162
Views
35
CrossRef citations to date
0
Altmetric
Review

The use of plasma-derived C1 inhibitor in the treatment of hereditary angioedema

, MD, , , &
Pages 3173-3181 | Published online: 26 Nov 2007

Bibliography

  • LEVY LR, LEPOW IH: Assay and properties of serum inhibitor of C'1-esterase. Proc. Soc. Exp. Biol. Med. (1959) 101:608-611.
  • PENSKY J, LEVY LR, LEPOW IH: Partial purification of a serum inhibitor of C1 esterase. J. Biol. Chem. (1961) 236:1674-1679.
  • RATNOFF OD, PENSKY J, DONALDSON VH, AMIR J: The inhibitory properties of plasma against activated plasma thromboplastin antecedent (factor XIa) in hereditary angioneurotic edema. J. Lab. Clin. Med. (1972) 80(6):803-809.
  • HARPEL PC, COOPER NR: Studies on human plasma C1 inactivator-enzyme interactions. I. Mechanisms of interaction with C1s, plasmin, and trypsin. J. Clin. Invest. (1975) 55(3):593-604.
  • SIM RB, ARLAUD GJ, COLOMB MG: C1 inhibitor-dependent dissociation of human complement component C1 bound to immune complexes. Biochem. J. (1979) 179(3):449-457.
  • ZICCARDI RJ: Activation of the early components of the classical complement pathway under physiologic conditions. J. Immunol. (1981) 126(5):1769-1773.
  • SCHAPIRA M, SCOTT CF, COLMAN RW: Contribution of plasma protease inhibitors to the inactivation of kallikrein in plasma. J. Clin. Invest. (1982) 69(2):462-468.
  • VAN DER GRAAF F, KOEDAM JA, BOUMA BN: Inactivation of kallikrein in human plasma. J. Clin. Invest. (1983) 71(1):149-158.
  • DE AGOSTINI A, LIJNEN HR, PIXLEY RA, COLMAN RW, SCHAPIRA M: Inactivation of factor XII active fragment in normal plasma. Predominant role of C-1-inhibitor. J. Clin. Invest. (1984) 73(6):1542-1549.
  • HARPEL PC, LEWIN MF, KAPLAN AP: Distribution of plasma kallikrein between C-1 inactivator and α2-macroglobulin in plasma utilizing a new assay for α2-macroglobulin–kallikrein complexes. J. Biol. Chem. (1985) 260(7):4257-4263.
  • PIXLEY RA, SCHAPIRA M, COLMAN RW: The regulation of human factor XIIa by plasma proteinase inhibitors. J. Biol. Chem. (1985) 260(3):1723-1729.
  • BOOTH NA, WALKER E, MAUGHAN R, BENNETT B: Plasminogen activator in normal subjects after exercise and venous occlusion: t-PA circulates as complexes with C1-inhibitor and PAI-1. Blood (1987) 69(6):1600-1604.
  • MATSUSHITA M, THIEL S, JENSENIUS JC, TERAI I, FUJITA T: Proteolytic activities of two types of mannose-binding lectin-associated serine protease. J. Immunol. (2000) 165(5):2637-2642.
  • GAL P, HARMAT V, KOCSIS A et al.: A true autoactivating enzyme. Structural insight into mannose-binding lectin-associated serine protease-2 activations. J. Biol. Chem. (2005)280:33435-33444.
  • CUGNO M, BOS I, LUBBERS Y, HACK CE, AGOSTONI A: In vitro interaction of C1-inhibitor with thrombin. Blood Coagul. Fibrinolysis (2001) 12(4):253-260.
  • PATSTON PA, GETTINS P, BEECHEM J, SCHAPIRA M: Mechanism of serpin action: evidence that C1 inhibitor functions as a suicide substrate. Biochemistry (1991) 30(36):8876-8882.
  • LOMAS DA, BELORGEY D, MALLYA M et al.: Molecular mousetraps and the serpinopathies. Biochem. Soc. Trans. (2005) 33(Part 2):321-330.
  • BEINROHR L, HARMAT V, DOBO J et al.: C1-inhibitor serpin domain structure reveals the likely mechanism of heparin potentiation and conformational disease. J. Biol. Chem. (2007) 282:21100-21109.
  • COUTINHO M, AULAK KS, DAVIS AE III: Functional analysis of the serpin domain of C1 inhibitor. J. Immunol. (1994) 153(8):3648-3654.
