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. 2013 Jan;5(1):59-61.
doi: 10.4168/aair.2013.5.1.59. Epub 2012 Aug 23.

A case of hereditary angioedema in a 7-year-old korean girl

Meeyong Shin  1 , Kangmo Ahn
Affiliations

A case of hereditary angioedema in a 7-year-old korean girl

Meeyong Shin et al. Allergy Asthma Immunol Res. 2013 Jan.

Abstract

Hereditary angioedema (HAE) is a rare autosomal dominant disease that usually occurs in adolescence and early adulthood. It is characterized by recurrent non-pitting edema involving the skin and intestinal tract, especially the extremities and face. It is not associated with urticaria and pruritus. The cause is known to be the deficiency of C1 inhibitor. We herein report a 7-year-old girl with HAE who had recurrent episodes of swelling of the extremities and face without urticaria and pruritus. Her great grandmother had suffered from the same symptoms. The level of serum C4 was 8.01 mg/dL (normal: 10-40 mg/dL). The level of C1 inhibitor was 5.0 mg/dL (normal: 18-40 mg/dL). To our knowledge, this is the first pediatric case with typical clinical symptoms of HAE and C1 esterase inhibitor deficiency in Korea.

Keywords: Hereditary angioedema; Korea; angioedema; young children.

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Figures

Figure
Figure
The swollen face of the patient during an attack of angioedema.
See this image and copyright information in PMC

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