메뉴 건너뛰기




Volumn 8, Issue 3, 2010, Pages 421-432

Mild hemophilia A

Author keywords

Bleeding; Desmopressin; FVIII replacement therapy; Inhibitor; Mild hemophilia A; Missense mutations

Indexed keywords

ACTIVATED PROTHROMBIN COMPLEX; ANTIFIBRINOLYTIC AGENT; BLOOD CLOTTING FACTOR 8; BLOOD CLOTTING FACTOR 8 CONCENTRATE; CYCLOPHOSPHAMIDE; DESMOPRESSIN; IMMUNOSUPPRESSIVE AGENT; PREDNISONE; RECOMBINANT BLOOD CLOTTING FACTOR 7A; RITUXIMAB; STEROID; TRANEXAMIC ACID;

EID: 77949285551     PISSN: 15387933     EISSN: 15387836     Source Type: Journal    
DOI: 10.1111/j.1538-7836.2009.03717.x     Document Type: Review
Times cited : (91)

References (158)
  • 1
    • 0035077234 scopus 로고    scopus 로고
    • Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis
    • White GC, Rosendaal F, Aledort LM, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001, 85.
    • (2001) Thromb Haemost , vol.85
    • White, G.C.1    Rosendaal, F.2    Aledort, L.M.3    Aledort, L.M.4    Lusher, J.M.5    Rothschild, C.6    Ingerslev, J.7
  • 2
    • 0028078717 scopus 로고
    • Hemophilia A
    • Hoyer LW. Hemophilia A. N Engl J Med 1994, 330:38-47.
    • (1994) N Engl J Med , vol.330 , pp. 38-47
    • Hoyer, L.W.1
  • 3
    • 0035822038 scopus 로고    scopus 로고
    • The hemophiliac - from royal genes to gene therapy
    • Mannucci PM, Tuddenham EGD. The hemophiliac - from royal genes to gene therapy. N Engl J Med 2001, 344:1773-9.
    • (2001) N Engl J Med , vol.344 , pp. 1773-1779
    • Mannucci, P.M.1    Tuddenham, E.G.D.2
  • 4
    • 77949295316 scopus 로고    scopus 로고
    • Mild Hemophilia. Treatment of Hemophilia Monograph
    • World Federation of Hemophilia, Available at:
    • Schulman S. Mild Hemophilia. Treatment of Hemophilia Monograph. 2006, 41:1-6. http://www.wfh.org/2/docs/Publications/Diagnosis_and_Treatment/TOH41_Mild-Hemophilia.pdf, World Federation of Hemophilia, Available at:
    • (2006) , vol.41 , pp. 1-6
    • Schulman, S.1
  • 5
    • 51249097825 scopus 로고    scopus 로고
    • Haemophilia care in China: achievements of a decade of World Federation of Hemophilia treatment centre twinning activities
    • Poon MC, Luke KH. Haemophilia care in China: achievements of a decade of World Federation of Hemophilia treatment centre twinning activities. Haemophilia 2008, 14:879-88.
    • (2008) Haemophilia , vol.14 , pp. 879-888
    • Poon, M.C.1    Luke, K.H.2
  • 6
  • 7
    • 43149102964 scopus 로고    scopus 로고
    • Italian Registry of Haemophilia and Allied Disorders. Objectives, methodology and data analysis
    • Iorio A, Oliovecchio E, Morfini M, Mannucci PM. Italian Registry of Haemophilia and Allied Disorders. Objectives, methodology and data analysis. Haemophilia 2008, 14:444-53.
    • (2008) Haemophilia , vol.14 , pp. 444-453
    • Iorio, A.1    Oliovecchio, E.2    Morfini, M.3    Mannucci, P.M.4
  • 9
    • 0019948521 scopus 로고
    • Current status of Swedish hemophiliacs: a demographic survey
    • Larsson SA, Nilsson IM, Blombäck M. Current status of Swedish hemophiliacs: a demographic survey. Acta Med Scand 1982, 212:195-200.
    • (1982) Acta Med Scand , vol.212 , pp. 195-200
    • Larsson, S.A.1    Nilsson, I.M.2    Blombäck, M.3
  • 12
    • 0036799519 scopus 로고    scopus 로고
    • What factors influence the age at diagnosis of hemophilia? Results of the French hemophilia cohort
    • Chambost H, Gaboulaud V, Coatmélec B, Rafowicz A, Schneider P, Calvez T. What factors influence the age at diagnosis of hemophilia? Results of the French hemophilia cohort. J Pediatr 2002, 141:548-52.
    • (2002) J Pediatr , vol.141 , pp. 548-552
    • Chambost, H.1    Gaboulaud, V.2    Coatmélec, B.3    Rafowicz, A.4    Schneider, P.5    Calvez, T.6
  • 14
    • 70149101680 scopus 로고    scopus 로고
    • Together we care: new challenges for global haemophilia treatment centers
    • Franchini M, Lippi G. Together we care: new challenges for global haemophilia treatment centers. Indian J Med Res 2009, 129:345-7.
    • (2009) Indian J Med Res , vol.129 , pp. 345-347
    • Franchini, M.1    Lippi, G.2
  • 15
    • 77949302669 scopus 로고    scopus 로고
    • Symptomatic carriers of hemophilia. Treatment of Hemophilia Monograph
    • World Federation of Hemophilia
    • Mauser-Bunschoten EP. Symptomatic carriers of hemophilia. Treatment of Hemophilia Monograph. 2008, 46:1-17. World Federation of Hemophilia
    • (2008) , vol.46 , pp. 1-17
    • Mauser-Bunschoten, E.P.1
  • 19
    • 0033862227 scopus 로고    scopus 로고
    • The Factor VIII acute phase response requires the participation of NFkappaB and C/EBP
    • Begbie M, Notley C, Tinlin S, Sawyer L, Lillicrap D. The Factor VIII acute phase response requires the participation of NFkappaB and C/EBP. Thromb Haemost 2000, 84:216-22.
    • (2000) Thromb Haemost , vol.84 , pp. 216-222
    • Begbie, M.1    Notley, C.2    Tinlin, S.3    Sawyer, L.4    Lillicrap, D.5
  • 20
    • 34147218347 scopus 로고    scopus 로고
    • Emerging technologies and quality assurance: the United Kingdom National External Quality Assessment Scheme perspective
    • Jennings I, Kitchen DP, Woods TA, Kitchen S, Walker ID. Emerging technologies and quality assurance: the United Kingdom National External Quality Assessment Scheme perspective. Semin Thromb Hemost 2007, 33:243-9.
    • (2007) Semin Thromb Hemost , vol.33 , pp. 243-249
    • Jennings, I.1    Kitchen, D.P.2    Woods, T.A.3    Kitchen, S.4    Walker, I.D.5
  • 21
    • 27844489519 scopus 로고    scopus 로고
    • Cross-laboratory audit of normal reference ranges and assessment of ABO-blood group, gender and age on detected levels of plasma coagulation factors
    • Favaloro EJ, Soltani S, McDonald J, Grezchnik E, Easton L. Cross-laboratory audit of normal reference ranges and assessment of ABO-blood group, gender and age on detected levels of plasma coagulation factors. Blood Coagul Fibrinolysis 2005, 16:597-605.
