ramgren O (1962) Haemophilia in Sweden. Acta Med Scand 171: 237PubMedGoogle Scholar
  508. Ramsay DM, Khoo KK (1975) A five-year study of a hemophilia reference center. J Clin Pathol 28: 696PubMedGoogle Scholar
  509. Rapaport SI, Patch MJ, Moore FJ (1960) Antihemophilic globulin levels in carriers of hemophilia. J Clin Invest 39: 1619PubMedGoogle Scholar
  510. Rasche H, Bindewald H, Köhle W et al. (1977) Notfallbehandlung von Blutungskomplikationen bei Hemmkorper-Hamophilie mit aktivierten Prothrombinkomplex-Konzentraten. Dtsch Med Wochenschr 102: 319–323PubMedGoogle Scholar
  511. Ratnoff OD, Jones PK (1977) The laboratory diagnosis of the carrier state for classic haemophilia. Ann Intern Med 36: 521Google Scholar
  512. Ratnoff OD, Lewis JH (1975) Heckathorns disease: variable functional deficiency of antihemophilic factor (factor VIIIC). Blood 46: 161PubMedGoogle Scholar
  513. Ratnoff OD, Menitove J (1983) Coincident classic hemophilia and “idiopathic” thrombocytopenic purpura in patients under treatment with concentrates of antihemophilic factor (factor VIII). N Engl J Med 308: 439PubMedGoogle Scholar
  514. Reisner HM (1983) Immunological approaches to the study of the hemophilias. In: Abe T (ed) Proc 3rd Int Symp HT, Tokio. Kyoritsu Printings, Tokyo, pp 19–29Google Scholar
  515. Reisner HM, Roberts HR, Krumholz S et al. (1977) Immunochemical characterization of a polyclonal human antibody to factor IX. Blood 50: 11–19PubMedGoogle Scholar
  516. Reisner HM, Katz HJ, Goldin LR et al. (1978) Use of a simple visual assay of Willebrand factor for diagnosis and carrier identification. Br J Haematol 40: 339PubMedGoogle Scholar
  517. Reisner HM, Barrow ES, Graham JB (1979) Radioimmunoassay for coagulant factor VHI-related antigen. Thromb Res 14: 235–239PubMedGoogle Scholar
  518. Reisner HM, Price WA, Blatt PM et al. (1980a) Factor VIII coagulant antigen in hemophilic plasma: a comparison of five alloantibodies. Blood 56: 615–619PubMedGoogle Scholar
  519. Reisner HM, Strand EA, Chung KS et al. (1980b) An agarose plate method for detecting alloantisera to coagulant factor IX and factor IX antigen. Br J Haematol 44: 313PubMedGoogle Scholar
  520. Rizza CR, Biggs R (1969) Blood products in the management of haemophilia and Christmas disease. In: Poller L (ed) Recent advances in blood coagulation. Churchill Livingstone, London Edinburgh New York, pp 179–195Google Scholar
  521. Rizza CR, Matthews JM (1982) Effect of frequent factor VIII replacement on the level of factor VIII antibodies in hemophiliacs. Br J Haematol 52: 13–24PubMedGoogle Scholar
  522. Rizza CR, Spooner JRD (1977) Home treatment of hemophilia and Christmas disease. Five years experience. Br J Haematol 37: 53Google Scholar
  523. Rizza CR, Spooner JRD (1983) Treatment of hemophilia and related disorders in Britain and Northern Ireland during 1976–198, report on behalf of the directors of hemophilia centres in the United Kingdom. Br Med J 286: 929Google Scholar
  524. Rizza CR, Rhymes IL, Austen DEG et al. (1975) Detection of carriers of haemophilia: a “blind” study. Br J Haematol 30: 44Google Scholar
  525. Rizza CR, Kernoff PBA, Matthews JM (1977) A comparison of coagulation factor replacement with and without prednisolone in the treatment of haematuria in haemophilia and Christmas disease. Thromb Haemost 37: 86PubMedGoogle Scholar
  526. Rizzetto M, Morello C, Mannucci PM et al. (1982) Delta infection and liver disease in hemophilic carriers of hepatitis B surface antigen. J Infect Dis 145: 18–22PubMedGoogle Scholar
  527. Roberts HR (1981) Hemophiliacs with inhibitor. Therapeutic options. N Engl J Med 305: 757PubMedGoogle Scholar
  528. Rock GA, Palmer DS, Cruickshank WH ( 1979 a) Visualization of VIII: C-like material in a CRM negative hemophiliac. Thromb Res 16: 747–757Google Scholar
