WAO Guideline for the Management of Hereditary Angioedema

World Allergy Organization Journal - Tập 5 Số 12 - Trang 182-199 - 2012
Timothy J. Craig1, Emel Aygören Pürsün2, Konrad Bork3, Tom Bowen4, Henrik Balle Boysen5, Henriette Farkas6, Anete Sevciovic Grumach7,8, Constance H. Katelaris9, Richard F. Lockey10, Hilary Longhurst11, William R. Lumry12, Markus Magerl13, I. Martinez‐Saguer2, Bruce Ritchie14, Alexander Nast13, Ruby Pawankar15, Bruce L. Zuraw16, Marcus Maurer13
1Department of Medicine, Pediatrics and Graduate Studies, Penn State University, Hershey, PA
2Center for Pediatric and Juvenile Medicine, J.W. Goethe University, Frankfurt/(Main), Germany
3Department of Dermatology, Johannes Gutenberg–University Mainz, Mainz, Germany
4Departments of Medicine and Pediatrics, University of Calgary, Calgary, Canada
5HAEi, Orsay, France
63rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary
7Department of Dermatology, Faculty of Medicine, University of São Paulo, São Paulo, Brazil
8Outpatient Group of Recurrent Infections and Laboratory of Immunology, Faculty of Medicine ABC
9Department of Medicine, Campbelltown Hospital, University of Western Sydney, Sydney, New South Wales, Australia
10Allergy, Asthma and Immunology Associates of Tampa Bay, University of South Florida, Tampa, FL
11Department of Immunology, Barts Health NHS Trust, London, United Kingdom
12Allergy and Asthma Specialists, Dallas, TX
13Department of Dermatology and Allergy, Allergie-Centrum-Charité, Charité-Universitätsmedizin Berlin, Berlin, Germany
14Department of Medicine University of Alberta, Edmonton, Canada
15Division of Allergy, Dept. of Pediatrics, Nippon Medical School, Tokyo, Japan
16President WAO, Professor of Medicine, Department of Medicine, University of California San Diego and San Diego VA Healthcare.

Tóm tắt

Từ khóa


Tài liệu tham khảo

Brozek, 2009, Grading quality of evidence and strength of recommendations in clinical practice guidelines. Part 1 of 3. An overview of the GRADE approach and grading quality of evidence about interventions, Allergy, 64, 669, 10.1111/j.1398-9995.2009.01973.x

Cicardi, 2012, Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group, Allergy, 67, 147, 10.1111/j.1398-9995.2011.02751.x

Munch, 1985, Non-hereditary angioedema treated with tranexamic acid. A 6-month placebo controlled trial with follow-up 4 years later, Allergy, 40, 92, 10.1111/j.1398-9995.1985.tb02666.x

Lewis, 1991, Idiopathic gastric acid hypersecretion: treatment implications for refractory acid/peptic disorders, Aliment Pharmacol Ther, 5, 15

Gluszko, 1994, Administration of gamma interferon in human subjects decreases plasminogen activation and fibrinolysis without influencing C1 inhibitor, J Lab Clin Med, 123, 232

Cicardi, 1995, Reduction in transmission of hepatitis C after the introduction of a heat-treatment step in the production of C1-inhibitor concentrate, Transfusion, 35, 209, 10.1046/j.1537-2995.1995.35395184276.x

Waytes, 1996, Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate, N Engl J Med, 334, 1630, 10.1056/NEJM199606203342503

Cicardi, 1997, Side effects of long-term prophylaxis with attenuated androgens in hereditary angioedema: comparison of treated and untreated patients, J Allergy Clin Immunol, 99, 194, 10.1016/S0091-6749(97)70095-2

Goring, 1998, Hereditary angioedema in the German-speaking region [in German], Hautarzt, 49, 114

Kunschak, 1998, A randomized, controlled trial to study the efficacy and safety of C1 inhibitor concentrate in treating hereditary angioedema, Transfusion, 38, 540, 10.1046/j.1537-2995.1998.38698326333.x