    • (2005) Blood Coagul Fibrinolysis , vol.16 , pp. 597-605
    • Favaloro, E.J.1    Soltani, S.2    McDonald, J.3    Grezchnik, E.4    Easton, L.5
  • 22
    • 69949109672 scopus 로고    scopus 로고
    • Age-dependent increase of FVIII:C in mild haemophilia A
    • Miesbach W, Alesci S, Krekeler S, Seifried E. Age-dependent increase of FVIII:C in mild haemophilia A. Haemophilia 2009, 16:1022-6.
    • (2009) Haemophilia , vol.16 , pp. 1022-1026
    • Miesbach, W.1    Alesci, S.2    Krekeler, S.3    Seifried, E.4
  • 23
    • 39549098650 scopus 로고    scopus 로고
    • Mild hemophilia A with factor VIII assay discrepancy: using thrombin generation assay to assess the bleeding phenotype
    • Trossaert M, Regnault V, Sigaud M, Boisseau P, Fressinaud E, Lecompte T. Mild hemophilia A with factor VIII assay discrepancy: using thrombin generation assay to assess the bleeding phenotype. J Thromb Haemost 2008, 6:486-93.
    • (2008) J Thromb Haemost , vol.6 , pp. 486-493
    • Trossaert, M.1    Regnault, V.2    Sigaud, M.3    Boisseau, P.4    Fressinaud, E.5    Lecompte, T.6
  • 25
    • 44249088320 scopus 로고    scopus 로고
    • Mild/moderate haemophilia A: new insights into molecular mechanisms and inhibitor development
    • D'Oiron R, Pipe SW, Jacquemin M. Mild/moderate haemophilia A: new insights into molecular mechanisms and inhibitor development. Haemophilia 2008, 14(Suppl. 3):138-46.
    • (2008) Haemophilia , vol.14 , Issue.SUPPL. 3 , pp. 138-146
    • D'Oiron, R.1    Pipe, S.W.2    Jacquemin, M.3
  • 26
    • 0037330233 scopus 로고    scopus 로고
    • The molecular basis of hemophilia A: genotype-phenotype relationships and inhibitor development
    • Goodeve AC, Peake IR. The molecular basis of hemophilia A: genotype-phenotype relationships and inhibitor development. Semin Thromb Hemost 2003, 29:23-30.
    • (2003) Semin Thromb Hemost , vol.29 , pp. 23-30
    • Goodeve, A.C.1    Peake, I.R.2
  • 29
    • 0037155149 scopus 로고    scopus 로고
    • Four hydrophobic amino acids of the factor VIII C2 domain are constituents of both the membrane-binding and von Willebrand factor-binding motifs
    • Gilbert G, Kaufman RJ, Arena AA, Miao H, Pipe SW. Four hydrophobic amino acids of the factor VIII C2 domain are constituents of both the membrane-binding and von Willebrand factor-binding motifs. J Biol Chem 2002, 277:6374-81.
    • (2002) J Biol Chem , vol.277 , pp. 6374-6381
    • Gilbert, G.1    Kaufman, R.J.2    Arena, A.A.3    Miao, H.4    Pipe, S.W.5
  • 30
    • 0005924558 scopus 로고
    • Direct characterization of factor VIII in plasma: detection of a mutation altering a thrombin cleavage site (arginine-372 - histidine)
    • Arai M, Inaba H, Higuchi M, Antonarakis SE, Kazazian HH, Fujimaki M, Hoyer LW. Direct characterization of factor VIII in plasma: detection of a mutation altering a thrombin cleavage site (arginine-372 - histidine). Proc Natl Acad Sci USA 1989, 86:4277-81.
    • (1989) Proc Natl Acad Sci USA , vol.86 , pp. 4277-4281
    • Arai, M.1    Inaba, H.2    Higuchi, M.3    Antonarakis, S.E.4    Kazazian, H.H.5    Fujimaki, M.6    Hoyer, L.W.7
  • 31
    • 0023746491 scopus 로고
    • Mutations of factor VIII cleavage sites in hemophilia A
    • Gitschier J, Kogan S, Levinson B, Tuddenham EG. Mutations of factor VIII cleavage sites in hemophilia A. Blood 1988, 72:1022-8.
    • (1988) Blood , vol.72 , pp. 1022-1028
    • Gitschier, J.1    Kogan, S.2    Levinson, B.3    Tuddenham, E.G.4
  • 32
    • 0035982216 scopus 로고    scopus 로고
    • A Tyr346--≥Cys substitution in the interdomain acidic region a1 of factor VIII in an individual with factor VIII:C assay discrepancy
    • Mumford AD, Laffan M, O'Donnell J, McVey JH, Johnson DJ, Manning RA, Kemball-Cook G. A Tyr346--≥Cys substitution in the interdomain acidic region a1 of factor VIII in an individual with factor VIII:C assay discrepancy. Br J Haematol 2002, 118:589-94.
    • (2002) Br J Haematol , vol.118 , pp. 589-594
    • Mumford, A.D.1    Laffan, M.2    O'Donnell, J.3    McVey, J.H.4    Johnson, D.J.5    Manning, R.A.6    Kemball-Cook, G.7
  • 33
    • 37749007969 scopus 로고    scopus 로고
    • Tyr346--≥Cys mutation results in factor VIII:C assay discrepancy and a normal bleeding phenotype - is this mild haemophilia A?
    • Lyall H, Hill M, Westby J, Grimley C, Dolan G. Tyr346--≥Cys mutation results in factor VIII:C assay discrepancy and a normal bleeding phenotype - is this mild haemophilia A? Haemophilia 2008, 14:78-80.
    • (2008) Haemophilia , vol.14 , pp. 78-80
    • Lyall, H.1    Hill, M.2    Westby, J.3    Grimley, C.4    Dolan, G.5
  • 34
    • 9644274261 scopus 로고    scopus 로고
    • Mutating factor VIII: lessons from structure to function
    • Fay PJ, Jenkins PV. Mutating factor VIII: lessons from structure to function. Blood Rev 2005, 19:15-27.
    • (2005) Blood Rev , vol.19 , pp. 15-27
    • Fay, P.J.1    Jenkins, P.V.2
  • 35
    • 0029670912 scopus 로고    scopus 로고
    • The sequence Glu1811-Lys1818 of human blood coagulation factor VIII comprises a binding site for activated factor IX
    • Lenting PJ, van de Loo JW, Donath MJ, van Mourik JA, Mertens K. The sequence Glu1811-Lys1818 of human blood coagulation factor VIII comprises a binding site for activated factor IX. J Biol Chem 1996, 271:1935-40.
    • (1996) J Biol Chem , vol.271 , pp. 1935-1940
    • Lenting, P.J.1    van de Loo, J.W.2    Donath, M.J.3    van Mourik, J.A.4    Mertens, K.5
  • 36
    • 0033065031 scopus 로고    scopus 로고
    • Diagnostic importance of the two-stage factor VIII:C assay demonstrated by a case of mild haemophilia associated with His1954→Leu substitution in the factor VIII A3 domain
    • Keeling DM, Sukhu K, Kemball-Cook G, Waseem N, Bagnall R, Lloyd JV. Diagnostic importance of the two-stage factor VIII:C assay demonstrated by a case of mild haemophilia associated with His1954→Leu substitution in the factor VIII A3 domain. Br J Haematol 1999, 105:1123-6.
