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

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.

[1]  T. Craig,et al.  Patients perception of self-administrated medication in the treatment of hereditary angioedema. , 2015, Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology.

[2]  Y. Tomino,et al.  Clinical manifestations, diagnosis, and treatment of hereditary angioedema: survey data from 94 physicians in Japan. , 2015, Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology.

[3]  H. Longhurst,et al.  Elevated D-dimers in attacks of hereditary angioedema are not associated with increased thrombotic risk , 2015, Allergy.

[4]  R. Lockey,et al.  WAO Guideline for the Management of Hereditary Angioedema , 2012, The World Allergy Organization journal.

[5]  M. Cicardi,et al.  Hereditary angio-oedema , 2012, The Lancet.

[6]  M. Triggiani,et al.  Evidence‐based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group , 2012, Allergy.

[7]  Z. Ikezawa,et al.  [National prevalence survey of hereditary angioedema in Japan]. , 2011, Arerugi = [Allergy].

[8]  P. Keith,et al.  2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema , 2010, Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology.

[9]  N. Marzella,et al.  Cinryze, a human plasma-derived c1 esterase inhibitor for prophylaxis of hereditary angioedema. , 2009, P & T : a peer-reviewed journal for formulary management.

[10]  B. Zuraw.  Clinical practice. Hereditary angioedema. , 2008, The New England journal of medicine.

[11]  M. Frank.  8. Hereditary angioedema. , 2008, The Journal of allergy and clinical immunology.

[12]  K. Kragballe,et al.  Hereditary Angioedema. , 2018, The New England journal of medicine.

[13]  J. Lindner,et al.  Safety and efficacy of pasteurized C1 inhibitor concentrate (Berinert® P) in hereditary angioedema: a review , 2003 .