  • BOCK SC, SKRIVER K, NIELSEN E et al.: Human C1 inhibitor: primary structure, cDNA cloning, and chromosomal localization. Biochemistry (1986) 25(15):4292-4301.
  • Donaldson VH, Evans RR: A biochemical abnormality in herediatry angioneurotic edema: absence of serum inhibitor of C′ 1-esterase. Am. J. Med. (1963) 31:37-44.
  • PICKERING RJ, GOOD RA, KELLY JR, GEWURZ H: Replacement therapy in hereditary angioedema. Successful treatment of two patients with fresh frozen plasma. Lancet (1969) 1(7590):326-330.
  • HILL BJ, THOMAS SH, MCCABE C: Fresh frozen plasma for acute exacerbations of hereditary angioedema. Am. J. Emerg. Med. (2004) 22(7):633.
  • PREMATTA M, GIBBS JG, PRATT EL, STOUGHTON TR, CRAIG TJ: Fresh frozen plasma for the treatment of hereditary angioedema. Ann. Allergy Asthma Immunol. (2007) 98(4):383-388.
  • VOGELAAR EF, BRUMMELHUIS HG, KRIJNEN HW: Contributions to the optimal use of human blood. 3. Large-scale preparation of human c1 esterase inhibitor concentrate for clinical use. Vox Sang. (1974) 26(2):118-127.
  • TERPSTRA FG, KLEIJN M, KOENDERMAN AH et al.: Viral safety of C1-inhibitor NF. Biologicals (2007) 35(3):173-181.
  • 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. (1980) 302(10):542-546.
  • WICKERHAUSER M, WILLIAMS C, KOLEN BL, BUSBY TF: Heat stability of lyophilized C1 inactivator concentrates. Vox Sang. (1987) 53(1):1-6.
  • WAYTES AT, ROSEN FS, FRANK MM: Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate. N. Engl. J. Med. (1996) 334(25):1630-1634.
  • 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. Transfusion (1995) 35(3):209-212.
  • DE SERRES J, GRONER A, LINDNER J: Safety and efficacy of pasteurized C1 inhibitor concentrate (Berinert P) in hereditary angioedema: a review. [email protected]. Transfus. Apher. Sci. (2003) 29(3):247-254.
  • Kodama J, Uchida K, Nakayama R: Clinical effect of C1 Inhibitor concentrate in patients with hereditary angioneurotic edema. Shinryo to Shinyaku(1987) 24:2041-2053.
  • MARTINEZ-SAGUER I, MÜLLER W, AYGÖREN PÜRSÜN E et al.: Pharmacokinetic parameters of C1-inhibitor concentrate in 40 patients with hereditary angio-oedema (HAE) – a prospective study. Haemophilia (2002) 8:574.
  • QUASTEL M, HARRISON R, CICARDI M, ALPER CA, ROSEN FS: Behavior in vivo of normal and dysfunctional C1 inhibitor in normal subjects and patients with hereditary angioneurotic edema. J. Clin. Invest. (1983) 71(4):1041-1046.
  • CICARDI M, BERGAMASCHINI L, MARASINI B et al.: Hereditary angioedema: an appraisal of 104 cases. Am. J. Med. Sci. (1982) 284(1):2-9.
  • MINTA JO: The role of sialic acid in the functional activity and the hepatic clearance of C1-INH. J. Immunol. (1981) 126(1):245-249.
  • CAI S, DAVIS AE III: Complement regulatory protein C1 inhibitor binds to selectins and interferes with endothelial–leukocyte adhesion. J. Immunol. (2003) 171(9):4786-4791.
  • POULLE M, BURNOUF-RADOSEVICH M, BURNOUF T: Large-scale preparation of highly purified human C1-inhibitor for therapeutic use. Blood Coagul. Fibrinolysis (1994) 5:543-549.
  • MARASINI B, CICARDI M, AGOSTONI A: Treatment of hereditary angioedema with a plasmatic concentrate of CU esterase inhibitor. Boll. Soc. Ital. Cardiol. (1976) 21(8):1449-1452.
  • MARASINI B, CICARDI M, MARTIGNONI GC, AGOSTONI A: Treatment of hereditary angioedema. Klin. Wochenschr. (1978) 56(16):819-823.
  • AGOSTONI A, BERGAMASCHINI L, MARTIGNONI G, CICARDI M, MARASINI B: Treatment of acute attacks of hereditary angioedema with C1-inhibitor concentrate. Ann. Allergy (1980) 44(5):299-301.