    • (1999) Br J Haematol , vol.105 , pp. 1123-1126
    • Keeling, D.M.1    Sukhu, K.2    Kemball-Cook, G.3    Waseem, N.4    Bagnall, R.5    Lloyd, J.V.6
  • 37
    • 0034086972 scopus 로고    scopus 로고
    • Assay discrepancy in mild haemophilia A due to a factor VIII missense mutation (Asn694Ile) in a large Danish family
    • Schwaab R, Oldenburg J, Kemball-Cook G, Albert T, Juhler C, Hanfland P, Ingerslev J. Assay discrepancy in mild haemophilia A due to a factor VIII missense mutation (Asn694Ile) in a large Danish family. Br J Haematol 2000, 109:523-8.
    • (2000) Br J Haematol , vol.109 , pp. 523-528
    • Schwaab, R.1    Oldenburg, J.2    Kemball-Cook, G.3    Albert, T.4    Juhler, C.5    Hanfland, P.6    Ingerslev, J.7
  • 38
    • 33845486921 scopus 로고    scopus 로고
    • In vitro kinetics of factor VIII activity in patients with mild haemophilia A and a discrepancy between one-stage and two-stage factor VIII assay results
    • Rodgers SE, Duncan EM, Barbulescu DM, Quinn DM, Lloyd JV. In vitro kinetics of factor VIII activity in patients with mild haemophilia A and a discrepancy between one-stage and two-stage factor VIII assay results. Br J Haematol 2007, 136:138-45.
    • (2007) Br J Haematol , vol.136 , pp. 138-145
    • Rodgers, S.E.1    Duncan, E.M.2    Barbulescu, D.M.3    Quinn, D.M.4    Lloyd, J.V.5
  • 39
    • 0011380826 scopus 로고    scopus 로고
    • Discrepant one and two stage FVIII assays are very common in mild haemophilia A: correlation with FVIII gene mutations
    • Abstract OC195
    • Nesbitt IM, Hinks J, Sampson B, Kitchen S, Peake I, makris M, Goodeve A. Discrepant one and two stage FVIII assays are very common in mild haemophilia A: correlation with FVIII gene mutations. Thromb Haemost 2001, (Suppl.). Abstract OC195
    • (2001) Thromb Haemost , Issue.SUPPL.
    • Nesbitt, I.M.1    Hinks, J.2    Sampson, B.3    Kitchen, S.4    Peake, I.5    makris, M.6    Goodeve, A.7
  • 40
    • 0029886287 scopus 로고    scopus 로고
    • Mutations in a subgroup of patients with mild haemophilia A and a familial discrepancy between the one-stage and two-stage factor VIII:C methods
    • Rudzki Z, Duncan EM, Casey GJ, Neumann M, Favaloro EJ, Lloyd JV. Mutations in a subgroup of patients with mild haemophilia A and a familial discrepancy between the one-stage and two-stage factor VIII:C methods. Br J Haematol 1996, 94:400-6.
    • (1996) Br J Haematol , vol.94 , pp. 400-406
    • Rudzki, Z.1    Duncan, E.M.2    Casey, G.J.3    Neumann, M.4    Favaloro, E.J.5    Lloyd, J.V.6
  • 41
    • 0032932325 scopus 로고    scopus 로고
    • Mild hemophilia A caused by increased rate of factor VIII A2 subunit dissociation: evidence for nonproteolytic inactivation of factor VIIIa in vivo
    • Pipe SW, Eickhorst AN, McKinley SH, Saenko EL, Kaufman RJ. Mild hemophilia A caused by increased rate of factor VIII A2 subunit dissociation: evidence for nonproteolytic inactivation of factor VIIIa in vivo. Blood 1999, 93:176-83.
    • (1999) Blood , vol.93 , pp. 176-183
    • Pipe, S.W.1    Eickhorst, A.N.2    McKinley, S.H.3    Saenko, E.L.4    Kaufman, R.J.5
  • 42
    • 0035254221 scopus 로고    scopus 로고
    • Haemophilia A mutations associated with 1-stage/2-stage activity discrepancy disrupt protein- protein interactions within the triplicated A domains of thrombin-activated factor VIIIa
    • Pipe SW, Saenko EL, Eickhorst AN, Kemball-Cook G, Kaufman RJ. Haemophilia A mutations associated with 1-stage/2-stage activity discrepancy disrupt protein- protein interactions within the triplicated A domains of thrombin-activated factor VIIIa. Blood 2001, 97:685-91.
    • (2001) Blood , vol.97 , pp. 685-691
    • Pipe, S.W.1    Saenko, E.L.2    Eickhorst, A.N.3    Kemball-Cook, G.4    Kaufman, R.J.5
  • 43
    • 0036845615 scopus 로고    scopus 로고
    • Hemophilia A mutations within the factor VIII A2-A3 subunit destabilize factor VIIIa and cause one-stage/two-stage activity discrepancy
    • Hakeos WH, Miao H, Sirachainan N, Kemball-Cook G, Saenko EL, Kaufman RJ, Pipe SW. Hemophilia A mutations within the factor VIII A2-A3 subunit destabilize factor VIIIa and cause one-stage/two-stage activity discrepancy. Thromb Haemost 2002, 88:781-7.
    • (2002) Thromb Haemost , vol.88 , pp. 781-787
    • Hakeos, W.H.1    Miao, H.2    Sirachainan, N.3    Kemball-Cook, G.4    Saenko, E.L.5    Kaufman, R.J.6    Pipe, S.W.7
  • 44
    • 33846922115 scopus 로고    scopus 로고
    • Inhibitor development in one patient and laboratory discrepancies in several families with both mild haemophilia and Arg531Cys mutation
    • Cid AR, Casaña P, Cabrera N, Haya S, Cortina V, Aznar JA. Inhibitor development in one patient and laboratory discrepancies in several families with both mild haemophilia and Arg531Cys mutation. Haemophilia 2007, 13:206-8.
    • (2007) Haemophilia , vol.13 , pp. 206-208
    • Cid, A.R.1    Casaña, P.2    Cabrera, N.3    Haya, S.4    Cortina, V.5    Aznar, J.A.6
  • 45
    • 51249089221 scopus 로고    scopus 로고
    • One-stage and chromogenic FVIII:C assay discrepancy in mild haemophilia A and the relationship with the mutation and bleeding phenotype
    • Cid AR, Calabuig M, Cortina V, Casaña P, Haya S, Moret A, Cabrera N, Aznar JA. One-stage and chromogenic FVIII:C assay discrepancy in mild haemophilia A and the relationship with the mutation and bleeding phenotype. Haemophilia 2008, 14:1049-54.
    • (2008) Haemophilia , vol.14 , pp. 1049-1054
    • Cid, A.R.1    Calabuig, M.2    Cortina, V.3    Casaña, P.4    Haya, S.5    Moret, A.6    Cabrera, N.7    Aznar, J.A.8
  • 46
    • 0034254264 scopus 로고    scopus 로고
    • Hemophilic factor VIII C1- and C2-domain missense mutations and their modeling to the 1.5-angstrom human C2-domain crystal structure
    • Liu ML, Shen BW, Nakaya S, Pratt KP, Fujikawa K, Davie EW, Stoddard BL, Thompson AR. Hemophilic factor VIII C1- and C2-domain missense mutations and their modeling to the 1.5-angstrom human C2-domain crystal structure. Blood 2000, 96:979-87.
