Two case reports of hereditary angioedema in the same family

Junichi Maehara, Takahiro Mitsui, Katsuhiro Ogawa, Koichiro Yonemitsu
2011 Journal of the Japanese Society of Intensive Care Medicine  
A 50-year-old woman with facial swelling after dental treatment visited our emergency department. We initially suspected that the swelling was caused by a drug-induced allergy; therefore, the patient was administered anti-allergy therapy. However, the patient did not respond to the therapy. On the basis of examination and a medical interview, we suspected that the swelling was caused by hereditary angioedema (HAE). The condition was eventually diagnosed as type I HAE on the basis of low levels
more » ... f complement component 4 (C4) and C1-esterase inhibitor (C1-inh) and their functional activities. The patient was treated with a regimen of antifibrinolytics and 17-α-alkylated androgens for both short-term and long-term prevention of recurrence. The patient's medical interview revealed sudden death of a family member. Herein, we report 2 cases of HAE. In addition, we provide several arguments about the necessity of an epidemiologic survey on HAE, free access to HAE information, and establishment of a C1-inh supply system in our country.
doi:10.3918/jsicm.18.633 fatcat:z7s2wn6y6jeerg4ullxpb3j6ay