Screening for hereditary angioedema (HAE) at 13 emergency centers in Osaka, Japan: A prospective observational study.
Hereditary angioedema (HAE) with deficiency of C1 inhibitor (C1-INH) is an autosomal-dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. The objective is to study the incidence of HAE among patients who visit the emergency department.This was a 3-year prospective observational screening study involving 13 urban tertiary emergency centers in Osaka prefecture, Japan. Patients were included if they met the following criteria: unexplained edema of the body, upper airway obstruction accompanied by edema, anaphylaxis, acute abdomen with intestinal edema (including ileus and acute pancreatitis), or asthma attack. C1-INH activity and C4 level were measured at the time of emergency department admission during the period between July 2011 and June 2014.This study comprised 66 patients with a median age of 54.0 (IQR: 37.5-68.3) years. Three patients were newly diagnosed as having HAE, and 1 patient had already been diagnosed as having HAE. C1-INH activity levels of the patients with HAE were below the detection limit (<25%), whereas those of non-HAE patients (n = 62) were 106% (IQR: 85.5%-127.0%) (normal range, 70%-130%). The median level of C4 was significantly lower in the patients with HAE compared with those without HAE (1.2 [IQR: 1-3] mg/dL vs 22 [IQR: 16.5-29.5] mg/dL, P < 0.01) (normal range, 17-45 mg/dL).Three patients with undiagnosed HAE were diagnosed as having HAE in the emergency department during the 3-year period. If patients have signs and symptoms suspicious of HAE, the levels of C1-INH activity and C4 should be measured.
Department of Traumatology and Acute Critical Medicine, Osaka University Graduate School of Medicine Emergency and Critical Care Medical Center, Osaka Police Hospital Senri Critical Care Medical Center, Osaka Saiseikai Senri Hospital Emergency and Critical Care Medicine, Kishiwada Tokushukai Hospital Senshu Trauma and Critical Care Center, Rinku General Medical Center Emergency Division, Osaka Red Cross Hospital Department of Emergency Medicine, Osaka General Medical Center Osaka Prefectural Nakakawachi Medical Center of Acute Medicine Traumatology and Critical Care Medical Center, National Hospital Organization Osaka National Hospital Emergency and Critical Care Medical Center, Osaka City General Hospital Osaka Mishima Emergency Critical Care Center Department of Critical Care Medical Center, Kinki University School of Medicine Department of Trauma and Critical Care Medicine, Osaka City University Graduate School of Medicine, Osaka Department of Internal Medicine, Kyushu University Beppu Hospital, Oita, Japan.
Tomoya
T
Hirose
Futoshi
F
Kimbara
Masahiro
M
Shinozaki
Yasuaki
Y
Mizushima
Hidehiko
H
Yamamoto
Masashi
M
Kishi
Takeyuki
T
Kiguchi
Shigeru
S
Shiono
Mitsuhiro
M
Noborio
Akihiro
A
Fuke
Hiroshi
H
Akimoto
Takaaki
T
Kimura
Shinichiro
S
Kaga
Takahiko
T
Horiuchi
Takeshi
T
Shimazu
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D054179:Angioedemas, Hereditary / Q000097:blood / Q000175:diagnosis* / Q000503:physiopathology*; D050718:Complement C1 Inhibitor Protein / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D004636:Emergency Service, Hospital; D005260:Female; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D062606:Tertiary Care Centers
D050718:Complement C1 Inhibitor Protein; D003181:Complement C4