    • (2000) Blood , vol.96 , pp. 979-987
    • Liu, M.L.1    Shen, B.W.2    Nakaya, S.3    Pratt, K.P.4    Fujikawa, K.5    Davie, E.W.6    Stoddard, B.L.7    Thompson, A.R.8
  • 48
    • 0028058606 scopus 로고
    • Identification of individual tyrosine sulfation sites within factor VIII required for optimal activity and efficient thrombin cleavage
    • Michnick DA, Pittman DD, Wise RJ, Kaufman RJ. Identification of individual tyrosine sulfation sites within factor VIII required for optimal activity and efficient thrombin cleavage. J Biol Chem 1994, 269:20095-102.
    • (1994) J Biol Chem , vol.269 , pp. 20095-20102
    • Michnick, D.A.1    Pittman, D.D.2    Wise, R.J.3    Kaufman, R.J.4
  • 50
    • 57149090733 scopus 로고    scopus 로고
    • Molecular genetic testing of hemophilia A
    • Goodeve A. Molecular genetic testing of hemophilia A. Semin Thromb Hemost 2008, 34:491-501.
    • (2008) Semin Thromb Hemost , vol.34 , pp. 491-501
    • Goodeve, A.1
  • 52
    • 70449584919 scopus 로고    scopus 로고
    • Identification and prevalence of von Willebrand disease type 2N (Normandy) in Australia
    • Favaloro EJ, Mohammed S, Koutts J. Identification and prevalence of von Willebrand disease type 2N (Normandy) in Australia. Blood Coagul Fibrinolysis 2009, 20:706-14.
    • (2009) Blood Coagul Fibrinolysis , vol.20 , pp. 706-714
    • Favaloro, E.J.1    Mohammed, S.2    Koutts, J.3
  • 53
    • 33845468341 scopus 로고    scopus 로고
    • Recent acquisitions in acquired and congenital von Willebrand disorders
    • Franchini M, Lippi G. Recent acquisitions in acquired and congenital von Willebrand disorders. Clin Chim Acta 2007, 377:62-9.
    • (2007) Clin Chim Acta , vol.377 , pp. 62-69
    • Franchini, M.1    Lippi, G.2
  • 54
    • 0036673552 scopus 로고    scopus 로고
    • Identification of von Willebrand's disorder type 2N (Normandy) in Australia: a cross-laboratory investigation using different methodologies
    • Rodgers SE, Lerda NV, Favaloro EJ, Duncan EM, Casey GJ, Quinn DM, Hertzberg M, Lloyd JV. Identification of von Willebrand's disorder type 2N (Normandy) in Australia: a cross-laboratory investigation using different methodologies. Am J Clin Pathol 2002, 118:269-76.
    • (2002) Am J Clin Pathol , vol.118 , pp. 269-276
    • Rodgers, S.E.1    Lerda, N.V.2    Favaloro, E.J.3    Duncan, E.M.4    Casey, G.J.5    Quinn, D.M.6    Hertzberg, M.7    Lloyd, J.V.8
  • 55
    • 33746602713 scopus 로고    scopus 로고
    • Laboratory identification of von Willebrand Disease: technical and scientific perspectives
    • Favaloro EJ. Laboratory identification of von Willebrand Disease: technical and scientific perspectives. Semin Thromb Hemost 2006, 32:456-71.
    • (2006) Semin Thromb Hemost , vol.32 , pp. 456-471
    • Favaloro, E.J.1
  • 56
    • 57149107591 scopus 로고    scopus 로고
    • The role of molecular genetics in diagnosing von Willebrand disease
    • James P, Lillicrap D. The role of molecular genetics in diagnosing von Willebrand disease. Semin Thromb Hemost 2008, 34:502-8.
    • (2008) Semin Thromb Hemost , vol.34 , pp. 502-508
    • James, P.1    Lillicrap, D.2
  • 58
    • 0034661520 scopus 로고    scopus 로고
    • Mortality among males with hemophilia: relations with source of medical care. The Hemophilia Surveillance System Project Investigators
    • Soucie JM, Nuss R, Evatt B, Abdelhak A, Cowan L, Hill H, Kolakoski M, Wilber N. Mortality among males with hemophilia: relations with source of medical care. The Hemophilia Surveillance System Project Investigators. Blood 2000, 96:437-42.
    • (2000) Blood , vol.96 , pp. 437-442
    • Soucie, J.M.1    Nuss, R.2    Evatt, B.3    Abdelhak, A.4    Cowan, L.5    Hill, H.6    Kolakoski, M.7    Wilber, N.8
  • 59
    • 84857036939 scopus 로고
    • A clinical and medico-social study of haemophilia in Sweden
    • Ramgren O. A clinical and medico-social study of haemophilia in Sweden. Acta Med Scand 1962, 171:111-90.
    • (1962) Acta Med Scand , vol.171 , pp. 111-190
    • Ramgren, O.1
  • 61
    • 0021309378 scopus 로고
    • Hemophilia in Sweden. Studies on demography of hemophilia and surgery in hemophilia and von Willebrand's disease
    • Larsson SA. Hemophilia in Sweden. Studies on demography of hemophilia and surgery in hemophilia and von Willebrand's disease. Acta Med Scand Suppl 1984, 684:1-72.
    • (1984) Acta Med Scand Suppl , vol.684 , pp. 1-72
    • Larsson, S.A.1
  • 62
    • 67649840909 scopus 로고    scopus 로고
    • Survival in a cohort of patients with haemophilia at the haemophilia care center in Vienna, Austria, from 1983 to 2006
    • Reitter S, Waldhoer T, Vutuc C, Lechner K, Pabinger I. Survival in a cohort of patients with haemophilia at the haemophilia care center in Vienna, Austria, from 1983 to 2006. Haemophilia 2009, 15:888-93.
    • (2009) Haemophilia , vol.15 , pp. 888-893
    • Reitter, S.1    Waldhoer, T.2    Vutuc, C.3    Lechner, K.4    Pabinger, I.5
  • 64
    • 0036736430 scopus 로고    scopus 로고
    • Factors predictive of death among HIV-uninfected persons with haemophilia and other congenital coagulation disorders
    • Diamondstone LS, Aledort LM, Goedert JJ. Factors predictive of death among HIV-uninfected persons with haemophilia and other congenital coagulation disorders. Haemophilia 2002, 8:660-7.
    • (2002) Haemophilia , vol.8 , pp. 660-667
    • Diamondstone, L.S.1    Aledort, L.M.2    Goedert, J.J.3
  • 65
    • 0028838605 scopus 로고
    • Mortality in patients with hemophilia. Changes in a Dutch population from 1986 to 1992 and 1973 to 1986
    • Triemstra M, Rosendaal FR, Smit C, van der Ploeg HM, Briet E. Mortality in patients with hemophilia. Changes in a Dutch population from 1986 to 1992 and 1973 to 1986. Ann Intern Med 1995, 123:823-7.
    • (1995) Ann Intern Med , vol.123 , pp. 823-827
    • Triemstra, M.1    Rosendaal, F.R.2    Smit, C.3    van der Ploeg, H.M.4    Briet, E.5
  • 66
    • 0029157066 scopus 로고
    • Mortality before and after HIV infection in the complete UK population of haemophiliacs. UK Haemophilia Centre Directors' Organisation
    • Darby SC, Ewart DW, Giangrande PL, Dolin PJ, Spooner RJ, Rizza CR. Mortality before and after HIV infection in the complete UK population of haemophiliacs. UK Haemophilia Centre Directors' Organisation. Nature 1995, 377:79-82.
