Pasteurized C1 inhibitor concentrate in hereditary angioedema: pharmacology, safety, efficacy and future directions

References

• Quincke HI. Ueber akutes umschriebenes Hautoedem. >Monatsh. Prakt. Dermatol.1, 129–131 (1882).
   Google Scholar
• Osler W. Hereditary angioneurotic edema. Am. J. Med. Sci.95, 362–367 (1888).
   Google Scholar
• Donaldson VH, Evans RR. A biochemical abnormality in hereditary angioneurotic edema: absence of serum inhibitor of C´1-esterase. Am. J. Med.35, 37–44 (1963).
   PubMed Web of Science ®Google Scholar
• Rosen FS, Pensky J, Donaldson V, Charache P. Hereditary angioneurotic edema: two genetic variants. Science148, 957–958 (1965).
   PubMed Web of Science ®Google Scholar
• Bowen B, Hawk JJ, Sibunka S, Hovick S, Weiler JM. A review of the reported defects in the human C1 esterase inhibitor gene producing hereditary angioedema including four new mutations. Clin. Immunol.98(2), 157–163 (2001).
   PubMed Web of Science ®Google Scholar
• Kalmar L, Bors A, Farkas H et al. Mutation screening of the C1 inhibitor gene among Hungarian patients with hereditary angioedema. Hum. Mutat.22(6), 498 (2003).
   PubMedGoogle Scholar
• Drouet C, Blanch A, Roche O et al. HAE: Mutation analysis of the C1INH gene. J. Allergy Clin. Immunol.114, S66–S74 (2004).
   PubMedGoogle Scholar
• Agostoni A, Cicardi M. Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine (Baltimore)71(4), 206–215 (1992).
   PubMed Web of Science ®Google Scholar
• Bork K, Barnstedt SE, Koch P, Traupe H. Hereditary angioedema with normal C1-inhibitor activity in women. Lancet356(9225), 213–217 (2000).
   PubMed Web of Science ®Google Scholar
• Dewald G, Bork K. Missense mutations in the coagulation factor XII (Hageman factor) gene in hereditary angioedema with normal C1 inhibitor. Biochem. Biophys. Res. Commun.343(4), 1286–1289 (2006).
   PubMed Web of Science ®Google Scholar
• Bork K. Hereditary angioedema with normal C1 inhibitor activity including hereditary angioedema with coagulation factor XII gene mutations. Immunol. Allergy Clin. North Am.26(4), 709–724 (2006).
   Google Scholar
• Longhurst HJ, Bork K. Hereditary angioedema: causes, manifestations and treatment. Br. J. Hosp. Med (London)67(12), 654–657 (2006).
   PubMedGoogle Scholar
• Bork K, Staubach P, Eckardt AJ, Hardt J. Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency. Am. J. Gastroenterol.101(3), 619–627 (2006).
   PubMed Web of Science ®Google Scholar
• Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am. J. Med.119(3), 267–274 (2006).
   PubMed Web of Science ®Google Scholar
• Bork K, Barnstedt SE. Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema. J. Am. Dent. Assoc.134(8), 1088–1094 (2003).
   PubMed Web of Science ®Google Scholar
• Cicardi M, Zingale L. How do we treat patients with hereditary angioedema. Transfus. Apher. Sci.29(3), 221–227 (2003).
   PubMed Web of Science ®Google Scholar
• Bork K. Current treatment of HAE and future treatment options. J. Allergy Clin. Immunol.114, S94–S96 (2004).
   Google Scholar
• Bowen T, Cicardi M, Farkas H et al. Canadian 2003 international consensus algorithm for the diagnosis, therapy, and management of hereditary angioedema. J. Allergy Clin. Immunol.114(3), 629–637 (2004).
   PubMed Web of Science ®Google Scholar
• Brackertz D, Kueppers F. Hereditary angioneurotic oedema. Lancet2(7830), 680 (1973).
   PubMed Web of Science ®Google Scholar
• Gadek JE, Hosea SW, Gelfand JA et al. Replacement therapy in hereditary angioedema: successful treatment of acute episodes of angioedema with partly purified C1 inhibitor. N. Engl. J. Med.302(10), 542–546 (1980).
   PubMed Web of Science ®Google Scholar
• Waytes AT, Rosen FS, Frank MM. Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate. N. Engl. J. Med.334(25), 1630–1634 (1996).
   PubMed Web of Science ®Google Scholar
• Kunschak M, Engl W, Maritsch F et al. A randomized, controlled trial to study the efficacy and safety of C1 inhibitor concentrate in treating hereditary angioedema. Transfusion38(6), 540–549 (1998).
   PubMed Web of Science ®Google Scholar
• Visentin DE, Yang WH, Karsh J. C1-esterase inhibitor transfusions in patients with hereditary angioedema. Ann. Allergy Asthma Immunol.80(6), 457–461 (1998).