  529. Rock GA, Cruickshank WH, Tackaber ES et al. (1979 b) Improved yields of factor VIII from heparin-ized plasma. Vox Sang 36: 294–300PubMedGoogle Scholar
  530. Rock GA, Cruickshank WH, Palmer DS (1981) Variant forms of procoagulant-like factor VIII n hemophiliacs. Thromb Res 21: 53–63PubMedGoogle Scholar
  531. Rodeck CH (1980) Fetoscopy guided by real-time ultrasound for pure fetal blood samples, fetal skin samples and examination of the fetus in utero. Br J Obstet Gynaecol 87: 449–456PubMedGoogle Scholar
  532. Rodeck CH, Campbell S (1978) Sampling pure fetal blood by fetoscopy in second trimester of pregnancy. Br Med J 2: 728–730PubMedGoogle Scholar
  533. Rohyans JA, Miser AW, Miser JS (1982) Subgaleal hemorrhage in infants with hemophilia: report of two cases and review of the literature. Pediatrics 70: 306–307PubMedGoogle Scholar
  534. Rosen S (1983) Assay of FVIII.C with the chromogenic substrate S–222. In: Blomback M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 25Google Scholar
  535. Rosner F (1969) Hemophilia in the Talmud and Rabbinic writings. Ann Intern Med 70: 833PubMedGoogle Scholar
  536. Rotblat F, Tuddenham EGD (1981) Immunologic studies of factor VIII coagulant activity (VIII:C) 1. Assays based on a haemophilic and an acquired antibody to VIII: C. Thromb Res 21: 431–445Google Scholar
  537. Rubin R, Niemetz J, Estren S (1975) Use of animal AHG concentrates (factor VIII) in the treatment of life-threatneing hemorrhage in patients with factor VIII antibodies. Recent advances in hemophilia 240: 362Google Scholar
  538. Ruggeri ZM, Mannucci PM, Lombardi R et al. (1982) Multimene composition of factor VIII/von Willebrand factor following administration of DDAVP: Implications for pathophysiology and therapy of von Willebrands disease subtypes. Blood 59: 1272–1278PubMedGoogle Scholar
  539. Saito H, Shioya M, Kamiya T et al. (1969) Congenital combined deficiency of factor V and factor VIII. A case report and the effect of transfusion of normal plasma and hemophilic blood. Thromb Diath Haemorr 22: 316–325Google Scholar
  540. Samama M et al. (1977) Haemophilia A in a girl with deletion of a part of the long arm of one X chromosome. Pathol Biol 25: 10–17PubMedGoogle Scholar
  541. Sandler SG, Rath CE, Wickerhauser M et al. (1973) Post-Konyne hepatitis: The ineffectiveness of screening for the hepatitis B antigen ( HBAg ). Transfusion 13: 221PubMedGoogle Scholar
  542. Sas G, Owens RE, Smith JK et al. (1975) In vitro spontaneous thrombin generation in human factor IX concentrates. Brit J Haematol 31: 25–35Google Scholar
  543. Savidge GF (1983) Current situation of home care in the United States. Proc 3rd Int Symp HT, Tokio, p 191Google Scholar
  544. Scharrer I (1979) Klinische Erfahrungen und gerinnungsanalytische Untersuchungen nach Gabe von DD AVP bei Patienten mit von Willebrand Syndrom, milder und Subhamophilie sowie bei einer blutenden Konduktorin der Hämophilie. In: Sutor AH (Hrsg) DDAVP in bleeding disorders. 1st Int Symp on DDAVP in bleeding disorders, Münster 1979. Schattauer, Stuttgart New YorkGoogle Scholar
  545. Scharrer I (1982) Comparison between combined treatment and treatment with activated prothrombin complex preparations alone. In: Mariani G, Russo MA, Mandelli F (eds) Activated prothrombin complex concentrates. Praeger Pubi, New York, p 216Google Scholar
  546. Schellong G, Sutor AH (1980) Hämolytische Anämie durch ein Anti-D-haltiges AHG-Präparat. In: Schimpf K (Hrsg) Fibrinogen, Fibrin und Fibrinkleber. Schattauer, Stuttgart New York, S 335–337Google Scholar
  547. Schimpf K (1977) Substitutionsbehandlung bei Hämophilie. Akute Maßnahmen und Komplikatio-nen. Wien Med Wochenschr 127: 329–337PubMedGoogle Scholar
