A familial case of hereditary angioneurotic edema in Japan

Intern Med. 1992 Mar;31(3):353-6.

A familial case of hereditary angioneurotic edema in Japan.

Yamato H, Nakashima Y, Ninomiya K, Sakurai S, Kuroiwa A.

Second Department of Internal Medicine, University of Occupational and
Environmental Health, School of Medicine, Kitakyushu, Japan.

A 53-year-old man was admitted with impairment of breathing following laryngeal
edema. Serum levels of CH50 (22 U/ml), C4 (3 mg/dl), C1-INH protein (10.6 mg/dl)
and C1-INH activity (LT 25%) were low. Complement study of the patient's family
members revealed that he was one of 5 patients in 3 generations with hereditary
angioneurotic edema (HANE). Administration of the androgen derivatives Danazol
(600 mg/day) and Oxymetholone (30 mg/day) effectively increased serum levels of
C1-INH activity and C4. Though eruption and hepatic dysfunction attributable to
administration of the drugs appeared, these side effects improved after
withdrawal of the drugs. Subsequently, the treatment with Danazol at a low dose
(100 mg/day) was resumed, and the patient has had no episodes of edema for the
past 3 years. Regarding the familial cases of HANE, fewer than 20 have been
reported in Japan.