Farkas, 1999, The efficacy of short-term danazol prophylaxis in hereditary angioedema patients undergoing maxillofacial and dental procedures, J Oral Maxillofac Surg, 57, 404, 10.1016/S0278-2391(99)90280-X

Bork, 2001, Treatment of 193 episodes of laryngeal edema with C1 inhibitor concentrate in patients with hereditary angioedema, Arch Intern Med, 161, 714, 10.1001/archinte.161.5.714

Gompels, 2002, A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency, J Clin Pathol, 55, 145, 10.1136/jcp.55.2.145

Weiler, 2002, Does heparin prophylaxis prevent exacerbations of hereditary angioedema?, J Allergy Clin Immunol, 109, 995, 10.1067/mai.2002.124892

Szeplaki, 2005, Adverse effects of danazol prophylaxis on the lipid profiles of patients with hereditary angioedema, J Allergy Clin Immunol, 115, 864, 10.1016/j.jaci.2004.12.1130

van Doorn, 2005, A phase I study of recombinant human C1 inhibitor in asymptomatic patients with hereditary angioedema, J Allergy Clin Immunol, 116, 876, 10.1016/j.jaci.2005.05.019

Bas, 2006, Novel pharmacotherapy of acute hereditary angioedema with bradykinin B2-receptor antagonist icatibant, Allergy, 61, 1490, 10.1111/j.1398-9995.2006.01197.x

Bork, 2007, Treatment of acute edema attacks in hereditary angioedema with a bradykinin receptor-2 antagonist (Icatibant), J Allergy Clin Immunol, 119, 1497, 10.1016/j.jaci.2007.02.012

Ott, 2007, Treatment of hereditary angioneurotic oedema (HANE) with tibolone, Clin Endocrinol (Oxf), 66, 180, 10.1111/j.1365-2265.2006.02704.x

Schneider, 2007, Critical role of kallikrein in hereditary angioedema pathogenesis: a clinical trial of ecallantide, a novel kallikrein inhibitor, J Allergy Clin Immunol, 120, 416, 10.1016/j.jaci.2007.04.028

Varga, 2007, Anti-cholesterol antibody levels in hereditary angioedema, J Cell Mol Med, 11, 1377, 10.1111/j.1582-4934.2007.00124.x

Visy, 2007, Helicobacter pylori infection as a triggering factor of attacks in patients with hereditary angioedema, Helicobacter, 12, 251, 10.1111/j.1523-5378.2007.00501.x

Birjmohun, 2008, Effects of short-term and long-term danazol treatment on lipoproteins, coagulation, and progression of atherosclerosis: two clinical trials in healthy volunteers and patients with hereditary angioedema, Clin Ther, 30, 2314, 10.1016/j.clinthera.2008.12.021

Cedzynski, 2008, Possible disease-modifying factors: the mannan-binding lectin pathway and infections in hereditary angioedema of children and adults, Arch Immunol Ther Exp (Warsz), 56, 69, 10.1007/s00005-008-0004-7

Szegedi, 2008, Long-term danazol prophylaxis does not lead to increased carotid intima-media thickness in hereditary angioedema patients, Atherosclerosis, 198, 184, 10.1016/j.atherosclerosis.2007.09.025

Craig, 2009, Efficacy of human C1 esterase inhibitor concentrate compared with placebo in acute hereditary angioedema attacks, J Allergy Clin Immunol, 124, 801, 10.1016/j.jaci.2009.07.017

Kreuz, 2009, C1-inhibitor concentrate for individual replacement therapy in patients with severe hereditary angioedema refractory to danazol prophylaxis, Transfusion, 49, 1987, 10.1111/j.1537-2995.2009.02230.x

Bas, 2010, Therapeutic efficacy of icatibant in angioedema induced by angiotensin-converting enzyme inhibitors: a case series, Ann Emerg Med, 56, 278, 10.1016/j.annemergmed.2010.03.032

Bernstein, 2010, Population pharmacokinetics of plasma-derived C1 esterase inhibitor concentrate used to treat acute hereditary angioedema attacks, Ann Allergy Asthma Immunol, 105, 149, 10.1016/j.anai.2010.06.005