  • AGOSTONI A, CICARDI M, BERGAMASCHINI L, BOCCASSINI G, TUCCI A: C1-inhibitor concentrate for treatment of hereditary angioedema [letter]. N. Engl. J. Med. (1980) 303(9):527.
  • LOGAN RA, GREAVES MW: Hereditary angio-oedema: treatment with C1 esterase inhibitor concentrate. J. R. Soc. Med. (1984) 77(12):1046-1048.
  • LAURENT J, INTRATOR L, BRANELLEC A et al.: Hereditary angioneurotic edema. Treatment of a pseudosurgical digestive attack with C1 inhibitor concentrate. Presse Med. (1987) 16(16):762-764.
  • 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. (1989) 83(3):677-682.
  • OBTULOWICZ K, GLUSZKO P, RADWAN J, SZCZEKLIK A: New possibilities of treating acute angioedema caused by C1-inhibitor deficiency. Pol. Tyg. Lek. (1989) 44(27):646-648.
  • WUTHRICH B: Hereditary angioedema – substitution therapy with C1-inhibitor concentrate. Hautarzt (1989) 40(10):651.
  • AGOSTONI A, CICARDI M: Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine (Baltimore) (1992) 71(4):206-215.
  • LEIMGRUBER A, JAQUES WA, SPAETH PJ: Hereditary angioedema: uncomplicated maxillofacial surgery using short-term C1 inhibitor replacement therapy. Int. Arch. Allergy Immunol. (1993) 101(1):107-112.
  • LANGTON D, WEINER J, FARY W: C1-esterase inhibitor concentrate prevents upper airway obstruction in hereditary angio-oedema [letter]. Med. J. Aust. (1994) 160(6):383-384.
  • MAVES KK, WEILER JM: Tonsillectomy in a patient with hereditary angioedema after prophylaxis with C1 inhibitor concentrate. Ann. Allergy (1994) 73(5):435-438.
  • BERGAMASCHINI L, CICARDI M, TUCCI A et al.: C1 INH concentrate in the therapy of hereditary angioedema. Allergy(1983)38:81-84.
  • 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. Transfusion (1998) 38(6):540-549.
  • VISENTIN DE, YANG WH, KARSH J: C1-esterase inhibitor transfusions in patients with hereditary angioedema. Ann. Allergy Asthma Immunol. (1998) 80(6):457-461.
  • ALVAREZ JM: Successful use of C1 esterase inhibitor protein in a patient with hereditary angioneurotic edema requiring coronary artery bypass surgery. J. Thorac. Cardiovasc. Surg. (2000) 119(1):168-171.
  • ECKERT S, EIFRIG B, STANDL T: Perioperative treatment of a patient with hereditary angioedema (HAE) in a case of a young patient with osteosynthesis from a femoral fracture. Anasthesiol. Intensivmed. Notfallmed. Schmerzther. (2000) 35(12):776-781.
  • MYERS JN, O'NEIL KM, CARREGAL VA: Successful extubation with use of C1 esterase inhibitor concentrate in a patient with hereditary angioedema [letter]. Mayo Clin. Proc. (2000) 75(8):869-870.
  • C1 esterase inhibitor: new preparation. A major advance in emergency treatment of hereditary angioneurotic oedema. Prescrire Int. (2001) 10(53):67-70.
  • BORK K, BARNSTEDT SE: Treatment of 193 episodes of laryngeal edema with c1 inhibitor concentrate in patients with hereditary angioedema. Arch. Intern. Med. (2001) 161(5):714-718.
  • FARKAS H, HARMAT G, FUST G, VARGA L, VISY B: Clinical management of hereditary angio-oedema in children. Pediatr. Allergy Immunol. (2002) 13(3):153-161.
  • PASTO CARDONA L, BORDAS ORPINELL J, MERCADAL ORFILA G, PEREZ DE LA VARA A, JODAR MASSANES R: Profhylaxis and treatment of hereditary and acquired angioedema at HUB; use of the C1-esterase inhibitor. Farm. Hosp. (2003) 27(6):346-352.
  • KASAMATSU Y, NAKAYAMA I, KOBAYASHI T: A case of lung cancer with hereditary angioedema treated effectively by chemo-radiotherapy with C1 esterase inhibitor concentrate and danazol. Nihon Kokyuki Gakkai Zasshi (2004) 42(5):435-439.
