Hypodiagnosis of hereditary angioedema (HAO) takes place in Russia. It is a genetically determined, orphan disease, having a potential threat to the patient’s life. As a result, many patients are left without pathogenetically substantiated specialized medical care, have unreasonable surgical interventions and die from fatal asphyxiation.
The article discusses the pathogenesis, clinical course of HAO, special attention is paid to various aspects of diagnosis of this disease, which is identified on the basis of anamnesis, clinical picture and laboratory examination data. Knowing the peculiaties of NAO by doctors of all specializations will contribute to the timely diagnosis of this disease, respectively, to the appointment of adequate basic therapy for patients.

Keywords: timely diagnosis of hereditary angiodema, orphan diseases, hereditary angioedema