Cicardi, 2010, Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema, N Engl J Med, 363, 532, 10.1056/NEJMoa0906393

Cicardi, 2010, Ecallantide for the treatment of acute attacks in hereditary angioedema, N Engl J Med, 363, 523, 10.1056/NEJMoa0905079

Craig, 2010, Prospective study of rapid relief provided by C1 esterase inhibitor in emergency treatment of acute laryngeal attacks in hereditary angioedema, J Clin Immunol, 30, 823, 10.1007/s10875-010-9442-1

Levy, 2010, EDEMA4: a phase 3, double-blind study of subcutaneous ecallantide treatment for acute attacks of hereditary angioedema, Ann Allergy Asthma Immunol, 104, 523, 10.1016/j.anai.2010.04.012

Zuraw, 2010, Recombinant human C1-inhibitor for the treatment of acute angioedema attacks in patients with hereditary angioedema, J Allergy Clin Immunol, 126, 821, 10.1016/j.jaci.2010.07.021

Zuraw, 2010, Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema, N Engl J Med, 363, 513, 10.1056/NEJMoa0805538

Bissler, 1994, Contiguous deletion and duplication mutations resulting in type 1 hereditary angioneurotic edema, Hum Genet, 93, 265, 10.1007/BF00212020

Zhang, 2000, AluY insertion (IVS4-52ins316alu) in the glycerol kinase gene from an individual with benign glycerol kinase deficiency, Hum Mutat, 15, 316, 10.1002/(SICI)1098-1004(200004)15:4<316::AID-HUMU3>3.0.CO;2-9

Bowen, 2003, Management of hereditary angioedema: a Canadian approach, Transfus Apher Sci, 29, 205, 10.1016/j.transci.2003.08.009

Bowen, 2004, Canadian 2003 International Consensus Algorithm For the Diagnosis, Therapy, and Management of Hereditary Angioedema, J Allergy Clin Immunol, 114, 629, 10.1016/j.jaci.2004.06.043

Bracho, 2005, Hereditary angioedema, Curr Opin Hematol, 12, 493, 10.1097/01.moo.0000179805.57486.4e

Chinen, 2005, Basic and clinical immunology, J Allergy Clin Immunol, 116, 411, 10.1016/j.jaci.2005.05.010

Gompels, 2005, C1 inhibitor deficiency: consensus document, Clin Exp Immunol, 139, 379, 10.1111/j.1365-2249.2005.02726.x

Bowen, 2008, Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema, Ann Allergy Asthma Immunol, 100, S30, 10.1016/S1081-1206(10)60584-4

Bowen, 2010, Hereditary angioedema consensus 2010, Allergy Asthma Clin Immunol, 6, 13, 10.1186/1710-1492-6-13

Bowen, 2010, Management of hereditary angioedema: 2010 Canadian approach, Allergy Asthma Clin Immunol, 6, 20, 10.1186/1710-1492-6-20

Bowen, 2010, 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema, Allergy Asthma Clin Immunol, 6, 24, 10.1186/1710-1492-6-24

Longhurst, 2010, HAE international home therapy consensus document, Allergy Asthma Clin Immunol, 6, 22, 10.1186/1710-1492-6-22

Dinkelacker E. Ueber acutes Oedem [dissertation]. Kiel, Germany: Unversity of Kiel; 1882.

Rosen, 1969, The "neurotic edema" (hereditary angioedema), N Engl J Med, 280, 1356, 10.1056/NEJM196906122802414

Cicardi, 1982, Hereditary angioedema: an appraisal of 104 cases, Am J Med Sci, 284, 2, 10.1097/00000441-198207000-00001

Bork, 2006, Hereditary angioedema: new findings concerning symptoms, affected organs, and course, Am J Med, 119, 267, 10.1016/j.amjmed.2005.09.064

Longhurst, 2006, Hereditary angioedema: causes, manifestations and treatment, Br J Hosp Med (Lond), 67, 654, 10.12968/hmed.2006.67.12.22439