  • BORK K, MENG G, STAUBACH P, HARDT J: Treatment with C1 inhibitor concentrate in abdominal pain attacks of patients with hereditary angioedema. Transfusion (2005) 45(11):1774-1784.
  • LONGHURST HJ: Emergency treatment of acute attacks in hereditary angioedema due to C1 inhibitor deficiency: what is the evidence? Int. J. Clin. Pract. (2005) 59(5):594-599.
  • ALTMAN AD, MCLAUGHLIN J, SCHELLENBERG R et al.: Hereditary angioedema managed with low-dose danazol and C1 esterase inhibitor concentrate: a case report. J. Obstet. Gynaecol. Can. (2006) 28(1):27-31.
  • LEVI M, CHOI G, PICAVET C, HACK C: Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. J. Allergy Clin. Immunol. (2006) 117(4):904-908.
  • NATHANI F, SULLIVAN H, CHURCHILL D: Pregnancy and C1 esterase inhibitor deficiency: a successful outcome. Arch. Gynecol. Obstet. (2006) 274(6):381-384.
  • STELTER K, LUBBERS CW, ANDRATSCHKE M, LEUNIG A: Quincke's edema: diagnosis and management of 102 patients with sudden upper airway obstruction. Laryngorhinootologie (2007).
  • MARESCAL C, DUCLOY-BOUTHORS AS, LAURENT J et al.: Parturition and angioneurotic oedema. Int. J. Obstet. Anesth. (1999) 8(2):135-137.
  • COX M, HOLDCROFT A: Hereditary angioneurotic oedema: current management in pregnancy. Anaesthesia (1995) 50(6):547-549.
  • AGOSTONI A, AYGOREN-PURSUN E, BINKLEY KE et al.: Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J. Allergy Clin. Immunol. (2004) 114(3 Suppl.):S51-S131.
  • BERGAMASCHINI L, CICARDI M: Recent advances in the use of C1 inhibitor as a therapeutic agent. Mol. Immunol. (2003) 40(2-4):155-158.
  • NZEAKO UC, FRIGAS E, TREMAINE WJ: Hereditary angioedema: a broad review for clinicians. Arch. Intern. Med. (2001) 161(20):2417-2429.
  • LONGHURST HJ, CARR S, KHAIR K: C1-inhibitor concentrate home therapy for hereditary angioedema: a viable, effective treatment option. Clin. Exp. Immunol. (2007) 147(1):11-17.
  • FAY A, ABINUN M: Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency). J. Clin. Pathol. (2002) 55(4):266-270.
  • CICARDI M, ZINGALE L, ZANICHELLI A, PAPPALARDO E, CICARDI B: C1 inhibitor: molecular and clinical aspects. Springer Semin. Immunopathol. (2005) 27(3):286-298.
  • GOMPELS MM, LOCK RJ, ABINUN M et al.: C1 inhibitor deficiency: consensus document. Clin. Exp. Immunol. (2005) 139(3):379-394.
  • 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. (2004) 114(3):629-637.
  • VARGA L, SZEPLAKI G, VISY B et al.: C1-inhibitor (C1-INH) autoantibodies in hereditary angioedema. Strong correlation with the severity of disease in C1-INH concentrate naive patients. Mol. Immunol. (2007) 44(6):1454-1460.
  • DONALDSON VH, BISSLER JJ, WELCH TR, BURTON MF, DAVIS AE III: Antibody to C1-inhibitor in a patient receiving C1-inhibitor infusions for treatment of hereditary angioneurotic edema with systemic lupus erythematosus reacts with a normal allotype of residue 458 of C1-inhibitor. J. Lab. Clin. Med. (1996) 128(4):438-443.
  • DORE M, MORONE G: L'angioedema ereditario. Su un raro caso di shock anafilattico da inibitore purificato di C1-esterasi. V Congresso Associazione Nazionale dei Medici d'Urgenza. Torino (18 – 21 Aprile 1990).
  • ZINGALE LC, PAPPALARDO E, ZANICHELLI A, AGOSTONI A, CICARDI M: C1 inhibitor concentrate: efficacy and adverse reactions. Int. Immunopharmacol. (2002) 318:1385 (Abstr.).
  • ALTER HJ, STRAMER SL, DODD RY: Emerging infectious diseases that threaten the blood supply. Semin. Hematol. (2007) 44(1):32-41.
  • CICARDI M, ZINGALE LC: The deficiency of C1 inhibitor and its treatment. Immunobiology (2007) 212(4-5):325-331.

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.