• 1
    Quincke H. Original reports: on acute localized cutaneous dropsy. Appl Dermatol Mon 1882: 1: 12931.
  • 2
    Osler W. Hereditary angio-neurotic oedema. Am J Med Sci 1888: 95: 3627.
  • 3
    Zuraw BL. Clinical practice: hereditary angioedema. N Engl J Med 2008: 359: 102736.
  • 4
    Winnewisser J, Rossi M, Spath P, Burgi H. Type I hereditary angio-oedema. Variability of clinical presentation and course within two large kindreds. J Intern Med 1997: 241: 3946.
  • 5
    Bowen T, Cicardi M, Farkas H, et al. 2010 international consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol 2010: 6: 2436.
  • 6
    Cicardi M, Bork K, Caballero T, et al. Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: a consensus report of an international working group. Allergy 2012: 67: 14757.
  • 7
    Craig T, Pursun EA, Bork K, et al. WAO guideline for the management of hereditary angioedema. WAO J 2012: 5: 18299.
  • 8
    Wahn V, Aberer W, Eberl W, et al. Hereditary angioedema (HAE) in children and adolescents—a consensus on therapeutic strategies. Eur J Pediatr 2012: 171: 133948.
  • 9
    Lang DM, Aberer W, Bernstein JA, et al. International consensus on hereditary and acquired angioedema. Ann Allergy Asthma Immunol 2012: 109: 395402.
  • 10
    Davis AE III. C1 inhibitor and hereditary angioneurotic edema. Ann Rev Immunol 1988: 6: 595628.
  • 11
    Davis AE III. The pathophysiology of hereditary angioedema. Clin Immunol 2005: 114: 39.
  • 12
    Landerman NS, Webster ME, Becker EL, Ratcliffe HE. Hereditary angioneurotic edema II. Deficiency of inhibitor for serum globulin permeability factor and/or plasma kallikrein. J Allergy 1962: 33: 33041.
  • 13
    Donaldson VH, Evans RR. A biochemical abnormality in hereditary angioedema: absence of serum inhibitor of C'1-esterase. Am J Med 1963: 35: 3744.
  • 14
    Rosen FS, Charache J, Pensky J, Donaldson V. Hereditary angioneurotic edema: two genetic variants. Science 1965: 148: 9578.
  • 15
    Pappalardo E, Cicardi M, Duponchel C, et al. Frequent de novo mutations and exon deletions in the C1inhibitor gene of patients with angioedema. J Allergy Clin Immunol 2000: 106: 114754.
  • 16
    Blanch A, Roche O, Urrutia I, et al. First case of homozygous C1 inhibitor deficiency. J Allergy Clin Immunol 2006: 118: 13305.
  • 17
    Buyukozturk S, Eroglu BK, Gelincik A, et al. A Turkish family with a novel mutation in the promoter region of the C1 inhibitor gene. J Allergy Clin Immunol 2009: 123: 9624.
  • 18
    Lopez-Lera A, Favier B, de la Cruz RM, Garrido S, Drouet C, Lopez-Trascasa M. A new case of homozygous C1-inhibitor deficiency suggests a role for Arg378 in the control of kinin pathway activation. J Allergy Clin Immunol 2010: 126: 130711.
  • 19
    Bork K, Meng G, Staubach P, Jardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med 2006: 119: 26774.
  • 20
    Matesic D, Perez ERF, Vlahakis NE, Hagan JB. Acute pancreatitis due to hereditary angioedema. Ann Allergy Asthma Immunol 2006: 97: 6114.
  • 21
    Prematta MJ, Bewtra AK, Levy RJ, et al. Per-attack reporting of prodromal symptoms concurrent with C1-inhibitor treatment of hereditary angioedema attacks. Adv Ther 2012: 29: 91322.
  • 22
    Ricketti AJ, Cleri DJ, Ramos-Bonner LS, Vernaleo JR. Hereditary angioedema presenting in late middle age after angiotensin-converting enzyme inhibitor treatment. Ann Allergy Asthma Immunol 2007: 98: 397401.
  • 23
    Bork K, Hardt J, Witzke G. Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol 2012: 130: 6927.
  • 24
    Grumach AS, Valle SOR, Toledo E, et al. Hereditary angioedema: first report of the Brazilian registry and challenges. J Eur Acad Dermatol Venereol 2012: 27: e33844.
