Abstract
A 33-year-old man who was taken to our emergency department after experiencing cardiopulmonary arrest exhibited edema of his face, neck and laryngopharynx upon arrival. We performed a cricothyroidotomy for emergency airway control, enabling cardiopulmonary resuscitation, as we were unable to provide ventilation using a bag valve mask and intubation. Spontaneous circulation was restored at nine minutes after arrival at the hospital. He was admitted to the ICU thereafter and underwent therapeutic hypothermia but was diagnosed as having suffered clinical brain death and died on the 24th day of hospital admission. We suspected a hereditary angioedema (HAE) based on his past history and his family history. He had been diagnosed as having this condition based on the presence of low levels of complement component 4 (C4) and complement factor 1 inhibitor (C1-INH) activity. HAE may be suspected if characteristic clinical and family histories are present. However, HAE is not widely recognized among Japanese clinicians, and a rapid diagnostic technique is unavailable. Furthermore, many hospitals do not stock C1-INH product, which is the only effective drug. Therefore, wider recognition of HAE and the developments of a rapid diagnostic technique, as well as a system for supplying C1-INH urgently, are needed.