  548. Schimpf K (1980) Hämophilie und Hepatitis. Die gelben Hefte, XX. Jahrgang 4: 159Google Scholar
  549. Schimpf K (1981) The status of supervised selftreatment in hemophilia management. In: Abe T (ed) Proc 2nd Int Symp HT, Tokyo, Kyoritsu Printings, Tokyo, pp 41–57Google Scholar
  550. Schimpf K, Baumann P (1976) Die ambulante Dauerbehandlung der Hämophilie B. Eine kontrollierte Studie. Dtsch Med Wochenschr 101: 233–238Google Scholar
  551. Schimpf K, Rothmann P (1980) Behandlung mit DDAVP während 18 Zahnextraktionen, 2 Muskelblutungen, 1 Bisswunde und einer Kieferhöhlenspülung bei insgesamt 8 Patienten mit milder Hämophilie A. In: Sutor AH (ed) DDAVP in bleeding disorders. 1st Int Symp on DDAVP in bleeding disorders, Münster 1979. Schattauer, Stuttgart New York, S 131Google Scholar
  552. Schimpf K, Rothmann P (1971) In vitro-und in vivo-Vergleich von Faktor VIII Konzentraten. Blut 42: 129–130Google Scholar
  553. Schimpf K, Westphal B (1981) In vitro activities and in vivo recovery of coagulation factor IX concentrates with a hepatitis-safe concentrate. Haemostasis 10 /1: 214Google Scholar
  554. Schimpf K, Fischer B, Rothmann P (1976 a) Die ambulante Dauerbehandlung der Hämophilie A. Dtsch Med Wochenschr 101: 141–148PubMedGoogle Scholar
  555. Schimpf K, Hellstern P, Scharrer I et al. (1983) Qualität von Faktor-VIII-Konzentraten. Dtsch Med Wochenschr 14: 560–561Google Scholar
  556. Schimpf K, Zimmermann K, Köpf B (1976 b) DIC and postoperative wound bleeding under factor IX substitution therapy in a case of hemophilia B, successful treatment with heparin. Thromb Res 8: 65–70Google Scholar
  557. Schimpf K, Rothmann P, Zimmermann K ( 1976 c) Factor VIII dosis in prophylaxis of hemophilia A, a further controlled study. In: Abe T (ed) Proc XI. Congr WFH. Academia Press, Tokyo, pp 365–366Google Scholar
  558. Schimpf K, Zimmermann K, Thamer G (1976d) Hepatitishäufigkeit, HB-Antigen-Frequenz bei Patienten des Hämophiliezentrums Heidelberg. Verh Dtsch Ges Inn Med 82: 414–417Google Scholar
  559. Schimpf K et al. (1977) Hemophilia A prophylaxis with factor VIII concentrate in a home-treatment Programm: a controlled study. Scand J Haematol 30: 79–80Google Scholar
  560. Schimpf K, Schumacher K, Zeltsch C et al. (1981a) Faktor Vlll-Standards und ihre Anwendung auf klinisch eingesetzte Faktor VIII-Konzentrate. 12. Hämophilie Symp, Hamburg, S 1–11Google Scholar
  561. Schimpf K, Zimmermann K, Bleyl U et al. ( 1981 b) Liver biopsy findings in hemophilia. In: Seligsohn U, Rimon A, Horoszowski H (eds) Hemophilia. Castle House Publ, London, p 149Google Scholar
  562. Schimpf K, Zeltsch C, Zeltsch P (1982) Myocardial infarction complicating activated prothrombin complex concentrate substitution in patient with haemophilia A. Lancet II: 1043Google Scholar
  563. Schramm W, Marx R, Ackermann et al. (1976) Derzeitiger Stand der Lokalblutstillung mit dem Neodym-Yag-Laser bei blutungsgefährdeten Patienten. In: Landbeck G, Marx R (Hrsg) 7. Hämophilie Symp, Hamburg 1976. Global, HeidelbergGoogle Scholar
  564. Schramm W, Marx R, Ackermann K (1978) Infrarot-Koagulation zur Lokalblutstillung blutungsge-fährdeter Patienten. In: Landbeck G, Marx R (Hrsg) 8. Hämophilie Symp, Hamburg. 1977. Global, Heidelberg, S 239–240Google Scholar
  565. Schramm W, Frösner GG, Scheid R et al. (1979) Hepatitis B und Nicht-A-Nicht B-Hepatitis bei Hämophilen. Blut 38: 72Google Scholar
  566. Schwägerl W, Bosch P, Niessner H et al. (1981) Der hämophile Pseudotumor und seine orthopädischchirurgische Behandlung. In: Landbeck G, Marx R, Stolte HP (Hrsg) 10. Hämophilie Symp, Hamburg, 1979. Pharm. Verlagsgesellschaft, München, S 248–253Google Scholar
  567. Seeler RA, Telischi M, Lengehennig PL et al. (1976) Comparison of anti-A and anti-B titers in factor VIII and IX concentrates. J Pediatr 89: 87PubMedGoogle Scholar