    • (1995) Nature , vol.377 , pp. 79-82
    • Darby, S.C.1    Ewart, D.W.2    Giangrande, P.L.3    Dolin, P.J.4    Spooner, R.J.5    Rizza, C.R.6
  • 68
    • 0031583763 scopus 로고    scopus 로고
    • Mortality from liver cancer and liver disease in haemophilic men and boys in UK given blood products contaminated with hepatitis C. UK Haemophilia Centre Directors' Organisation
    • Darby SC, Ewart DW, Giangrande PL, Spooner RJ, Rizza CR, Dusheiko GM, Lee CA, Ludlam CA, Preston FE. Mortality from liver cancer and liver disease in haemophilic men and boys in UK given blood products contaminated with hepatitis C. UK Haemophilia Centre Directors' Organisation. Lancet 1997, 350:1425-31.
    • (1997) Lancet , vol.350 , pp. 1425-1431
    • Darby, S.C.1    Ewart, D.W.2    Giangrande, P.L.3    Spooner, R.J.4    Rizza, C.R.5    Dusheiko, G.M.6    Lee, C.A.7    Ludlam, C.A.8    Preston, F.E.9
  • 72
    • 7344224341 scopus 로고    scopus 로고
    • Causes of death in Canadians with haemophilia 1980-1995. Association of Hemophilia Clinic Directors of Canada
    • Walker IR, Julian JA. Causes of death in Canadians with haemophilia 1980-1995. Association of Hemophilia Clinic Directors of Canada. Haemophilia 1998, 4:714-20.
    • (1998) Haemophilia , vol.4 , pp. 714-720
    • Walker, I.R.1    Julian, J.A.2
  • 76
    • 67649863784 scopus 로고    scopus 로고
    • Co-morbidity in the ageing haemophilia patient: the down side of increased life expectancy
    • Mauser-Bunschoten EP, Fransen van de Putte DE, Schutgens RE. Co-morbidity in the ageing haemophilia patient: the down side of increased life expectancy. Haemophilia 2009, 15:853-6.
    • (2009) Haemophilia , vol.15 , pp. 853-856
    • Mauser-Bunschoten, E.P.1    Fransen van de Putte, D.E.2    Schutgens, R.E.3
  • 78
    • 34547962876 scopus 로고    scopus 로고
    • Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV
    • Darby SC, Kan SW, Spooner RJ, Giangrande PL, Hill FG, Hay CR, Lee CA, Ludlam CA, Williams M. Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV. Blood 2007, 110:815-25.
    • (2007) Blood , vol.110 , pp. 815-825
    • Darby, S.C.1    Kan, S.W.2    Spooner, R.J.3    Giangrande, P.L.4    Hill, F.G.5    Hay, C.R.6    Lee, C.A.7    Ludlam, C.A.8    Williams, M.9
  • 79
    • 0042671199 scopus 로고    scopus 로고
    • Decreased mortality of ischemic heart disease among carriers of hemophilia
    • Šrámek A, Kriek M, Rosendaal FR. Decreased mortality of ischemic heart disease among carriers of hemophilia. Lancet 2003, 362:351-4.
    • (2003) Lancet , vol.362 , pp. 351-354
    • Šrámek, A.1    Kriek, M.2    Rosendaal, F.R.3
  • 80
    • 18044371836 scopus 로고    scopus 로고
    • Prevalence and risk factors for heart disease among males with hemophilia
    • Kulkarni R, Soucie JM, Evatt BL. Prevalence and risk factors for heart disease among males with hemophilia. Am J Hematol 2005, 79:36-42.
    • (2005) Am J Hematol , vol.79 , pp. 36-42
    • Kulkarni, R.1    Soucie, J.M.2    Evatt, B.L.3
  • 83
    • 0034774332 scopus 로고    scopus 로고
    • Hemophilia A, von Willebrand disease, and atherosclerosis of abdominal aorta and leg arteries: factor VIII and von Willebrand factor defects appear to protect abdominal aorta and leg arteries from atherosclerosis
    • Bilora F, Boccioletti V, Zanon E, Petrobelli F, Girolami A. Hemophilia A, von Willebrand disease, and atherosclerosis of abdominal aorta and leg arteries: factor VIII and von Willebrand factor defects appear to protect abdominal aorta and leg arteries from atherosclerosis. Clin Appl Thromb Hemost 2001, 7:311-3.
    • (2001) Clin Appl Thromb Hemost , vol.7 , pp. 311-313
    • Bilora, F.1    Boccioletti, V.2    Zanon, E.3    Petrobelli, F.4    Girolami, A.5
  • 85
    • 0035859790 scopus 로고    scopus 로고
    • Decreased coagulability has no clinically relevant effect on atherogenesis: observations in individuals with a hereditary bleeding tendency
    • Sramek A, Reiber JH, Gerrits WB, Rosendaal FR. Decreased coagulability has no clinically relevant effect on atherogenesis: observations in individuals with a hereditary bleeding tendency. Circulation 2001, 104:762-7.
    • (2001) Circulation , vol.104 , pp. 762-767
    • Sramek, A.1    Reiber, J.H.2    Gerrits, W.B.3    Rosendaal, F.R.4
  • 88
    • 0034020665 scopus 로고    scopus 로고
    • Cardiac surgery and catheterization in patients with haemophilia
    • Mackinlay N, Taper J, Renisson F, Rickard K. Cardiac surgery and catheterization in patients with haemophilia. Haemophilia 2000, 6:84-8.
    • (2000) Haemophilia , vol.6 , pp. 84-88
    • Mackinlay, N.1    Taper, J.2    Renisson, F.3    Rickard, K.4
  • 90
    • 67649887761 scopus 로고    scopus 로고
    • Are hemophiliacs protected against cancer development? Prospective controlled studies are needed
    • Franchini M, Lippi G. Are hemophiliacs protected against cancer development? Prospective controlled studies are needed. J Cell Mol Med 2009, 13:1185.
    • (2009) J Cell Mol Med , vol.13 , pp. 1185
    • Franchini, M.1    Lippi, G.2
  • 92
    • 0038383605 scopus 로고    scopus 로고
    • The epidemiology of inhibitors in haemophilia A: a systematic review
    • Wight J, Paisley S. The epidemiology of inhibitors in haemophilia A: a systematic review. Haemophilia 2003, 9:418-35.
    • (2003) Haemophilia , vol.9 , pp. 418-435
    • Wight, J.1    Paisley, S.2
  • 93
    • 77449116760 scopus 로고    scopus 로고
    • The multifactorial etiology of inhibitor development in hemophilia: genetics and environment
    • Gouw SC, van den Berg HM. The multifactorial etiology of inhibitor development in hemophilia: genetics and environment. Semin Thromb Hemost 2009, 35:723-34.
    • (2009) Semin Thromb Hemost , vol.35 , pp. 723-734
    • Gouw, S.C.1    van den Berg, H.M.2
  • 94
    • 0031856491 scopus 로고    scopus 로고
    • Factor VIII inhibitors in mild and moderate-severity haemophilia A
    • Hay CRM. Factor VIII inhibitors in mild and moderate-severity haemophilia A. Haemophilia 1998, 4:558-63.