  • 25
    Pedrosa M, Caballero T, Gomez-Traseira C, Olveira A, Lopez-Serrano C. Usefulness of abdominal ultrasonography in the follow-up of patients with hereditary C1-inhibitor deficiency. Ann Allergy Asthma Immunol 2009: 102: 4836.
  • 26
    Donaldson VH, Rosen FS. Hereditary angioneurotic edema: a clinical survey. Pediatrics 1966: 37: 101727.
  • 27
    Farkas H. Pediatric hereditary angioedema due to C1-inhibitor deficiency. Allergy Asthma Clin Immunol 2010: 6: 1827.
  • 28
    Bygum A, Fagerberg CR, Ponard D, Monnier N, Lunardi J, Drouet C. Mutational spectrum and phenotypes in Danish families with hereditary angioedema because of C1 inhibitor deficiency. Allergy 2011: 66: 7684.
  • 29
    Roche O, Blanch A, Caballero T, Sastre N, Callejo D, Lopez-Trascasa M. Hereditary angioedema due to C1 inhibitor deficiency: patient registry and approach to the prevalence in Spain. Ann Allergy Asthma Immunol 2005: 94: 498503.
  • 30
    Elhachem C, Amiour M, Guillot M, Laurent J. Hereditary angioneurotic edema: a case report in a 3-year-old child. Arch Pediatr 2005: 12: 12326.
  • 31
    Lumry WR, Castaldo AJ, Vernon MK, Blaustein MB, Wilson DA, Horn PT. The humanistic burden of hereditary angioedema: impact on health-related quality of life, productivity, and depression. Allergy Asthma Proc 2010: 31: 40714.
  • 32
    Bewtra AK, Levy RJ, Jacobson KW, Wasserman RL, Machnig T, Craig TJ. C1-inhibitor therapy for hereditary angioedema attacks: prospective patient assessments of health-related quality of life. Allergy Asthma Proc 2012: 33: 42731.
  • 33
    Tarzi MD, Hickey A, Forster T, Mohammadi M, Longhurst HJ. An evaluation of tests used for the diagnosis and monitoring of C1 inhibitor deficiency: normal serum C4 does not exclude hereditary angio-edema. Clin Exp Immunol 2007: 149: 5136.
  • 34
    Kelemen Z, Moldovan D, Mihaly E, et al. Baseline level of functional C1-inhibitor correlates with disease severity scores in hereditary angioedema. Clin Immunol 2010: 134: 3548.
  • 35
    Nielsen EW, Thidemann H, Holt JJ, Mollnes TE. C1 inhibitor and diagnosis of hereditary angioedema in newborns. Pediatr Res 1994: 35: 1847.
  • 36
    Jacobs J. Recurrent attacks of hereditary angioedema: a case of delayed diagnosis. Allergy Asthma Proc 2011: 32: S236.
  • 37
    Gower RG. HAE update: determining optimal patient specific therapy. Allergy Asthma Proc 2013: 32: 712.
  • 38
    Zuraw BL. Current and future therapy for hereditary angioedema. Clin Immunol 2005: 114: 106.
  • 39
    Gelfand JA, Serins RJ, Alling DW, Frank MM. Treatment of hereditary angioedema with danazol. Reversal of clinical and biochemical abnormalities. N Engl J Med 1976: 295: 14448.
  • 40
    Banerji A, Sloane DE, Sheffer AL. Hereditary angioedema: a current state-of-the-art review, V: attenuated androgens for the treatment of hereditary angioedema. Ann Allergy Asthma Immunol 2008: 100: S1922.
  • 41
    Thoufeeq MH, Ishtiaq J, Abuzakouk M. Danazol-induced hepatocellular carcinoma in a patient with hereditary angioedema. J Gastrointest Cancer 2012: 43: 2802.
  • 42
    Farkas H, Harmat G, Fust G, Varga L, Visy B. Clinical management of hereditary angio-oedema in children. Pediatr Allergy Immunol 2002: 13: 15361.
  • 43
    Tappeiner G, Hintner H, Glatzl J, Wolff K. Hereditary angio-oedema: treatment with danazol: report of a case. Br J Dermatol 1979: 100: 20712.
  • 44
    Barakat A, Castaldo AJ. Hereditary angioedema: danazol therapy in a 5-year old child. Am J Dis Child 1993: 147: 9312.
  • 45
    Sheffer AL, Austen KF, Rosen FS. Tranexamic acid therapy in hereditary angioneurotic edema. N Engl J Med 1972: 287: 4524.