  568. Seligsohn U (1973) Hemophilia and other clotting disorders. Isr J Med Sei 9: 1338Google Scholar
  569. Seligsohn U, Kasper CK, Osterud B et al. ( 1979 a) Activated factor VII: Presence in factor IX concentrates and persistence in the circulation after infusion. Blood 53: 828PubMedGoogle Scholar
  570. Seligsohn U, Zivelin A, Perez C et al. (1979 b) Detection of hemophilia A carriers by replicate factor VIII activity and factor VIII antigenicity determination. Br J Haematol 42: 433–439PubMedGoogle Scholar
  571. Seligsohn U, Zivelin A, Zwang E (1982) Combined factor V and factor VIII deficiency among non-Ashkenazi jews. N Engl J Med 307: 1191Google Scholar
  572. Shapiro SS (1979) Antibodies to blood coagulation factors. Clin Haematol 8: 207PubMedGoogle Scholar
  573. Shapiro SS, Holburn RR (1970) Pathophysiology of anticoagulants in hemophilia A and B. In: Brinkhous KM (ed) Hemophilia and new hemorrhagic states. University of North Carolina Press, Chapel Hill, pp 141–151Google Scholar
  574. Shapiro SS, Hultin M (1975) Acquired inhibitors to the blood coagulation factors. Sem Thromb Haemostas 1: 336–385Google Scholar
  575. Shen MC (1982) A comparative study of carrier detection in haemophilia A by linear discriminant unction. Br J Haematol 52: 283–293PubMedGoogle Scholar
  576. Sherman LA, Gaston LW, Kaplan ME et al. (1972) Fibrinogen St. Louis: A new inherited fibrinogen variant, coincidentally associated with hemophilia A. J Clin Invest 51: 590–597PubMedGoogle Scholar
  577. Simeone JF, Robinson F, Rothman SLG et al. (1977) Computerized tomographic demonstration of a retroperitoneal haematoma causing femoral neuropathy. Report of two cases. J Neurosurg 47: 946PubMedGoogle Scholar
  578. Silverstein A (1960) Intracranial bleeding in hemophilia. Arch Neurol 3: 141PubMedGoogle Scholar
  579. Sjamsoedin LJM, Heijnen L, Mauser-Bunschoten EP et al. (1981) The effect of activated prothrom-bin-complex concentrate (FEIBA) on joint and muscle bleeding in patients with hemophilia A and antibodies to factor VIII: a double-blind clinical trial. N Engl J Med 305: 717–721PubMedGoogle Scholar
  580. Small M, Lowe GDO, Douglas JT et al. ( 1982 a) Factor IX thrombogenicity: In vivo effects on coagulation activation and a case report of disseminated intravascular coagulation. Thromb Hae-most 48: 76–77PubMedGoogle Scholar
  581. Small M, Rose PE, McMillan N et al. (1982b) Haemophilia and the kidney. Br Med J 285:1609 Smith JK, Bidwell E (1979) Therapeutic material used in the treatment of coagulation defects. Clin Haematol 8: 185Google Scholar
  582. Smith KJ, Thompson AR (1981) Labeled factor IX kinetics in patients with hemophilia B. Blood 58: 625–629PubMedGoogle Scholar
  583. Smith PS, Keyes NC, Forman EN (1982) Socioeconomic evaluation of a state-funded comprehensive hemophilia-care program. N Engl J Med 306: 575–579PubMedGoogle Scholar
  584. Soff GA, Levin J (1981) Familial multiple coagulation factor deficiencies: 1. Review of the literature: differentiation of single hereditary disorders associated with multiple factor deficiencies from coincidental concurrence of single factor deficiency states. Semin Thromb Hemost 7: 112PubMedGoogle Scholar
  585. Soff GA, Levin J, Bell WR (1981) Familial multiple coagulation factor deficiencies: II. Combined factor VIII, IX and XI deficiency and combined factor IX and XI deficiency: Two previously uncharacterized familial multiple factor deficiency syndromes. Semin Thromb Hemost 7: 149Google Scholar
  586. Spero JA, Lewis JH, Thiel DH van et al. (1978 a) Asymptomatic structural liver disease in hemophilia. N Engl J Med 298: 1373–1378PubMedGoogle Scholar