    • (1998) Haemophilia , vol.4 , pp. 558-563
    • Hay, C.R.M.1
  • 95
    • 33747091225 scopus 로고    scopus 로고
    • Inhibitors in mild/moderate haemophilia: an update
    • Franchini M, Salvagno GL, Lippi G. Inhibitors in mild/moderate haemophilia: an update. Thromb Haemost 2006, 96:113-8.
    • (2006) Thromb Haemost , vol.96 , pp. 113-118
    • Franchini, M.1    Salvagno, G.L.2    Lippi, G.3
  • 96
    • 33751016399 scopus 로고    scopus 로고
    • Characteristics of inhibitors in mild/moderate haemophilia A
    • Peerlinck K, Jacquemin M. Characteristics of inhibitors in mild/moderate haemophilia A. Haemophilia 2006, 12(Suppl. 6):43-7.
    • (2006) Haemophilia , vol.12 , Issue.SUPPL. 6 , pp. 43-47
    • Peerlinck, K.1    Jacquemin, M.2
  • 97
    • 0026548917 scopus 로고
    • Incidence of development of factor VIII and factor IX inhibitors in haemophiliacs
    • Ehrenforth S, Kreuz W, Scharrer I, Kornhuber B. Incidence of development of factor VIII and factor IX inhibitors in haemophiliacs. Lancet 1992, 339:594-8.
    • (1992) Lancet , vol.339 , pp. 594-598
    • Ehrenforth, S.1    Kreuz, W.2    Scharrer, I.3    Kornhuber, B.4
  • 99
    • 0026622961 scopus 로고
    • Prevalence of inhibitors in a population of 3435 hemophilia patients in France. French Hemophilia Study Group
    • Sultan Y. Prevalence of inhibitors in a population of 3435 hemophilia patients in France. French Hemophilia Study Group. Thromb Haemost 1992, 67:600-2.
    • (1992) Thromb Haemost , vol.67 , pp. 600-602
    • Sultan, Y.1
  • 100
    • 0023856830 scopus 로고
    • The natural history of factor VIII:C inhibitors in patients with hemophilia A: a national cooperative study. II. Observations on the initial development of factor VIII:C inhibitors
    • McMillan CW, Shapiro SS, Whitehurst D, Hoyer LW, Rao AV, Lazerson J. The natural history of factor VIII:C inhibitors in patients with hemophilia A: a national cooperative study. II. Observations on the initial development of factor VIII:C inhibitors. Blood 1988, 71:344-8.
    • (1988) Blood , vol.71 , pp. 344-348
    • McMillan, C.W.1    Shapiro, S.S.2    Whitehurst, D.3    Hoyer, L.W.4    Rao, A.V.5    Lazerson, J.6
  • 102
    • 0015741266 scopus 로고
    • Factor VIII inhibitor in a patient with mild hemophilia A
    • _;73
    • Lechner K, Ludwig E, Niessner H, Thaler E. Factor VIII inhibitor in a patient with mild hemophilia A. Haemostasis 1972-73, 1:261-70.
    • (1972) Haemostasis , vol.1 , pp. 261-270
    • Lechner, K.1    Ludwig, E.2    Niessner, H.3    Thaler, E.4
  • 103
    • 0021995950 scopus 로고
    • Factor VIII antibody in a patient with mild haemophilia
    • Bovill EG, Burns SL, Golden EA. Factor VIII antibody in a patient with mild haemophilia. Br J Haematol 1985, 61:323-8.
    • (1985) Br J Haematol , vol.61 , pp. 323-328
    • Bovill, E.G.1    Burns, S.L.2    Golden, E.A.3
  • 104
    • 0034019353 scopus 로고    scopus 로고
    • High responding factor VIII inhibitors in mild haemophilia - is there a link with recent changes in clinical practice?
    • White B, Cotter M, Byrne M, O'Shea E, Smith OP. High responding factor VIII inhibitors in mild haemophilia - is there a link with recent changes in clinical practice? Haemophilia 2000, 6:113-5.
    • (2000) Haemophilia , vol.6 , pp. 113-115
    • White, B.1    Cotter, M.2    Byrne, M.3    O'Shea, E.4    Smith, O.P.5
  • 105
    • 25444441199 scopus 로고    scopus 로고
    • Inhibitors in mild and moderate haemophilia A
    • Lee CA, Berntorp E, Hoots WK. In, ed, Oxford, Blackwell Publishing
    • Hay CRM, Lee CA. Inhibitors in mild and moderate haemophilia A. Inhibitors in patients with haemophilia 2002, 92-7. Lee CA, Berntorp EHoots WK. In, ed, Oxford, Blackwell Publishing
    • (2002) Inhibitors in patients with haemophilia , pp. 92-97
    • Hay, C.R.M.1    Lee, C.A.2
  • 106
    • 6644227418 scopus 로고    scopus 로고
    • The Malmo International Brother Study (MIBS): further support for genetic predisposition to inhibitor development in hemophilia patients
    • Astermark J, Berntorp E, White GC, Kroner BL. The Malmo International Brother Study (MIBS): further support for genetic predisposition to inhibitor development in hemophilia patients. Haemophilia. 2001, 7:267-72.
    • (2001) Haemophilia. , vol.7 , pp. 267-272
    • Astermark, J.1    Berntorp, E.2    White, G.C.3    Kroner, B.L.4
  • 109
    • 0027340359 scopus 로고
    • Analysis of factor VIII mRNA reveals defects in everyone of 28 haemophilia A patients
    • Naylor JA, Green PM, Rizza CR, Giannelli F. Analysis of factor VIII mRNA reveals defects in everyone of 28 haemophilia A patients. Hum Mol Genet 1993, 2:11-7.
    • (1993) Hum Mol Genet , vol.2 , pp. 11-17
    • Naylor, J.A.1    Green, P.M.2    Rizza, C.R.3    Giannelli, F.4
  • 110
    • 0034467035 scopus 로고    scopus 로고
    • Factor VIII inhibitors in two families with mild haemophilia A: structural analysis of the mutations
    • Knobe KE, Villoutreix BO, Tengborn LI, Petrini P, Ljung RC. Factor VIII inhibitors in two families with mild haemophilia A: structural analysis of the mutations. Haemostasis 2000, 30:268-79.
    • (2000) Haemostasis , vol.30 , pp. 268-279
    • Knobe, K.E.1    Villoutreix, B.O.2    Tengborn, L.I.3    Petrini, P.4    Ljung, R.C.5
  • 112
    • 0036186809 scopus 로고    scopus 로고
    • The identification and classification of 41 novel mutations in the factor VIII gene (F8C)
    • Cutler JA, Mitchell MJ, Smith MP, Savidge GF. The identification and classification of 41 novel mutations in the factor VIII gene (F8C). Hum Mutat 2002, 19:274-8.
    • (2002) Hum Mutat , vol.19 , pp. 274-278
    • Cutler, J.A.1    Mitchell, M.J.2    Smith, M.P.3    Savidge, G.F.4
  • 115
    • 0030884459 scopus 로고    scopus 로고
    • Loss of tolerance to exogenous and endogenous factor VIII in a mild hemophilia A patient with an Arg593 to Cys mutation
    • Thompson AR, Murphy ME, Liu M, Saenko EL, Healey JF, Lollar P, Scandella D. Loss of tolerance to exogenous and endogenous factor VIII in a mild hemophilia A patient with an Arg593 to Cys mutation. Blood 1997, 90:1902-10.