  • 46
    Farkas H, Csuka D, Zotter Z, Varga L, Fust G. Prophylactic therapy in children with hereditary angioedema. J Allergy Clin Immunol 2013: 131: 57982.
  • 47
    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: 3838.
  • 48
    Craig TJ, Levy RL, Wasserman RL, et al. Efficacy of human C1 esterase inhibitor concentrate compared with placebo in acute hereditary angioedema. J Allergy Clin Immunol 2009: 124: 8018.
  • 49
    Craig TJ, Bewtra AK, Bahna SL, et al. C1 esterase inhibitor concentrate in 1085 hereditary angioedema attacks—final results of the I.M.P.A.C.T. 2 study. Allergy 2011: 66: 160411.
  • 50
    Zuraw BL, Busse PJ, White M, et al. Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema. N Engl J Med 2010: 363: 51322.
  • 51
    Zuraw BL, Kalfus I. Safety and efficacy of prophylactic nanofiltered C1-inhibitor in hereditary angioedema. Am J Med 2012: 125: 938.e17.
  • 52
    Farkas H, Csuka D, Zotter Z, et al. Treatment of attacks with plasma derived C1-inhibitor concentrate in pediatric hereditary angioedema patients. J Allergy Clin Immunol 2013: 131: 90911.
  • 53
    Schneider L, Hurewitz D, Wasserman R, et al. C1-INH concentrate for treatment of acute hereditary angioedema: a pediatric cohort from the I.M.P.A.C.T. studies. Pediatr Allergy Immunol 2013: 24: 5460.
  • 54
    Lumry W, Manning ME, Hurewitz DS, et al. Nanofiltered C1-esterase inhibitor for the acute management and prevention of hereditary angioedema attacks due to C1-inhibitor deficiency in children. J Pediatr 2013: 162: 101722.
  • 55
    Zuraw B, Cicardi M, Levy RJ, et al. Recombinant human C1-inhibitor for the treatment of acute angioedema attacks in patients with hereditary angioedema. J Allergy Clin Immunol 2010: 126: 8217.
  • 56
    Moldovan D, Reshef A, Fabiani J, et al. Efficacy and safety of recombinant human C1-inhibitor for the treatment of attacks of hereditary angioedema: European open-label extension study. Clin Exp Allergy 2012: 42: 92935.
  • 57
    Hack CE, Relan A, Baboeram A. Immunosafety of recombinant human C1-inhibitor in hereditary angioedema: evaluation of IgE antibodies. Clin Drug Investig 2013: 33: 27581.
  • 58
    Cicardi M, Levy RL, McNeil DL, et al. Ecallantide for the treatment of acute attacks in hereditary angioedema. N Engl J Med 2010: 363: 52331.
  • 59
    Levy RJ, Lumry WR, McNeil DL, et al. EDEMA4: a phase 3, double-blind study of subcutaneous ecallantide treatment for acute attacks of hereditary angioedema. Ann Allergy Asthma Immunol 2010: 104: 5239.
  • 60
    Lumry WR, Bernstein JA, Li HH, et al. Efficacy and safety of ecallantide in treatment of recurrent attacks of hereditary angioedema: open-label continuation study. Allergy Asthma Proc 2013: 34: 15561.
  • 61
    Dyax Corp. Kalbitor [package insert]. Cambridge, MA: Dyax Corp; December 2009.
  • 62
    MacGinnitie AJ, Davis-Lorton M, Stolz LE, Tachdjian R. Use of ecallantide in pediatric hereditary angioedema. Pediatrics 2013: 132: e4907. doi: 10.1542/peds.2013-0646.
  • 63
    Cicardi M, Banerji A, Bracho F, et al. Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema. N Engl J Med 2010: 363: 53241.
  • 64
    Lumry WR, Li HH, Levy RJ, et al. Randomized placebo-controlled trial of the bradykinin B2 receptor antagonist icatibant for the treatment of hereditary angioedema: the FAST-3 trial. Ann Allergy Asthma Immunol 2011: 104: 5239.
  • 65
    Cole SW, Lundquist LM. Icatibant for the treatment of hereditary angioedema. Ann Pharmacother 2013: 47: 4955.
  • 66
    Farkas H, Zotter Z, Csuka D. Short-term prophylaxis in hereditary angioedema due to deficiency of the C1-inhibitor—a long-term survey. Allergy 2012: 67: 158693.