Kaplan, 2005, Angioedema, J Am Acad Dermatol, 53, 373, 10.1016/j.jaad.2004.09.032

Seidman, 1990, Angioedema related to angiotensin-converting enzyme inhibitors, Otolaryngol Head Neck Surg, 102, 727, 10.1177/019459989010200617

Nussberger, 1998, Plasma bradykinin in angio-oedema, Lancet, 351, 1693, 10.1016/S0140-6736(97)09137-X

Agostoni, 1999, Angioedema due to angiotensin-converting enzyme inhibitors, Immunopharmacology, 44, 21, 10.1016/S0162-3109(99)00107-1

Rosen, 1965, Hereditary angioneurotic edema: two genetic variants, Science, 148, 957, 10.1126/science.148.3672.957

Roche, 2005, Hereditary angioedema due to C1 inhibitor deficiency: patient registry and approach to the prevalence in Spain, Ann Allergy Asthma Immunol, 94, 498, 10.1016/S1081-1206(10)61121-0

Bygum, 2009, Hereditary angio-oedema in Denmark: a nationwide survey, Br J Dermatol, 161, 1153, 10.1111/j.1365-2133.2009.09366.x

Donaldson, 1964, Action of complement in hereditary angioneurotic edema: the role of C'1-esterase, J Clin Invest, 43, 2204, 10.1172/JCI105094

Donaldson, 1983, Kinin generation in hereditary angioneurotic edema (H.A.N.E.) plasma, Adv Exp Med Biol, 156, 183

Kaplan, 2002, Pathways for bradykinin formation and inflammatory disease, J Allergy Clin Immunol, 109, 195, 10.1067/mai.2002.121316

Donaldson, 1973, Kinin formation in hereditary angioneurotic edema (HANE) plasma, Int Arch Allergy Appl Immunol, 45, 206, 10.1159/000231029

Fields, 1983, Kinin formation in hereditary angioedema plasma: evidence against kinin derivation from C2 and in support of "spontaneous" formation of bradykinin, J Allergy Clin Immunol, 72, 54, 10.1016/0091-6749(83)90052-0

Maurer, 2011, New topics in bradykinin research, Allergy, 66, 1397, 10.1111/j.1398-9995.2011.02686.x

Binkley, 2000, Clinical, biochemical, and genetic characterization of a novel estrogen-dependent inherited form of angioedema, J Allergy Clin Immunol, 106, 546, 10.1067/mai.2000.108106

Bork, 2000, Hereditary angioedema with normal C1-inhibitor activity in women, Lancet, 356, 213, 10.1016/S0140-6736(00)02483-1

Bork, 2006, Hereditary angio-oedema with normal C1 inhibitor in a family with affected women and men, Br J Dermatol, 154, 542, 10.1111/j.1365-2133.2005.07048.x

Caballero, 2011, Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part I. Classification, epidemiology, pathophysiology, genetics, clinical symptoms, and diagnosis, J Investig Allergol Clin Immunol, 21, 333

Karim, 2004, Normal complement C4 values do not exclude hereditary angioedema, J Clin Pathol, 57, 213, 10.1136/jcp.2003.12021

Tarzi, 2007, An evaluation of tests used for the diagnosis and monitoring of C1 inhibitor deficiency: normal serum C4 does not exclude hereditary angio-oedema, Clin Exp Immunol, 149, 513, 10.1111/j.1365-2249.2007.03438.x

Wagenaar-Bos, 2008, Functional C1-inhibitor diagnostics in hereditary angioedema: assay evaluation and recommendations, J Immunol Methods, 338, 14, 10.1016/j.jim.2008.06.004

Zuraw, 2003, Diagnosis and management of hereditary angioedema: an American approach, Transfus Apher Sci, 29, 239, 10.1016/j.transci.2003.08.008

Bork, 2006, Hereditary angioedema with normal C1 inhibitor activity including hereditary angioedema with coagulation factor XII gene mutations, Immunol Allergy Clin North Am, 26, 709, 10.1016/j.iac.2006.09.003

