eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

Angioedema: Follow-up

Author: Shih-Wen Huang, MD, Medical Director of Allergy Service, Professor, Department of Pediatrics, Division of Immunology and Infectious Diseases, University of Florida College of Medicine
Contributor Information and Disclosures

Updated: Oct 21, 2009

Follow-up

Further Inpatient Care

  • Supportive care in angioedema: Patients with acquired C1INH deficiency often have malignancy, especially lymphoproliferative disorder. Management of the underlying disorder should be a priority.
  • Fluid replacement: When a major loss of fluid from the vascular compartment occurs, replacement with physiologic intravascular fluids is necessary to prevent shock.
  • Pain control: When an episode of abdominal colic occurs, narcotics may be required to relieve pain.

Further Outpatient Care

  • Fluid replacement and pain control should be the same as with inpatient care.

Inpatient & Outpatient Medications

  • Prescribe synthetic attenuated androgens such as danazol, stanozolol, and oxandrolone.
  • Consider antifibrinolytic agents, fresh frozen plasma (FFP), or vapor-heated C1INH concentrate.

Deterrence/Prevention

  • Preparation for surgery, dental procedures, and childbirth includes androgens, FFP, or vapor-heated C1INH concentrate.
  • Patients should avoid trauma and contact sports.

Complications

  • Sudden onset of severe laryngeal edema can lead to death.
  • Severe abdominal pain may sometimes subject the patient to surgery.

Prognosis

  • Severe airway edema accounts for almost a 30% mortality rate in untreated patients.

Patient Education

  • Patients should be closely monitored by specialists for lifelong care.
  • Patients should be careful to avoid trauma.
  • Patients should wear a MedicAlert bracelet or carry an identification card at all times.
  • Patients should keep up with prophylactic medication.
  • More information is available from the Hereditary AngioEdema Association.
  • For excellent patient education resources, visit eMedicine's Allergy Center and Skin, Hair, and Nails Center. Also, see eMedicine's patient education article Hives and Angioedema.

Miscellaneous

Special Concerns

  • Pregnancy and delivery are always a medical concern for patients with hereditary angioedema (HAE). However, in a large-scale follow-up study, danazol apparently did not result in any adverse effects.15 The baby demonstrated mild signs of virilization. These results confirm that danazol, even when it is administered at low doses and late in pregnancy (ie, after sexual differentiation of the fetus) can still interfere with normal female external genital phenotype. Therefore, a careful evaluation appears to be appropriate when attenuated androgens are proposed for pregnant women with hereditary angioedema.
  • For more information, see the Hereditary Angioedema Association Web site.
 


More on Angioedema

Overview: Angioedema
Differential Diagnoses & Workup: Angioedema
Treatment & Medication: Angioedema
Follow-up: Angioedema
Multimedia: Angioedema
References
[ CLOSE WINDOW ]

References

  1. 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. Sep 2004;114(3 Suppl):S51-131. [Medline].

  2. Patel NN, Patel DN. Hereditary angioedema with normal C1 inhibitor. Am J Med. Nov 2008;121(11):949-51. [Medline].

  3. Visy B, Fust G, Varga L, et al. Sex hormones in hereditary angioneurotic oedema. Clin Endocrinol (Oxf). Apr 2004;60(4):508-15. [Medline].

  4. Cichon S, Martin L, Hennies HC, Muller F, Van Driessche K, Karpushova A. Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III. Am J Hum Genet. Dec 2006;79(6):1098-104. [Medline].

  5. Prieto A, Tornero P, Rubio M, Fernandez-Cruz E, Rodriguez-Sainz C. Missense mutation Thr309Lys in the coagulation factor XII gene in a Spanish family with hereditary angioedema type III. Allergy. Feb 2009;64(2):284-6. [Medline].

  6. Duan QL, Binkley K, Rouleau GA. Genetic analysis of Factor XII and bradykinin catabolic enzymes in a family with estrogen-dependent inherited angioedema. J Allergy Clin Immunol. Jan 27 2009;[Medline].

  7. Huang SW. Results of an on-line survey of patients with hereditary angioedema. Allergy Asthma Proc. Mar-Apr 2004;25(2):127-31. [Medline].

  8. Cugno M, Zanichelli A, Bellatorre AG, Griffini S, Cicardi M. Plasma biomarkers of acute attacks in patients with angioedema due to C1-inhibitor deficiency. Allergy. Feb 2009;64(2):254-7. [Medline].

  9. Birjmohun RS, Kees Hovingh G, Stroes ES, et al. 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. Dec 2008;30(12):2314-23. [Medline].

  10. Williams A, Baird LG. DX-88 and HAE: a developmental perspective. Transfus Apher Sci. Dec 2003;29(3):255-8. [Medline].

