Suffocation due to Acute Airway Edema in a Patient with Hereditary Angioedema Highlighted the Need for Urgent Improvements in Treatment Availability in Japan

Intern Med. 2018 Nov 1;57(21):3193-3197. doi: 10.2169/internalmedicine.9262-17. Epub 2018 Apr 27.

Abstract

A 42-year-old Japanese man with hereditary angioedema suffered accidental trauma to his jaw in Shizuoka Prefecture, Japan, which gradually caused facial edema. Since plasma-derived human C1 inhibitor (pdh C1-INH) was unavailable, he had to be transferred to Juntendo University Hospital in Tokyo. Due to his severe edema, he suffered asphyxiation leading to cardiopulmonary arrest upon arrival. The patient was resuscitated and promptly treated with pdh C1-INH. In Japan, the self-administration of pdh C1-INH is not allowed, and every prefecture does not always possess stocks of pdh C1-INH. This case emphasizes the need for urgent improvements in treatment availability in Japan.

Keywords: Japan; acute attack; hereditary angioedema; plasma-derived human C1 inhibitor; suffocation; trauma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Airway Obstruction / complications*
  • Angioedemas, Hereditary / complications*
  • Angioedemas, Hereditary / drug therapy*
  • Asphyxia / etiology*
  • Asphyxia / therapy
  • Cardiopulmonary Resuscitation
  • Complement C1 Inhibitor Protein / supply & distribution
  • Complement C1 Inhibitor Protein / therapeutic use*
  • Edema / complications*
  • Face
  • Heart Arrest / etiology
  • Heart Arrest / therapy
  • Humans
  • Japan
  • Jaw / injuries
  • Male
  • Tokyo

Substances

  • Complement C1 Inhibitor Protein
  • SERPING1 protein, human