Weiler, 2006, Genetic test indications and interpretations in patients with hereditary angioedema, Mayo Clin Proc, 81, 958, 10.4065/81.7.958

Caballero, 2011, Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part II. Treatment, follow-up, and special situations, J Investig Allergol Clin Immunol, 21, 422

Temino, 2008, The spectrum and treatment of angioedema, Am J Med, 121, 282, 10.1016/j.amjmed.2007.09.024

Zuberbier, 2009, EAACI/GA(2)LEN/EDF/WAO guideline: management of urticaria, Allergy, 64, 1427, 10.1111/j.1398-9995.2009.02178.x

Bork, 2009, Hereditary angioedema caused by missense mutations in the factor XII gene: clinical features, trigger factors, and therapy, J Allergy Clin Immunol, 124, 129, 10.1016/j.jaci.2009.03.038

Farkas, 2001, Angiooedema due to acquired deficiency of C1-esterase inhibitor associated with leucocytoclastic vasculitis, Acta Derm Venereol, 81, 298, 10.1080/00015550152572985

Kaplan, 2001, C1 inhibitor deficiency: hereditary and acquired forms, J Investig Allergol Clin Immunol, 11, 211

Cugno, 2008, Angioedema due to acquired C1inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation, Autoimmun Rev, 8, 156, 10.1016/j.autrev.2008.05.003

Levi, 2006, Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency, J Allergy Clin Immunol, 117, 904, 10.1016/j.jaci.2006.01.002

Bygum, 2009, Self-administration of intravenous C1-inhibitor therapy for hereditary angioedema and associated quality of life benefits, Eur J Dermatol, 19, 147, 10.1684/ejd.2008.0603

Gras, 2009, Icatibant for hereditary angioedema, Drugs Today (Barc), 45, 855, 10.1358/dot.2009.45.12.1424267

Bork, 2010, Recurrent angioedema and the threat of asphyxiation, Dtsch Arztebl Int, 107, 408

Martinez-Saguer, 2010, Characterization of acute hereditary angioedema attacks during pregnancy and breast-feeding and their treatment with C1 inhibitor concentrate, Am J Obstet Gynecol, 203, 131, 10.1016/j.ajog.2010.03.003

Groner, 2012, Pathogen safety of human C1 esterase inhibitor concentrate, Transfusion, 52, 2104, 10.1111/j.1537-2995.2012.03590.x

Ärzteschaft, 2000, Schwerwiegende Thrombenbildung nach Berinert® HS, Deutsches Ärzteblatt, 97, A

Bork, 2009, Hereditary angioedema: increased number of attacks after frequent treatments with C1 inhibitor concentrate, Am J Med, 122, 780, 10.1016/j.amjmed.2009.02.024

Bork, 2011, Hereditary angioedema: long-term treatment with one or more injections of C1 inhibitor concentrate per week, Int Arch Allergy Immunol, 154, 81, 10.1159/000319213

Varga, 2008, Treatment of type I and II hereditary angioedema with Rhucin, a recombinant human C1 inhibitor, Expert Rev Clin Immunol, 4, 653, 10.1586/1744666X.4.6.653

Sheffer, 2011, Ecallantide (DX-88) for acute hereditary angioedema attacks: integrated analysis of 2 double-blind, phase 3 studies, J Allergy Clin Immunol, 128, 153, 10.1016/j.jaci.2011.03.006

Bygum, 2011, Rapid resolution of erythema marginatum after icatibant in acquired angioedema, Acta Derm Venereol, 91, 185, 10.2340/00015555-1055

Bork, 2011, Risk of laryngeal edema and facial swellings after tooth extraction in patients with hereditary angioedema with and without prophylaxis with C1 inhibitor concentrate: a retrospective study, Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 112, 58, 10.1016/j.tripleo.2011.02.034

Zuraw, 2008, Hereditary angiodema: a current state-of-the-art review, IV: short-and long-term treatment of hereditary angioedema: out with the old and in with the new?, Ann Allergy Asthma Immunol, 100, S13, 10.1016/S1081-1206(10)60581-9