   PubMedGoogle Scholar
• Bork K, Barnstedt SE. Treatment of 193 episodes of laryngeal edema with C1 inhibitor concentrate in patients with hereditary angioedema. Arch. Intern. Med.161(5), 714–718 (2001).
   PubMed Web of Science ®Google Scholar
• Bork K, Meng G, Staubach P, Hardt J. Treatment with C1 inhibitor concentrate in abdominal pain attacks of patients with hereditary angioedema. Transfusion45(11), 1774–1784 (2005).
   PubMed Web of Science ®Google Scholar
• Levi M, Choi G, Picavet C, Hack CE. Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. J. Allergy Clin. Immunol.117(4), 904–908 (2006).
   PubMed Web of Science ®Google Scholar
• Gompels MM, Lock RJ, Abinun M et al. C1 inhibitor deficiency: consensus document. Clin. Exp. Immunol.139(3), 379–394 (2005).
   PubMed Web of Science ®Google Scholar
• Longhurst HJ. Emergency treatment of acute attacks in hereditary angioedema due to C1 inhibitor deficiency: what is the evidence? Int. J. Clin. Pract.59(5), 594>–599 (2005).
   Google Scholar
• Farkas H, Gyeney L, Gidofalvy E, Fust G, Varga L. The efficacy of short-term danazol prophylaxis in hereditary angioedema patients undergoing maxillofacial and dental procedures. J. Oral Maxillofac. Surg.57(4), 404–408 (1999).
   PubMed Web of Science ®Google Scholar
• Frank MM, Gelfand JA, Atkinson JP. Hereditary angioedema: the clinical syndrome and its management. Ann. Intern. Med.84(5), 580–593 (1976).
   PubMed Web of Science ®Google Scholar
• Cicardi M, Bergamaschini L, Cugno M, Hack E, Agostoni G, Agostoni A. Long-term treatment of hereditary angioedema with attenuated androgens: a survey of a 13-year experience. J. Allergy Clin. Immunol.87(4), 768–773 (1991).
   PubMed Web of Science ®Google Scholar
• Frank MM. Hereditary angioedema: the clinical syndrome and its management in the United States. Immunol. Allergy Clin. North Am.26(4), 653–668 (2006).
   PubMed Web of Science ®Google Scholar
• Bork K, Bygum A, Hardt J. Benefits and risks of danazol in hereditary angioedema: a long-term survey of 118 patients. Ann. Allergy Asthma Immunol. (2007) (In press).
   Google Scholar
• Zurlo JJ, Frank MM. The long-term safety of danazol in women with hereditary angioedema. Fertil. Steril.54(1), 64–72 (1990).
   PubMed Web of Science ®Google Scholar
• Cicardi M, Castelli R, Zingale LC, Agostoni A. Side effects of long-term prophylaxis with attenuated androgens in hereditary angioedema: comparison of treated and untreated patients. J. Allergy Clin. Immunol.99(2), 194–196 (1997).
   PubMed Web of Science ®Google Scholar
• Bork K, Schneiders V. Danazol-induced hepatocellular adenoma in patients with hereditary angio-oedema. J. Hepatol.36(5), 707–709 (2002).
   PubMed Web of Science ®Google Scholar
• Szeplaki G, Varga L, Valentin S et al. Adverse effects of danazol prophylaxis on the lipid profiles of patients with hereditary angioedema. J. Allergy Clin. Immunol.115(4), 864–869 (2005).
   PubMed Web of Science ®Google Scholar
• De Serres J, Groener A, Lindner J. Safety and efficacy of pasteurized C1 inhibitor concentrate (Berinert P) in hereditary angioedema: a review. Transfus. Apher. Sci.29(3), 247–254 (2003).
   PubMed Web of Science ®Google Scholar
• Martinez-Saguer I, Mueller W, Puersuen EA et al. Pharmacokinetic parameters of C1 inhibitor concentrate in 40 patients with hereditary angioedema (HAE) – a prospective study. Haemophilia8, 574 (2002).
   Google Scholar
• Bork K, Hardt J, Schicketanz KH, Ressel N. Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency. Arch. Intern. Med.163(10), 1229–1235 (2003).
   PubMed Web of Science ®Google Scholar
• Farkas H, Harmat G, Fust G, Varga L, Visy B. Clinical management of hereditary angio-oedema in children. Pediatr. Allergy Immunol.13(3), 153–161 (2002).
   PubMed Web of Science ®Google Scholar
• Bork K, Koch P. Episodes of severe dyspnea caused by snoring-induced recurrent edema of the soft palate in hereditary angioedema. J. Am. Acad. Dermatol.45(6), 968–969 (2001).
   PubMedGoogle Scholar
• Farkas H, Jakab L, Temesszentandrasi G et al. Hereditary angioedema: a decade of human C1-inhibitor concentrate therapy. J. Allergy Clin. Immunol.120(4), 941–947 (2007).