    • (1997) Blood , vol.90 , pp. 1902-1910
    • Thompson, A.R.1    Murphy, M.E.2    Liu, M.3    Saenko, E.L.4    Healey, J.F.5    Lollar, P.6    Scandella, D.7
  • 119
    • 0031792547 scopus 로고    scopus 로고
    • Is a change of factor VIII product a risk factor for the development of a factor VIII inhibitor?
    • Baglin T, Beacham E. Is a change of factor VIII product a risk factor for the development of a factor VIII inhibitor? Thromb Haemost 1998, 80:1036-7.
    • (1998) Thromb Haemost , vol.80 , pp. 1036-1037
    • Baglin, T.1    Beacham, E.2
  • 120
    • 0032915482 scopus 로고    scopus 로고
    • Is a change of factor VIII product a risk factor for the development of a factor VIII inhibitor?
    • Yee TT, Lee CA. Is a change of factor VIII product a risk factor for the development of a factor VIII inhibitor? Thromb Haemost 1999, 81:852.
    • (1999) Thromb Haemost , vol.81 , pp. 852
    • Yee, T.T.1    Lee, C.A.2
  • 123
    • 0036588780 scopus 로고    scopus 로고
    • Continuous infusion of coagulation factors
    • Batorova A, Martinowitz U. Continuous infusion of coagulation factors. Haemophilia 2002, 8:170-7.
    • (2002) Haemophilia , vol.8 , pp. 170-177
    • Batorova, A.1    Martinowitz, U.2
  • 125
    • 76149093682 scopus 로고    scopus 로고
    • Laboratory identification of factor inhibitors: the perspective of a large tertiary hemophilia centre
    • Kershaw G, Jayakodi D, Dunkley S. Laboratory identification of factor inhibitors: the perspective of a large tertiary hemophilia centre. Semin Thromb Hemost 2009, 35:760-8.
    • (2009) Semin Thromb Hemost , vol.35 , pp. 760-768
    • Kershaw, G.1    Jayakodi, D.2    Dunkley, S.3
  • 128
    • 77449099048 scopus 로고    scopus 로고
    • Investigations from external quality assurance programs reveal a high degree of variation in the laboratory identification of coagulation factor inhibitors
    • Favaloro EJ, Bonar R, Kershaw G, Duncan E, Sioufi J, Marsden K. Investigations from external quality assurance programs reveal a high degree of variation in the laboratory identification of coagulation factor inhibitors. Semin Thromb Hemost 2009, 35:794-805.
    • (2009) Semin Thromb Hemost , vol.35 , pp. 794-805
    • Favaloro, E.J.1    Bonar, R.2    Kershaw, G.3    Duncan, E.4    Sioufi, J.5    Marsden, K.6
  • 129
    • 34547673114 scopus 로고    scopus 로고
    • The use of desmopressin as a hemostatic agent
    • Franchini M. The use of desmopressin as a hemostatic agent. Am J Hematol 2007, 82:731-5.
    • (2007) Am J Hematol , vol.82 , pp. 731-735
    • Franchini, M.1
  • 130
    • 0033926486 scopus 로고    scopus 로고
    • Desmopressin (DDAVP) in the treatment of bleeding disorders: the first twenty years
    • Mannucci PM. Desmopressin (DDAVP) in the treatment of bleeding disorders: the first twenty years. Haemophilia 2000, 6(Suppl. 1):60-7.
    • (2000) Haemophilia , vol.6 , Issue.SUPPL. 1 , pp. 60-67
    • Mannucci, P.M.1
  • 132
    • 0021794047 scopus 로고
    • Response of patients with mild and moderate hemophilia A and von Willebrand disease to treatment with desmopressin
    • de la Fuente B, Kasper CK, Rickles FR, Hoyer LW. Response of patients with mild and moderate hemophilia A and von Willebrand disease to treatment with desmopressin. Ann Intern Med 1985, 103:6-14.
    • (1985) Ann Intern Med , vol.103 , pp. 6-14
    • de la Fuente, B.1    Kasper, C.K.2    Rickles, F.R.3    Hoyer, L.W.4
  • 133
    • 0036588696 scopus 로고    scopus 로고
    • The use of haemostatic drugs in haemophilia: Desmopressin and antifibrinolytic agents
    • Villar A, Jimenez-Yuste V, Quintana M, Hernandez-Navarro F. The use of haemostatic drugs in haemophilia: Desmopressin and antifibrinolytic agents. Haemophilia 2002, 8:189-93.
    • (2002) Haemophilia , vol.8 , pp. 189-193
    • Villar, A.1    Jimenez-Yuste, V.2    Quintana, M.3    Hernandez-Navarro, F.4
  • 134
    • 0037325364 scopus 로고    scopus 로고
    • Desmopressin in mild hemophilia A: Indications, limitations, efficacy, and safety
    • Lethagen S. Desmopressin in mild hemophilia A: Indications, limitations, efficacy, and safety. Semin Thromb Hemost 2003, 29:101-6.
    • (2003) Semin Thromb Hemost , vol.29 , pp. 101-106
    • Lethagen, S.1
  • 135
    • 0023614209 scopus 로고
    • Intravenous and subcutaneous administration of desmopressin (DDAVP) to hemophiliacs: Pharmacokinetics and factor VIII responses
    • Mannucci PM, Vicente V, Alberca I, Sacchi E, Longo G, Harris AS, Lindquist A. Intravenous and subcutaneous administration of desmopressin (DDAVP) to hemophiliacs: Pharmacokinetics and factor VIII responses. Thromb Haemost 1987, 58:1037-9.
    • (1987) Thromb Haemost , vol.58 , pp. 1037-1039
    • Mannucci, P.M.1    Vicente, V.2    Alberca, I.3    Sacchi, E.4    Longo, G.5    Harris, A.S.6    Lindquist, A.7
  • 137
  • 138
    • 37449021072 scopus 로고    scopus 로고
    • Desmopressin for the treatment of haemophilia
    • Castaman G. Desmopressin for the treatment of haemophilia. Haemophilia 2008, 14(Suppl. 1):15-20.
    • (2008) Haemophilia , vol.14 , Issue.SUPPL. 1 , pp. 15-20
    • Castaman, G.1
  • 140
    • 0031819851 scopus 로고    scopus 로고
    • DDAVP use during pregnancy: an analysis of its safety for mother and child
    • Ray JG. DDAVP use during pregnancy: an analysis of its safety for mother and child. Obstet Gynecol Surv 1998, 53:450-5.
    • (1998) Obstet Gynecol Surv , vol.53 , pp. 450-455
    • Ray, J.G.1
  • 141
    • 17044373272 scopus 로고    scopus 로고
    • Use of desmopressin (DDAVP) during early pregnancy in FVIII-deficient women
    • Mannucci PM. Use of desmopressin (DDAVP) during early pregnancy in FVIII-deficient women. Blood 2005, 105:3382.
    • (2005) Blood , vol.105 , pp. 3382
    • Mannucci, P.M.1
  • 143
    • 67649496311 scopus 로고    scopus 로고
    • Characterization of a novel mutation in the F8 promoter region associated with mild hemophilia A and resistance to DDAVP therapy
    • Riccardi F, Rivolta GF, Franchini M, Pattacini C, Neri TM, Tagliaferri A. Characterization of a novel mutation in the F8 promoter region associated with mild hemophilia A and resistance to DDAVP therapy. J Thromb Haemost 2009, 7:1234-5.
