A case of hereditary angioedema not manifestated classical autosomal dominant trait.
- Author:
Jung A LEE
1
;
Byoung Gye NAH
;
Hyounjeong JUN
;
Jeoug Chul SEO
;
Mi Kyeong KIM
Author Information
1. Department of Internal Medicine, College of Medicine, Chungbuk National University, Cheongjoo, Korea.
- Publication Type:Case Report
- Keywords:
Hereditary angioedema;
C1 esterase inhibitor(C1 INHr)
- MeSH:
Adolescent;
Adult;
Angioedemas, Hereditary*;
Complement C1 Inhibitor Protein;
Complement System Proteins;
Emergency Service, Hospital;
Eyelids;
Fathers;
Foot;
Hand;
Humans;
Immunodiffusion;
Male;
Siblings;
Skin;
Testis
- From:Korean Journal of Allergy
1997;17(4):574-579
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
32 year-old male patient has experienced the repeated swelling of the skin on the eyelid, both hand, foot, lower and upper extrimities and testicle spontaneously without any trauma since 17 years old. This happening has subsided with or without treatment after 2-3 days. His great grandfather had suffered from the same events but grandfather or father or even his two brothers and one sister didn't have any swellings like him. Two days after this attack, he visited emergency room, his serum complement level was decreased such as C1q 9.7mg/dl (10-20mg/dl), C3 52mg/dl (55-120mg/dl), C4 4mg/dl (20-50mg/dl) measured by single radial immunodiffusion (SRID), but two weeks after full recovery, C1q 11.2mg/dl, C3 79mg/dl, C4 5mg/dl. The level of C1 esterase inhibitor was decreased upto 8.1 mg/dl (> 11 mg/dl) measured by nephlemeter(Mitshibishi Co., Japan). Now he is just under the close observation without androgen treatment because the attack of HAE(hereditary angioedema) happens much less and less severe after adolescence.
