Hereditary Angioedema: The Clinical Syndrome and its Management in the United States

Immunology and Allergy Clinics of North America

Volumn 26, Issue 4, 2006, Pages 653-668

  Frank, Michael M ^a  

^a DUKE UNIVERSITY MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ADRENALIN; AMINOCAPROIC ACID; AMINOTRANSFERASE; ANDROGEN; ANTIHISTAMINIC AGENT; COMPLEMENT COMPONENT C1S INHIBITOR; CREATINE KINASE; CYCLOPHOSPHAMIDE; DANAZOL; DX88; FRUCTOSE BISPHOSPHATE ALDOLASE; GLUCOCORTICOID; ICATIBANT; KALLIKREIN INHIBITOR; METHYLTESTOSTERONE; NARCOTIC AGENT; OXANDROLONE; PLACEBO; PLASMIN INHIBITOR; PROGESTERONE; STANOZOLOL; TRANEXAMIC ACID; UNCLASSIFIED DRUG;

ABNORMALLY HIGH SUBSTRATE CONCENTRATION IN BLOOD; AIRWAY OBSTRUCTION; AMINOTRANSFERASE BLOOD LEVEL; ANGIONEUROTIC EDEMA; ANXIETY DISORDER; BLADDER DISEASE; BLADDER TELANGIECTASIA; CAUSE OF DEATH; CLINICAL TRIAL; CREATINE KINASE BLOOD LEVEL; DISEASE ASSOCIATION; DISEASE SEVERITY; DRUG CONTRAINDICATION; DRUG EFFICACY; DRUG HALF LIFE; DRUG INDUCED HEADACHE; DRUG MECHANISM; DRUG SUBSTITUTION; DRUG TREATMENT FAILURE; DYSLIPIDEMIA; ENDOCRINE FUNCTION; HAIR DISEASE; HEMATURIA; HUMAN; LIBIDO DISORDER; LIPID BLOOD LEVEL; LIVER DISEASE; LONG TERM CARE; MENSTRUATION DISORDER; MUSCLE CRAMP; MUSCLE WEAKNESS; MYALGIA; MYOPATHY; NONHUMAN; OPTIMAL DRUG DOSE; OUTCOME ASSESSMENT; PATHOPHYSIOLOGY; PELIOSIS HEPATIS; PRIORITY JOURNAL; PROPHYLAXIS; PROTEIN DEFECT; RESPIRATORY TRACT DISEASE; REVIEW; SEDATION; SHORT COURSE THERAPY; SIDE EFFECT; UNITED STATES; VIRILIZATION;

6-AMINOCAPROIC ACID; ANDROGENS; ANGIONEUROTIC EDEMA; ANTIFIBRINOLYTIC AGENTS; CLINICAL TRIALS; COMPLEMENT C1 INHIBITOR PROTEIN; HUMANS; MUTATION; PLASMIN; UNITED STATES;

EID: 33750517560     PISSN: 08898561     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.iac.2006.09.005     Document Type: Review

References (50)