    • (2009) J Thromb Haemost , vol.7 , pp. 1234-1235
    • Riccardi, F.1    Rivolta, G.F.2    Franchini, M.3    Pattacini, C.4    Neri, T.M.5    Tagliaferri, A.6
  • 145
    • 65849151147 scopus 로고    scopus 로고
    • Mild and moderate factor VIII deficiency: inhibitor risks
    • Aledort LM. Mild and moderate factor VIII deficiency: inhibitor risks. J Thromb Haemost 2009, 7:928-9.
    • (2009) J Thromb Haemost , vol.7 , pp. 928-929
    • Aledort, L.M.1
  • 146
    • 33644978157 scopus 로고    scopus 로고
    • Can we prevent inhibitors in mild and moderate factor VIII-deficient patients?
    • Aledort LM. Can we prevent inhibitors in mild and moderate factor VIII-deficient patients? J Thromb Haemost 2006, 4:914.
    • (2006) J Thromb Haemost , vol.4 , pp. 914
    • Aledort, L.M.1
  • 147
    • 0032560732 scopus 로고    scopus 로고
    • Hemostatic drugs
    • Mannucci PM. Hemostatic drugs. N Engl J Med 1998, 339:245-53.
    • (1998) N Engl J Med , vol.339 , pp. 245-253
    • Mannucci, P.M.1
  • 148
    • 0034000569 scopus 로고    scopus 로고
    • Guidelines on replacement therapy for haemophilia and inherited coagulation disorders in Italy
    • Santagostino E, Mannucci PM. Guidelines on replacement therapy for haemophilia and inherited coagulation disorders in Italy. Haemophilia 2000, 6:1-10.
    • (2000) Haemophilia , vol.6 , pp. 1-10
    • Santagostino, E.1    Mannucci, P.M.2
  • 149
    • 46149129139 scopus 로고
    • Effect of local antifibrinolytic treatment with tranexamic acid in hemophiliacs undergoing oral surgery
    • Sindet-Pedersen S, Stenbjerg S. Effect of local antifibrinolytic treatment with tranexamic acid in hemophiliacs undergoing oral surgery. J Oral Maxillofac Surg 1986, 44:703-7.
    • (1986) J Oral Maxillofac Surg , vol.44 , pp. 703-707
    • Sindet-Pedersen, S.1    Stenbjerg, S.2
  • 150
    • 47649096167 scopus 로고    scopus 로고
    • Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders. A United Kingdom Haemophilia Center Doctors' Organisation (UKHCDO) guideline approved by the British Committee for Standards in Haematology
    • Keeling D, Tait C, Makris M. Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders. A United Kingdom Haemophilia Center Doctors' Organisation (UKHCDO) guideline approved by the British Committee for Standards in Haematology. Haemophilia 2008, 14:671-84.
    • (2008) Haemophilia , vol.14 , pp. 671-684
    • Keeling, D.1    Tait, C.2    Makris, M.3
  • 151
    • 58849134142 scopus 로고    scopus 로고
    • One-stage clotting versus chromogenic assays for assessing recombinant factor VIII: two faces of a haemostasis coin
    • Lippi G, Franchini M, Favaloro EJ. One-stage clotting versus chromogenic assays for assessing recombinant factor VIII: two faces of a haemostasis coin. Blood Coagul Fibrinolysis 2009, 20:1-3.
    • (2009) Blood Coagul Fibrinolysis , vol.20 , pp. 1-3
    • Lippi, G.1    Franchini, M.2    Favaloro, E.J.3
  • 152
    • 0034770548 scopus 로고    scopus 로고
    • Successful treatment of high titer inhibitors in mild hemophilia A with avoidance of factor VIII and immunosuppressive therapy
    • Robbins D, Kulkarni R, Gera R, Scott-Emuakpor AB, Bosma K, Penner JA. Successful treatment of high titer inhibitors in mild hemophilia A with avoidance of factor VIII and immunosuppressive therapy. Am J Hematol 2001, 68:184-8.
    • (2001) Am J Hematol , vol.68 , pp. 184-188
    • Robbins, D.1    Kulkarni, R.2    Gera, R.3    Scott-Emuakpor, A.B.4    Bosma, K.5    Penner, J.A.6
  • 153
    • 2342520667 scopus 로고    scopus 로고
    • Effective and safe use of recombinant factor VIIa (NovoSeven) in elderly mild haemophilia A patients with high-titre antibodies against factor VIII
    • Leebeek FW, Kappers-Klunne MC, Jie KS. Effective and safe use of recombinant factor VIIa (NovoSeven) in elderly mild haemophilia A patients with high-titre antibodies against factor VIII. Haemophilia 2004, 10:250-3.
    • (2004) Haemophilia , vol.10 , pp. 250-253
    • Leebeek, F.W.1    Kappers-Klunne, M.C.2    Jie, K.S.3
  • 154
    • 0036227304 scopus 로고    scopus 로고
    • Factor VIII inhibitor in a patient with mild haemophilia A and an Asn618-->Ser mutation responsive to immune tolerance induction and cyclophosphamide
    • Vlot AJ, Wittebol S, Strengers PF, Turenhout EA, Voorberg J, van den Berg HM, Mauser-Bunschoten EP. Factor VIII inhibitor in a patient with mild haemophilia A and an Asn618-->Ser mutation responsive to immune tolerance induction and cyclophosphamide. Br J Haematol 2002, 117:136-40.
    • (2002) Br J Haematol , vol.117 , pp. 136-140
    • Vlot, A.J.1    Wittebol, S.2    Strengers, P.F.3    Turenhout, E.A.4    Voorberg, J.5    van den Berg, H.M.6    Mauser-Bunschoten, E.P.7
  • 156
    • 33747109787 scopus 로고    scopus 로고
    • The efficacy of rituximab in the treatment of inhibitor-associated hemostatic disorders
    • Franchini M, Veneri D, Lippi G, Stenner R. The efficacy of rituximab in the treatment of inhibitor-associated hemostatic disorders. Thromb Haemost 2006, 96:119-25.
    • (2006) Thromb Haemost , vol.96 , pp. 119-125
    • Franchini, M.1    Veneri, D.2    Lippi, G.3    Stenner, R.4
  • 157
    • 51249104017 scopus 로고    scopus 로고
    • Immune tolerance with rituximab in congenital haemophilia with inhibitors: a systematic literature review based on individual patients' analysis
    • Franchini M, Mengoli C, Lippi G, Targher G, Montagnana M, Salvagno GL, Zaffanello M, Cruciani M. Immune tolerance with rituximab in congenital haemophilia with inhibitors: a systematic literature review based on individual patients' analysis. Haemophilia 2008, 14:903-12.
    • (2008) Haemophilia , vol.14 , pp. 903-912
    • Franchini, M.1    Mengoli, C.2    Lippi, G.3    Targher, G.4    Montagnana, M.5    Salvagno, G.L.6    Zaffanello, M.7    Cruciani, M.8
  • 158
    • 73049110802 scopus 로고    scopus 로고
    • Genetic testing for von Willebrand disease: the case against
    • Favaloro EJ. Genetic testing for von Willebrand disease: the case against. J Thromb Haemost 2010, 8:6-12.
    • (2010) J Thromb Haemost , vol.8 , pp. 6-12
    • Favaloro, E.J.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.