   PubMed Web of Science ®Google Scholar
• Leimgruber A, Jaques WA, Spaeth PJ. Hereditary angioedema: uncomplicated maxillofacial surgery using short-term C1 inhibitor replacement therapy. Int. Arch. Allergy Immunol.101(1), 107–112 (1993).
   PubMed Web of Science ®Google Scholar
• Langton D, Weiner J, Fary W. C1-esterase inhibitor concentrate prevents upper airway obstruction in hereditary angio-oedema. Med. J. Aust.160(6), 383–384 (1994).
   PubMedGoogle Scholar
• Maves KK, Weiler JM. Tonsillectomy in a patient with hereditary angioedema after prophylaxis with C1 inhibitor concentrate. Ann. Allergy73(5), 435–438 (1994).
   PubMedGoogle Scholar
• Mohr M, Pollok-Kopp B, Gotze O, Burchardi H. The use of a C1-inhibitor concentrate for short-term preoperative prophylaxis in two patients with hereditary angioedema. Anaesthesist45(7), 626–630 (1996).
   PubMed Web of Science ®Google Scholar
• Lehmann A, Lang J, Boldt J, Saggau W. Successful off-pump coronary artery bypass graft surgery in a patient with hereditary angioedema. J. Cardiothorac. Vasc. Anesth.16(4), 473–476 (2002).
   PubMed Web of Science ®Google Scholar
• Bork K, Witzke G. Long-term prophylaxis with C1-inhibitor (C1 INH) concentrate in patients with recurrent angioedema caused by hereditary and acquired C1-inhibitor deficiency. J. Allergy Clin. Immunol.83(3), 677–682 (1989).
   PubMed Web of Science ®Google Scholar
• Hermans C. Successful management with C1-inhibitor concentrate of hereditary angioedema attacks during two successive pregnancies: a case report. Arch. Gynecol. Obstet.276(3), 271–276 (2007).
   PubMedGoogle Scholar
• Juers M, Groener A. Virus safety of current plasma-derived C1-INH: the Aventis Behring experience. J. Allergy Clin. Immunol.114, S96–S98 (2004).
   Google Scholar
• Carugati A, Pappalardo E, Zingale LC, Cicardi M. C1-inhibitor deficiency and angioedema. Mol. Immunol.38(2–3), 161–173 (2001).
   PubMedGoogle Scholar
• Klarmann D, Kreuz W, Joseph-Steiner J, Ehrenforth S. Hepatitis C and pasteurized C1-inhibitor concentrate. Transfusion36(1), 84–85 (1996).
   PubMedGoogle Scholar
• De Filippi F, Castelli R, Cicardi M et al. Transmission of hepatitis G virus in patients with angioedema treated with steam-heated plasma concentrates of C1 inhibitor. Transfusion38(3), 307–311 (1998).
   PubMed Web of Science ®Google Scholar
• Bork K. Prospective virus safety follow-up after therapy with a pasteurised C1 inhibitor concentrate. Biomed. Progr.2, 59–61 (1992).
   Google Scholar
• Cicardi M, Mannucci PM, Castelli R, Rumi MG, Agostoni A. Reduction in transmission of hepatitis C after the introduction of a heat-treatment step in the production of C1-inhibitor concentrate. Transfusion35(3), 209–212 (1995).
   PubMed Web of Science ®Google Scholar
• Longhurst HJ, Carr S, Khair K. C1-inhibitor concentrate home therapy for hereditary angioedema: a viable, effective treatment option. Clin. Exp. Immunol.147(1), 11–17 (2007).
   PubMed Web of Science ®Google Scholar
• Zuraw BL. Novel therapies for hereditary angioedema. Immunol. Allergy Clin. North Am.26(4), 691–708 (2006).
   PubMedGoogle Scholar
• Schneider L, Lumry W, Vegh A, Williams AH, Schmalbach T. Critical role of kallikrein in hereditary angioedema pathogenesis: a clinical trial of ecallantide, a novel kallikrein inhibitor. J. Allergy Clin. Immunol.6, 6 (2007).
   Google Scholar
• Bork K, Frank J, Grundt B, Schlattmann P, Nussberger J, Kreuz W. Treatment of acute edema attacks in hereditary angioedema with a bradykinin receptor-2 antagonist (Icatibant). J. Allergy Clin. Immunol.119(6), 1497–1503 (2007).
   PubMed Web of Science ®Google Scholar
• Choi G, Soeters MR, Farkas H et al. Recombinant human C1-inhibitor in the treatment of acute angioedema attacks. Transfusion47(6), 1028–1032 (2007).
   PubMed Web of Science ®Google Scholar
• Cicardi M, Bergamaschini L, Marasini B et al. Hereditary angioedema: an appraisal of 104 cases. Am. J. Med. Sci.284(1), 2–9 (1982).
   PubMed Web of Science ®Google Scholar