Maurer, 2010, Hereditary angioedema: an update on available therapeutic options, J Dtsch Dermatol Ges, 8, 663

Van Sickels, 2010, Hereditary angioedema: treatment, management, and precautions in patients presenting for dental care, Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 109, 168, 10.1016/j.tripleo.2009.09.031

Gompels, 2011, Cinryze (C1-inhibitor) for the treatment of hereditary angioedema, Expert Rev Clin Immunol, 7, 569, 10.1586/eci.11.50

Lunn, 2010, Cinryze as the first approved C1 inhibitor in the USA for the treatment of hereditary angioedema: approval, efficacy and safety, J Blood Med, 1, 163, 10.2147/JBM.S9576

Tallroth, 2011, Long-term prophylaxis of hereditary angioedema with a pasteurized C1 inhibitor concentrate, Int Arch Allergy Immunol, 154, 356, 10.1159/000321830

Blackmore, 1977, Danazol in the treatment of hereditary angio-neurotic oedema, J Int Med Res, 5, 38

Agostoni, 1980, Danazol and stanozolol in long-term prophylactic treatment of hereditary angioedema, J Allergy Clin Immunol, 65, 75, 10.1016/0091-6749(80)90181-5

Wharton, 1982, Hereditary angioedema: response to danazol, Practitioner, 226, 935

Zurlo, 1990, The long-term safety of danazol in women with hereditary angioedema, Fertil Steril, 54, 64, 10.1016/S0015-0282(16)53638-3

Cicardi, 1991, Long-term treatment of hereditary angioedema with attenuated androgens: a survey of a 13-year experience, J Allergy Clin Immunol, 87, 768, 10.1016/0091-6749(91)90120-D

Bork, 2002, Danazol-induced hepatocellular adenoma in patients with hereditary angio-oedema, J Hepatol, 36, 707, 10.1016/S0168-8278(02)00035-1

Bork, 2008, Benefits and risks of danazol in hereditary angioedema: a long-term survey of 118 patients, Ann Allergy Asthma Immunol, 100, 153, 10.1016/S1081-1206(10)60424-3

Farkas, 2010, The effect of long-term danazol prophylaxis on liver function in hereditary angioedemada longitudinal study, Eur J Clin Pharmacol, 66, 419, 10.1007/s00228-009-0771-z

Maurer, 2011, Long-term prophylaxis of hereditary angioedema with androgen derivates: a critical appraisal and potential alternatives, J Dtsch Dermatol Ges, 9, 99

Sheffer, 1972, Tranexamic acid therapy in hereditary angioneurotic edema, N Engl J Med, 287, 452, 10.1056/NEJM197208312870907

Crosher, 1987, Intravenous tranexamic acid in the management of hereditary angio-oedema, Br J Oral Maxillofac Surg, 25, 500, 10.1016/0266-4356(87)90143-4

Farkas, 2002, Clinical management of hereditary angio-oedema in children, Pediatr Allergy Immunol, 13, 153, 10.1034/j.1399-3038.2002.01014.x

Caballero, 2012, International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency, J Allergy Clin Immunol, 129, 308, 10.1016/j.jaci.2011.11.025

Boyle, 2005, Hereditary angio-oedema in children: a management guideline, Pediatr Allergy Immunol, 16, 288, 10.1111/j.1399-3038.2005.00275.x

Farkas, 2010, Pediatric hereditary angioedema due to C1-inhibitor deficiency, Allergy Asthma Clin Immunol, 6, 18, 10.1186/1710-1492-6-18

Martinez-Saguer, 2010, Pharmacokinetic analysis of human plasma-derived pasteurized C1-inhibitor concentrate in adults and children with hereditary angioedema: a prospective study, Transfusion, 50, 354, 10.1111/j.1537-2995.2009.02394.x

Caballero T. Angio-oedema due to hereditary C1 inhibitor deficiency in children. Allergol Immunopathol (Madr). 2012.

Kjaer L, Bygum A. Hereditary angioedema in childhood: a challenging diagnosis you cannot afford to miss. Pediatr Dermatol. 2012.