1. Graves R. Clinical lectures on the practice of medicine, 1843. In: Major M.H. (Ed). Classic descriptions of disease. 3rd ed. (1955), Charles C. Thomas, Springfield, IL 623-624
2. Quincke H. Concerning the acute localized oedema of the skin. Monatsh. Prakt. Dermat I (1882) 129-131
3. Osler W. Hereditary angio-neurotic oedema. Am J Med Sci 95 (1888) 362-367
4. Frank M.M., Gelfand J.A., and Atkinson J.P. Hereditary angioedema: the clinical syndrome and its management. Ann Intern Med 84 (1976) 589-593
5. Agostoni A., Cicardi M., Cugno M., et al. Angioedema due to angiotensin-converting enzyme inhibitors. Immunopharmacol 44 (1999) 21-25
6. Frank M.M. Effect of sex hormones on the complement-related clinical disorder of hereditary angioedema. Arthritis Rheum 22 (1979) 1295-1299
7. Blanco del Val A., Sedano Martenez M.E., Carrascal Arranz M.I., et al. Hereditary angioedemal with exclusive abdominal presentation. An Pediatr (Barc) 61 (2004) 346-347
8. Landerman N.S., Webster M.E., Becker E.L., et al. Hereditary angioneurotic edema. J Allergy 33 (1962) 330-341
9. Donaldson V.H., and Evans R.R. A biochemical abnormality in hereditary angioneurotic edema. Am J Med 35 (1963) 37-44
10. Rosen F.S., Pensky J., Donaldson V., et al. Hereditary angioneurotic edems: two genetic varients. Science 148 (1965) 957-958
11. Donaldson V.H., and Rosen F.S. Action of complement in hereditary angioedema: the role of C'1 esterase. J Clin Invest 43 (1969) 2204-2213
12. Strang C.J., Cholin S., Spragg J., et al. Angioedema induced by a peptide derived from complement component C2. J Exp Med 168 5 (1988) 1685-1698
13. Donaldson V.H., Rosen F.S., and Bing D.H. Role of the second component of complement (C2) and plasmin in kinin release in hereditary angioneurotic edema (H.A.N.E.) plasma. Trans Assoc Am Physicians 90 (1977) 174-183
14. Brickman C.M., Frank M.M., and Kaliner M. Urine-histamine levels in patients with hereditary angioedema (HAE). J Allergy Clin Immunol 82 3 pt 1 (1988) 403-406
15. Brickman C.M., Tsokos G.C., Balow J.E., et al. Immunoregulatory diseases associated with hereditary angioedema: 1. Clinical manifestations of autoimmune disease. J Allergy Clin Immunol 77 5 (1986) 749-757
16. Zuraw B.L. Urticaria, angioedema, and autoimmunity. Clin Lab Med 17 3 (1997) 559-569
17. Cicardi M., Zingale L.C., Pappalardo E., et al. Autoantibodies and lymphoproliferative diseases in acquired C1-inhibitor deficiencies. Medicine (Baltimore) 82 4 (2003) 274-281
18. Brickman C.M., Tsokos G.C., Chused T.M., et al. Hereditary angioedema. 2. Serologic and cellular abnormalities. J Allergy Clin Immunol 77 5 (1986) 758-767
19. Spaulding W.B. Methyltestosterone therapy for hereditary episodic edema (hereditary angioneurotic edema). Ann Intern Med 53 (1960) 739-745
20. Gelfand J.A., Sherins R.J., Alling D.W., et al. Treatment of hereditary angioedema with danazol. N Engl J Med 295 (1976) 1444-1448
21. Davis P.J., Davis F.B., and Charache P. Long-term therapy of hereditary angioedema (HAE). Preventive management with fluoxymesterone and oxymetholone in severely affected males and females. Johns Hopkins Med J 135 6 (1974) 391-398
22. Rosse W.F., Logue G.L., Silberman J.R., et al. The effect of synthetic androgens in hereditary angioneurotic edema: alteration of C1 inhibitor and C4 levels. Trans Assoc Am Phys 89 (1976) 122-132
23. Sheffer A.L., Fearon D.T., and Austen K.F. Clinical and biochemical effects of stanozolol therapy for hereditary angioedema. J Allergy Clin Immunol 68 3 (1981) 181-187
24. Lappin D.F., McPhaden A.R., Yap P.L., et al. Monocyte C1-inhibitor synthesis in patients with C1-inhibitor deficiency. Eur J Clin Invest 19 (1989) 45-52
25. Pappalardo E., Zingale L.C., and Cicardi M. Increased expression of C1 inhibitor mRNA in patients with hereditary angioedema treated with danazol. Immunol Lett 86 (2003) 271-276