  11. van Doorn MB, Burggraaf J, van Dam T, et al. A phase I study of recombinant human C1 inhibitor in asymptomatic patients with hereditary angioedema. J Allergy Clin Immunol. Oct 2005;116(4):876-83. [Medline].

  12. Kunschak M, Engl W, Maritsch F. A randomized, controlled trial to study the efficacy and safety of C1 inhibitor concentrate in treating hereditary angioedema. Transfusion. Jun 1998;38(6):540-9. [Medline].

  13. Reshef A, Leibovich I, Goren A. Hereditary angioedema: new hopes for an orphan disease. Isr Med Assoc J. Dec 2008;10(12):850-5. [Medline].

  14. Epstein TG, Bernstein JA. Current and emerging management options for hereditary angioedema in the US. Drugs. 2008;68(18):2561-73. [Medline].

  15. Herrmann G, Schneider L, Krieg T. Efficacy of danazol treatment in a patient with the new variant of hereditary angio-oedema (HAE III). Br J Dermatol. Jan 2004;150(1):157-8. [Medline].

  16. Bernstein JA. Hereditary angioedema: a current state-of-the-art review, VIII: current status of emerging therapies. Ann Allergy Asthma Immunol. Jan 2008;100(1 Suppl 2):S41-6. [Medline].

  17. Bork K. Hereditary angioedema with normal C1 inhibitor activity including hereditary angioedema with coagulation factor XII gene mutations. Immunol Allergy Clin North Am. Nov 2006;26(4):709-24. [Medline].

  18. 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. Sep 2004;114(3):629-37. [Medline].

  19. Cicardi M, Agostoni A. Hereditary angioedema. N Engl J Med. Jun 20 1996;334(25):1666-7. [Medline].

  20. Cicardi M, Bergamaschini L, Cugno M. Long-term treatment of hereditary angioedema with attenuated androgens: a survey of a 13-year experience. J Allergy Clin Immunol. Apr 1991;87(4):768-73. [Medline].

  21. Cichon S, Martin L, Hennies HC, et al. Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III. Am J Hum Genet. Dec 2006;79(6):1098-104. [Medline]. [Full Text].

  22. Davis AE 3rd. The pathophysiology of hereditary angioedema. Clin Immunol. Jan 2005;114(1):3-9. [Medline].

  23. 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. May 19 2006;343(4):1286-9. [Medline].

  24. Donaldson VH. Hereditary angioneurotic edema. In: Current Therapy in Allergy, Immunology and Rheumatology. 5th ed. St Louis, MO: Mosby; 1996:75-8.

  25. Farkas H, Harmat G, Fust G. Clinical management of HAE in children. J Allergy Clin Immunol. 2004;114(3):S108-113.

  26. Farkas H, Harmat G, Fust G, Varga L, Visy B. Clinical management of hereditary angio-oedema in children. Pediatr Allergy Immunol. Jun 2002;13(3):153-61. [Medline].

  27. Icabant:HOE 140, JE 049, JE049. Drugs RD. 2004;5:343-8.

  28. Martinez-Saguer, Rusicke E, Aygoren-Pursun, et al. Clinical manifestation and therapy in children with HAE: A prospective follow-up. J Allergy Clin Immunol. 2004;114 (3):S107-108.

  29. Weiler CR, van Dellen RG. Genetic test indications and interpretations in patients with hereditary angioedema. Mayo Clin Proc. Jul 2006;81(7):958-72. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

HANE disease, hereditary angioedema, HAE, hereditary angioneurotic edema, angioedema, urticaria, subcutaneous swelling, generalized urticaria, C1 inhibitor, C1INH, HAE type 1, HAE1, HAE type 2, HAE2, HAE type 3, HAE3, AAE type 1, AAE1, AAE type 2, AAE2, acquired angioedema, C1INH deficiency, angioneurotic edema, nonhistaminergic angioedema, INAE, idiopathic angioedema, allergic angioedema, lymphoid, urticaria-associated angioedema

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Shih-Wen Huang, MD, Medical Director of Allergy Service, Professor, Department of Pediatrics, Division of Immunology and Infectious Diseases, University of Florida College of Medicine
Shih-Wen Huang, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Medical Editor

C Lucy Park, MD, Head, Division of Allergy, Immunology, and Pulmonology, Associate Professor, Department of Pediatrics, University of Illinois at Chicago
C Lucy Park, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Medical Association, Chicago Medical Society, Clinical Immunology Society, and Illinois State Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

John Wilson Georgitis, MD, Consulting Staff, Lafayette Allergy Services
John Wilson Georgitis, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American College of Chest Physicians, American Lung Association, American Medical Writers Association, and American Thoracic Society
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD, Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School
Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.