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Hereditary angioedema - Symptom

Alternative Names

Quincke's disease

Symptoms:

Airway blockage -- involves throat swelling and sudden hoarseness
Repeat episodes of abdominal cramping without obvious cause
Swelling in the arms, legs, lips, eyes, tongue, or throat
Swelling of the intestines -- can be severe and leads to vomiting, dehydration, diarrhea, pain, and occasionally shock

Signs and tests:

Blood tests will be done to check the levels of C1 inhibiting factor, C4 and C2.

Antibodies

Reviewed last on: 5/1/2008
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Stuart I. Henochowicz, MD, FACP, Associate Clinical Professor of Medicine, Division of Allergy, Immunology, and Rheumatology, Georgetown University Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

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

Levi M, Choi G, Picavet C, Hack CE. Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. J Allergy Clin Immunol. 2006 Apr;117(4):904-8. Epub 2006 Feb 14.

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):714-8.

Farkas H, Fust G, Fekete B, Karadi I, Varga L. Eradication of Helicobacter pylori and improvement of hereditary angioneurotic oedema. Lancet. 2001 Nov 17;358(9294):1695-6.

Visy B, Fust G, Bygum A, et al. Helicobacter pylori infection as a triggering factor of attacks in patients with hereditary angioedema. Helicobacter. 2007 Jun;12(3):251-7.

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