26. Kang H.R., Yim E.Y., Oh S.Y., et al. Normal C1 inhibitor mRNA expression level in type 1 hereditary angioedema patients: newly found C1 inhibitor gene mutations. Allergy 61 (2006) 260-264
27. Hosea S.W., Santaella M.L., Brown E.J., et al. Long-term therapy of hereditary angioedema with danazol. Ann Intern Med 93 6 (1980) 809-812
28. Zurlo J.J., and Frank M.M. The long-term safety of danazol in women with hereditary angioedema. Fertil Steril 54 (1990) 64-72
29. Cicardi M., Castelli R., Zingale L.C., et al. Side effects of long-term prophylaxis with attenuated androgens in hereditary angioedema: comparison of treated and untreated patients. J Allergy Clin Immunol 99 2 (1997) 194-196
30. 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 (2005) 864-869
31. Church J.A. Oxandrolone treatment of childhood hereditary angioedema. Ann Allergy Asthma Immunol 92 (2004) 377-378
32. Nilsson I.M., Andersson L., and Björkman S.E. Epsilon amino-caproic acid (E-ACA) as a therapeutic agent based on 5 years' clinical experience. Acta Med Scand Suppl 448 (1966) 1-46
33. Lundh B., Laurell A.-B., Wetterqvist H., et al. A case of hereditary angioneurotic edema successfully treated with epsilon-aminocaproic acid: studies on C'1 esterase inhibitor, C'1 activation, plasminogen level and histamine metabolism. Clin Exp Immunol 3 (1968) 733-745
34. Champion R.H., and Lachmann P.J. Hereditary angio-oedema treated with epsilon aminocaproic acid. Br J Dermatol 81 (1969) 763-765
35. Frank M.M., Sergent J.S., Kane M.A., et al. Epsilon aminocaproic acid therapy of hereditary angioneurotic edema. A double-blind study. N Engl J Med 286 (1972) 808-812
36. Sheffer A.L., Austen K.F., and Rosen F.S. Tranexamic acid therapy in hereditary angioneurotic edema. N Engl J Med 287 9 (1972) 452-454
37. Levi M., Choi G., Picavet C., et al. Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. J Allergy Clin Immunol 117 4 (2006) 904-908
38. Jaffe C.J., Atkinson J.P., Gelfand J.A., et al. Hereditary angioedema: the use of fresh frozen plasma for prophylaxis in patients undergoing oral surgery. J Allergy Clin Immunol 55 (1975) 386-393
39. Wall R.T., Frank M.M., and Hahn M. A review of 25 patients with hereditary angioedema requiring surgery (see comments). Anesthesiology 71 2 (1989) 309-311
40. Atkinson J.C., and Frank M.M. Oral manifestations and dental management of patients with hereditary angioedema. J Oral Pathol Med 20 3 (1991) 139-142
41. Sheffer A.L., Fearon D.T., Austen K.F., et al. Tranexamic acid: preoperative prophylactic therapy for patients with hereditary angioneurotic edema. J Allergy Clin Immunol 60 1 (1977) 38-40
42. Farkas H., Gyeney L., Gidofalvy E., et al. The efficacy of short-term danazol prophylaxis in hereditary angioedema patients undergoing maxillofacial and dental procedures. J Oral Maxillofac Surg 57 4 (1999) 404-408
43. Pickering R.J., Good R.A., Kelly J.R., et al. Replacement therapy in hereditary angioedema. Successful treatment of two patients with fresh frozen plasma. Lancet 1 7590 (1969) 326-330
44. Gadek J.E., Hosea S.W., Gelfand J.A., et al. Replacement therapy in hereditary angioedema. N Engl J Med 302 (1980) 542-546
45. Agostoni A., Bergamaschini L., Martignoni G., et al. Treatment of acute attacks of hereditary angioedema with C1-inhibitor concentrate. Ann Allergy 44 5 (1980) 299-301
46. Bork K., Meng G., Staubach P., et al. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med 119 (2006) 267-274
47. Waytes A.T., Rosen F.S., and Frank M.M. Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate. N Engl J Med 334 25 (1996) 1630-1634
48. Bowen B., Hawk J.J., Sibunka S., et al. A review of the reported defects in the human C1 exterase inhibitor gene producing hereditary angioedema including four new mutations. Clin Immunol 98 (2001) 157-163
49. Gompels M.M., Lock R.J., Abinun M., et al. C1 inhibitor deficiency: consensus document. Clin Exp Immunol 139 (2005) 379-394
50. Farkas H. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 2004;114(Suppl 3):S51-S131.