  587. Spero JA, Lewis JH, Thiel DH van et al. (1978 b) Liver disease in haemophiliacs. Lancet 11: 937Google Scholar
  588. Stanievich JF et al. (1980) Airway obstruction in a hemophilic child. Am Obi Blinol Laryng 89: 572Google Scholar
  589. Stein H (1981) Intracellular iron deposits and cell necrosis in haemophilic synovium and articular cartilage. In: Seligsohn U, Rimon A, Horoszowski H (eds) Haemophilia. Castle House Pubi, London, p 159Google Scholar
  590. Stein RS, Coiman RW (1973) Hemophilia with factor VIII inhibitor. Elimination of anamnestic response. Ann Intern Med 79: 84–87Google Scholar
  591. Steinbuch M, Boffa MC, Pejaudier L et al. (1983) Protein composition of prothrombin complex concentrates. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 215Google Scholar
  592. Stenbjerg S, Jörgensen J (1978) Activated F IX concentrate ( FEIBA) used in the treatment of haemophilic patients with antibody to F VIII. Acta Med Scand 203: 471–477Google Scholar
  593. Stenbjerg S, Jorgensen J (1977) Resistance to activated F IX concentrate (FEIBA). Scand J Haematol 18 /5: 421–426PubMedGoogle Scholar
  594. Stenbjerg S, Tauris P, Skottun T et al. (1980) Total dental extraction in a patient with F VIII inhibitor. Thromb Res 18: 889PubMedGoogle Scholar
  595. Stenbjerg S, Jorgensen J, Tauris P et al. (1982) Low dose factor VIII for the treatment of hemophilia with inhibitors. In: Mariani G, Russo MA, Mandelli F (eds) Activated prothrombin complex concentrates. Praeger Pubi, New York, p 206Google Scholar
  596. Stephan WU, Kotitschke R (1977) Prothrombinkomplex ( PPSB) aus kaltsterilisiertem Plasma. Forsch Transfusion Immunohäm 4: 72Google Scholar
  597. Stephan WU, Prince AM, Kotitschke R (1982) Faktor VIII-Konzentrat aus kaltsterilisiertem Humanplasma. In: Loo J van de, Asbeck F (eds) Hämostase, Thrombophilie und Arteriosklerose. Schattauer, Stuttgart New York, p 774Google Scholar
  598. Stevenson AC, Kerr CB (1967) On the distribution of frequencies of mutation to genes determining harmful traits in man. Mutat Res 4: 339–352PubMedGoogle Scholar
  599. Stirling M, Prescott RJ (1979) Minimum effective dose of intermediate factor VIII concentrate in haemophilics on home therapy. Lancet 1: 813–814PubMedGoogle Scholar
  600. Stout C et al. (1973) Fatal nontraumatic splenic rupture in hemophilia and the Kasabach-Merrit-Syndrome. South Med J 66: 791–795PubMedGoogle Scholar
  601. Strauss HS (1970) Problems related to anticoagulants in hemophiliacs. Bibl Haematol 34: 149–153PubMedGoogle Scholar
  602. Strauss HS, Kevy SV, Diamond LK (1965) Ineffectiviness of prophylactic epsilon aminocaproic acid in severe hemophilia. N Engl J Med 229: 116–122Google Scholar
  603. Sultan Y, Maisonneuve P (1977) Incidence des inhibiteurs du facteur VIII dans la population des hemophiles française. Nouv Rev Fr Hematol 18: 671–673Google Scholar
  604. Sultan Y, Brouet JC, Debre P (1974) Treatment of inhibitors to factor VIII with activated prothrombin concentrate. N Engl J Med 291: 1087PubMedGoogle Scholar
  605. Sultan Y, Maisonneuve P, Sylvestre R (1982) Plasma exchange in the treatment of hemophiliacs with antibodies to factor VIII. In: Mariani G, Russo MA, Mandelli F (eds) Activated prothrombin complex concentrates. Praeger Pubi, New York, p 55Google Scholar
  606. Suomela H, Myllyä G, Raaska E (1977) Preparation and properties of a therapeutic factor IX concentrate. Vox Sang 33: 37PubMedGoogle Scholar
  607. Sutor AH (1980) Hämostatische Nebenwirkungen der Substitutions-Therapie mit Plasma und Plasmakonzentration. In: Schimpf K (Hrsg) Fibrinogen, Fibrin und Fibrinkleber. Schattauer, Stuttgart New York, S 339–345Google Scholar
  608. Sutor AH, Jesdinsky-Buscher C (1975) Blutung bei Hämophilie unter massiver Therapie mit antihä-mophilem Globulin. Dtsch Med Wochenschr 100: 1183PubMedGoogle Scholar