Davis, 1979, Serum complement levels in infancy: age related changes, Pediatr Res, 13, 1043, 10.1203/00006450-197909000-00019

Roach, 1981, Influence of age and sex on serum complement components in children, Am J Dis Child, 135, 918

Nielsen, 1994, C1 inhibitor and diagnosis of hereditary angioedema in newborns, Pediatr Res, 35, 184, 10.1203/00006450-199402000-00012

Wahn, 2012, Hereditary angioedema (HAE) in children and adolescents--a consensus on therapeutic strategies, Eur J Pediatr, 171, 1339, 10.1007/s00431-012-1726-4

Yip, 1992, Hormonally exacerbated hereditary angioedema, Australas J Dermatol, 33, 35, 10.1111/j.1440-0960.1992.tb00050.x

Visy, 2004, Sex hormones in hereditary angioneurotic oedema, Clin Endocrinol (Oxf), 60, 508, 10.1111/j.1365-2265.2004.02009.x

Bouillet, 2008, Disease expression in women with hereditary angioedema, Am J Obstet Gynecol, 199, 484, 10.1016/j.ajog.2008.04.034

Cox, 1995, Hereditary angioneurotic oedema: current management in pregnancy, Anaesthesia, 50, 547, 10.1111/j.1365-2044.1995.tb06050.x

Duvvur, 2007, Hereditary angioedema and pregnancy, J Matern Fetal Neonatal Med, 20, 563, 10.1080/14767050701411851

Bouillet, 2010, Hereditary angioedema in women, Allergy Asthma Clin Immunol, 6, 17, 10.1186/1710-1492-6-17

Czaller, 2010, The natural history of hereditary angioedema and the impact of treatment with human C1-inhibitor concentrate during pregnancy: a long-term survey, Eur J Obstet Gynecol Reprod Biol, 152, 44, 10.1016/j.ejogrb.2010.05.008

Montinaro, 2010, Management of pregnancy and vaginal delivery by C1 inhibitor concentrate in two hereditary angioedema twins, Clin Immunol, 136, 456, 10.1016/j.clim.2010.05.008

Longhurst, 2007, C1-inhibitor concentrate home therapy for hereditary angioedema: a viable, effective treatment option, Clin Exp Immunol, 147, 11, 10.1111/j.1365-2249.2006.03256.x

Aygoren-Pursun, 2010, On demand treatment and home therapy of hereditary angioedema in Germany--the Frankfurt experience, Allergy Asthma Clin Immunol, 6, 21, 10.1186/1710-1492-6-21

Farkas, 2012, Home treatment of hereditary angioedema with icatibant administered by health care professionals, J Allergy Clin Immunol, 129, 851, 10.1016/j.jaci.2011.10.039

Kreuz, 2012, Home therapy with intravenous human C1-inhibitor in children and adolescents with hereditary angioedema, Transfusion, 52, 100, 10.1111/j.1537-2995.2011.03240.x

Tourangeau, 2012, Safety and efficacy of physician-supervised self-managed c1 inhibitor replacement therapy, Int Arch Allergy Immunol, 157, 417, 10.1159/000329635

Wilson, 2010, Economic costs associated with acute attacks and long-term management of hereditary angioedema, Ann Allergy Asthma Immunol, 104, 314, 10.1016/j.anai.2010.01.024

Zilberberg, 2010, The burden of hospitalizations and emergency department visits with hereditary angioedema and angioedema in the United States, 2007, Allergy Asthma Proc, 31, 511, 10.2500/aap.2010.31.3403

Gorman, 2008, Hereditary angioedema and pregnancy: a successful outcome using C1 esterase inhibitor concentrate, Can Fam Physician, 54, 365

Farkas, 2001, Eradication of Helicobacter pylori and improvement of hereditary angioneurotic oedema, Lancet, 358, 1695, 10.1016/S0140-6736(01)06720-4

Bork, 2003, Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema, J Am Dent Assoc, 134, 1088, 10.14219/jada.archive.2003.0323