Consequences of Misdiagnosed and Mismanaged Hereditary Angioedema Laryngeal Attacks: An Overview of Cases from the Romanian Registry

Emergency department (ED) physicians frequently encounter patients presenting with angioedema. Most of these involve histamine-mediated angioedema; however, less common forms of angioedema (bradykinin-mediated) also occur. It is vital physicians correctly recognize and treat this; particularly since bradykinin-mediated angioedema does not respond to antihistamines, corticosteroids or epinephrine and hereditary angioedema (HAE) laryngeal attacks can be fatal. Here we present four case reports illustrating how failures in recognizing, managing, and treating laryngeal edema due to HAE led to asphyxiation and death of the patient. Recognition of the specific type of angioedema is critical for rapid and effective treatment of HAE attacks. Bradykinin-mediated angioedema should be efficiently differentiated from the most common histamine-mediated form. Improved awareness of HAE and the associated risk of life-threatening laryngeal edema among emergency physicians, patients, and relatives and clear ED treatment protocols are warranted. Moreover, appropriate treatments should be readily available to reduce fatalities associated with laryngeal edema.

[1]  Yusuke Suzuki,et al.  Suffocation due to Acute Airway Edema in a Patient with Hereditary Angioedema Highlighted the Need for Urgent Improvements in Treatment Availability in Japan , 2018, Internal medicine.

[2]  R. Lockey,et al.  The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update , 2018, Allergy.

[3]  J. Peter,et al.  Hereditary angio-oedema in the Western Cape Province, South Africa. , 2018, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[4]  J. Bernstein,et al.  Angioedema in the emergency department: a practical guide to differential diagnosis and management , 2017, International Journal of Emergency Medicine.

[5]  S. Boor,et al.  Blindness, tetraspasticity, and other signs of irreversible brain damage in hereditary angioedema. , 2017, Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology.

[6]  C. Camargo,et al.  Emergency Department Management of Hereditary Angioedema Attacks: Patient Perspectives. , 2017, The journal of allergy and clinical immunology. In practice.

[7]  M. Cicardi,et al.  Pathophysiology of Hereditary Angioedema. , 2014, Pediatric allergy, immunology, and pulmonology.

[8]  Hong-Yu Zhang,et al.  Upper airway edema in 43 patients with hereditary angioedema. , 2014, Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology.

[9]  C. Camargo,et al.  A consensus parameter for the evaluation and management of angioedema in the emergency department. , 2014, Academic emergency medicine : official journal of the Society for Academic Emergency Medicine.

[10]  J. Bernstein,et al.  Emerging concepts in the diagnosis and treatment of patients with undifferentiated angioedema , 2012, International Journal of Emergency Medicine.

[11]  K. Bork,et al.  Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. , 2012, The Journal of allergy and clinical immunology.

[12]  J. Bernstein,et al.  Diagnosis and management of hereditary angioedema: an emergency medicine perspective. , 2012, The Journal of emergency medicine.

[13]  H. Farkas,et al.  Management of upper airway edema caused by hereditary angioedema , 2010, Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology.

[14]  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.

[15]  William H. Yang,et al.  Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. , 2008, Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology.

[16]  A. Tordai,et al.  Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond , 2004, Journal of Allergy and Clinical Immunology.

[17]  K. Bork,et al.  Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema. , 2003, Journal of the American Dental Association.

[18]  K. Bork,et al.  Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency. , 2003, Archives of internal medicine.

[19]  K. Bork,et al.  Treatment of 193 episodes of laryngeal edema with C1 inhibitor concentrate in patients with hereditary angioedema. , 2001, Archives of internal medicine.

[20]  K. Bork,et al.  Asphyxiation by laryngeal edema in patients with hereditary angioedema. , 2000, Mayo Clinic proceedings.

[21]  M. Cicardi,et al.  Hereditary and Acquired C1‐Inhibitor Deficiency: Biological and Clinical Characteristics in 235 Patients , 1992, Medicine.

[22]  K. Ohela Hereditary angioneurotic oedema in Finland. Clinical, immunological and genealogical studies. , 2009, Acta medica Scandinavica.

[23]  J. Atkinson,et al.  Hereditary angioedema: the clinical syndrome and its management. , 1976, Annals of internal medicine.

[24]  N. S. Landerman Hereditary angioneurotic edema. I. Case reports and review of the literature. , 1962, The Journal of allergy.

[25]  W. Osler HEREDITARY ANGIO‐NEUROTIC ŒDEMA , 1888 .