  609. Sutor AH, Jesdinsky-Buscher C (1976) Blutungszeitveränderungen bei Hämophilie-Behandlung mit lyophilisiertem antihämophilem Globulin ( AHG ). Dtsch Med Wochenschr 101: 1715Google Scholar
  610. Suzuki LA, Thompson AR (1982) Factor IX antigen by a rapid staphylococcal protein A-membrane binding radioimmunoassay: results in haemophilia B patients and carriers and in fetal samples. Br J Haematol 50: 673PubMedGoogle Scholar
  611. Swanton M (1959) Hemophilic arthropathy in dogs. Lab Invest 8: 1269PubMedGoogle Scholar
  612. Tabor E, Aronson DL, Gerety RJ (1980) Removal of hepatitis B-virus infectivity from factor IX complex by hepatitis B immune-globulin. Lancet II: 68Google Scholar
  613. Takamatsu J, Kamiye T, Ogata K et al. (1983) Sensitive solid phase enzyme immunoassay for factor IX antigen and classification of hemophilia B. Haemostasis 13: 9–16PubMedGoogle Scholar
  614. Takeda R, Mabuchi H (1974) A massive pulmonary hemorrhage resulting in cavitation occurring in a case of hemophilia A associated with diabetes mellitus. South Med J 67: 869PubMedGoogle Scholar
  615. Telfer MC, Chediak J, Solarksi A et al. (1983) Pulmonary function in hemophiliacs receiving commercial factor VIII concentrates. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 190 (abstr)Google Scholar
  616. Tezanos PM, Fernandez J, Zirulnik J et al. (1983) Central nervous system hemorrhages (CNSH) in hemophilic patients. Analysis of 101 episodes. In: Blombäck M (ed) Proc Congr WFH, Stockholm. Karger, Basel, p 17Google Scholar
  617. Theiss W, Schmidt G (1978) DDAVP in von Willebrands disease: repeated administration and he behaviour of the bleeding time. Thromb Res 13: 1119PubMedGoogle Scholar
  618. Theodorsson B, Hedner U, Nilsson IM et al. (1983) A technique for specific removal of factor IX alloantibodies from human plasma: partial characterization of the alloantibodies. Blood 61: 973–981PubMedGoogle Scholar
  619. Thomas P, Hepburn B, Kimand HC et al. (1982) Nonsteroidal antiinflammatory drugs in the treat-ment of hemophilic arthropathy. Am J Haematol 12: 131–137Google Scholar
  620. Thomas W, McDonald DI (1981) Potency determination of antiinhibitor coagulant complex-Autoplex. Haemostasis 10: 216Google Scholar
  621. Thompson AR (1977 a) Factor IX antigen by radioimmunoassay. Abnormal factor IX protein in patients on warfarin therapy and with hemophilia B. J Clin Invest 59: 900Google Scholar
  622. Thompson AR (1977 b) Factor IX antigen by radioimmunoassay in heterozygotes for hemophilia B. Thromb Res 11:193–203Google Scholar
  623. Thomson C, Forbes CD, Prentice CRM (1973) Relationship of factor VIII to ristocetin-induced platelet aggregation. Effect of heterologous and acquired factor VIII antibodies. Thromb Res 3: 363–372Google Scholar
  624. Tiarks CY, Pechet L (1981) Immunologic survey of the factor IX molecule in hemophilia B + patients, carriers and cord blood. Thromb Res 21: 391–398PubMedGoogle Scholar
  625. Toole J J, Knopf JL, Wozney JM et al. (1984) Molecular cloning of a cDNA encoding human antihaemophilic factor. Nature (Lond) 312: 342Google Scholar
  626. Tran TH, Duckert F (1983) Preparation of factor VHI-free plasma by immunoaffinity chromatography on insolubilized antibodies against factor VHI-related antigen. Haemostasis 13: 73PubMedGoogle Scholar
  627. Tran TH, Marbet GA, Duckert F (1981) Rabbit antibodies against the procoagulant activity (VIII: C) of human factor VIII. Thromb Haemost 46: 699–705Google Scholar
  628. Trotsenburg L van (1975) Neurological complications of haemophilia. In: Brinkhous KM, Hemker HC (eds) Handbook of Haemophilia. Excerpta Medica, AmsterdamGoogle Scholar
  629. Tsukada T, Ito M, Toida K, Matsuoka M (1976) Gastrointestinal bleeding in Hemophilia: Clinical study. In: Abe T (ed) Proc Xlth Congr WFH, Kyoto. Academia Press, Tokyo, pp 367–371Google Scholar
  630. Tuddenham EGD, Lane RS, Rotblat F et al. (1982) Response to infusions of polyelectrolyte fractionated human factor VIII concentrate in human haemophilia A and von Willebrands disease. Br J Haematol 52: 259–267PubMedGoogle Scholar
  631. Tullis JL, Melin M, Jurigian P (1965) Clinical use of human prothrombin complexes. N Engl J Med 273: 667–674PubMedGoogle Scholar
  632. Valderrama JA, Matthews JM (1965) The haemophilic pseudotumor or hemophilic subperiosteal haematoma. J Bone Joint Surg [Br] 47: 256–265Google Scholar
  633. Vehar GA, Keyt B, Eaton D et al. (1984) Structure of human factor VIII. Nature (Lond) 313:337 Velikay M, Kundi M, Nowotny C et al. (1981) Epidemologische Untersuchungen über die Lebenserwartung von Hämophilen. 12. Hämophilie Symposium HamburgGoogle Scholar
  634. Veitkamp JJ, Meilof J, Remmelts HG et al. (1970) Another genetic variant of haemophilia B: Haemophilia B Leyden. Scand J Haematol 7: 82Google Scholar
  635. Veltkamp JJ, Schrijver G, Willeumier W et al. (1974) Hemophilia in the Netherlands. Acta Med Scand [Suppl] 572: 1Google Scholar
  636. Vermylen J, Schetz J, Semeraro N et al. (1978) Evidence that activated prothrombin concentrates enhance platelet coagulant activity. Br J Haematol 38: 235PubMedGoogle Scholar
  637. Verroust F, Allain JP (1982) Immune response induced by porcine factor VIII in severe hemophiliacs with antibody to factor VIII. Thromb Res 48: 238Google Scholar
  638. Verstraete M (1977) Hemophilia home treatment in Belgium. Scand J Haematol 31: 29–33Google Scholar
  639. Verstraete M, Vermylen J (1975) Laboratory and clinical evaluation of concentrates for treatment of hemophilia A and B. Acta Clin Belg 30: 5Google Scholar
  640. Vigano S, Cattaneo M, Gervasoni W et al. (1980) Increased fibrinopeptide A after prothrombin complex concentrates. Thromb Haemost 44: 72PubMedGoogle Scholar
  641. Vincente V, Alberca I, Borrasca AL (1983) Inhibitor of protein C and combined deficiency of factors V and VIII. Br J Haematol 53: 686Google Scholar
  642. Wahlberg T, Blomback M, Brodin U (1982) Carriers and noncarriers of haemophilia A: I. Multivariate analysis of pedigree data, screening blood coagulation tests and factor VIII variables. Thromb Res 25: 401Google Scholar
  643. Wall RL, McConnell J, Moore D et al. (1967) Christmas disease, color-blindness and blood group Xga. Am J Med 43: 214PubMedGoogle Scholar
  644. Wallis J, Kaick G van, Schimpf K et al. (1981) Ultraschalldiagnostik von Muskelhamatomen bei Hamophiliepatienten. ROFO 2: 153–156Google Scholar
  645. Walsh PN, Rizza CR, Matthews JM et al. (1971) Epsilon-aminocaproic acid therapy for dental extractions in haemophilia and Christmas disease: a double blind controlled trial. Br J Haematol 20: 463PubMedGoogle Scholar
  646. Walsh PN, Rizza CR, Evans BE et al. (1975) The therapeutic role of epsilon-aminocaproic acid ( EACA) for dental extractions in hemophiliacs. Ann NY Acad Sci 240: 267PubMedGoogle Scholar
  647. Warner Al, Lusher JM (1983) DDAVP: A useful alternative to blood components in moderate hemophilia A and von Willebrand disease. J Pediatr 102: 228Google Scholar
  648. Weiss AE (1977) Doses of factor VIII for hemophilic bleeding (letter). N Engl J Med 297: 1238–1238Google Scholar
  649. Weiss AE, Webster WP, Strike LE et al. (1976) Survival of transfused factor VIII in hemophilic patients treated with epsilon aminocaproic acid. Transfusion 16: 209PubMedGoogle Scholar
  650. Wensley RT, Burn AM (1983) Induction of tolerance to factor VIII in haemophilia A with factor III inhibitors using moderate doses of factor VIII. Thromb Haemost 50: 271Google Scholar
  651. Wenzel E, Encica M, Nienhaus K et al. (1982) Prophylactic plasmapheresis and combined treatment with factor VIII in hemophiliacs with antibodies to factor VIII. In: Mariani G, Russo MA, Mandelli F (eds) Activated prothrombin complex concentrates. Praeger Publ, New York, p 60Google Scholar
  652. White GC, Roberts HR, Kingdon HS et al. (1977) Prothrombin complex concentrates: potentially thrombogenic materials and clues to the mechanism of thrombosis in vivo. Blood 49: 159–170PubMedGoogle Scholar
  653. White GC, McMillan CW, Blatt PM et al. (1982a) Factor VIII inhibitors: A clinical overview. Am J Haematol 335–342Google Scholar
  654. White GC, Zeitler KD, Lesesne HR et al. ( 1982 b) Chronic hepatitis in patients with hemophilia A: Histologic studies in patients with intermittently abnormal liver function tests. Blood 60: 1259–1262PubMedGoogle Scholar
  655. Whittaker DL, Copeland DL, Graham JB (1962) Linkage of colour blindness to hemophilias A nd B. Am J Hum Genet 14: 149–158PubMedGoogle Scholar
  656. Witwoet J (1978) Les pseudo-tumeurs hemophiliques (mise au point a propos de 7 cas). Rev Chir Orthop 64: 141–154Google Scholar
  657. WHO (1977) Methods for the detection of haemophilia carriers: a memorandum. Bull WHO 55: 675–702Google Scholar
  658. WHO (1978) Expert committee on biological standardization. 29th report. Technical Report Series 626, WHO, Geneva 1978Google Scholar
  659. Wood K, Omer A, Shaw MT (1969) Haemophilic arthropathy. Br J Radiol 42: 498–505PubMedGoogle Scholar
  660. Wood KR, Horowitz B (1980) International Forum: What is the importance of the “small pool concept” in the preparation of fraction I and cryoprecipitates for the prevention of post-transfu-sion hepatitis. Vox Sang 38: 113Google Scholar
  661. Wood WI, Capon DJ, Simonsen C et al. (1984) Expression of active human factor VIII from recombinant DNA clones. Nature (Lond) 312: 330Google Scholar
  662. Wright FW, Matthews JM, Brock LG et al. (1971) Complications of hemophilic disorders affecting the renal tract. Radiology 98: 571–576PubMedGoogle Scholar
  663. Wyke RJ, Tsiquaye KN, Thornton A et al. (1979) Transmission of non-A non-B hepatitis to chimpanzees by factor IX concentrates after fatal complications in patients with chronic liver disease. Lancet 1: 520–524PubMedGoogle Scholar
  664. Yang HC (1977) Purification and immunological studies of factor IX-Worcester. Blood 50: 289Google Scholar
  665. Yang HC (1978) Immunologic studies of factor IX (Christmas factor). II. Immunoradiometric assay of factor IX antigen. Br J Haematol 39: 215–244PubMedGoogle Scholar
  666. Zauber NP, Levin J (1977) Factor IX levels in patients with hemophilia B (Christmas disease) following transfusion with concentrates of factor IX or fresh frozen plasma ( FFP ). Medicine (Baltimore) 56: 213Google Scholar
  667. Zazgornig J, Pilgerstorfer HW (1974) Inkomplette renale tubuläre Azidose bei einem Hämophilen. Wien Klin Wochenschr 86: 590–593Google Scholar
  668. Ziemski JM, Lopaciuk S, Rudowski W (1971) 2 cases of surgical treatment of splenic rupture in patients with haemophilia type A. Acta Hepatogastroenterol 2: 87–90Google Scholar
  669. Zimmerman TS, Ratnoff OD, Littell AS (1971a) Detection of carriers of classic hemophilia using an immunologic assay for antihemophilic factor (factor VIII). J Clin Invest 50: 255PubMedGoogle Scholar
  670. Zimmerman TS, Ratnoff OD, Powell AE (1971b) Immunologic differentiation of classic hemophilia (factor VIII deficiency) and von Willebrands disease: with observations on combined deficiencies of antihemophilic factor and proaccelerin (factor V) and on an acquired circulating anticoagulant against antihemophilic factor. J Clin Invest 50: 244PubMedGoogle Scholar

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