Hereditary angioedema. Clinical guidelines. (D84.1)

  • Authors: Bliznetz E.A.1, Viktorova E.A.2, Vishneva E.A.3, Dzhobava E.M.4, Il'ina N.I.5, Kuzmenko N.B.6, Latysheva E.A.7, Latysheva T.V.8,9, Manto I.A.8, Namazova-Baranova L.S.3, Nenasheva N.M.10, Pampura A.N.11, Polyakov A.V.1, Selimzianova L.R.12, Shcherbina A.Y.13
  • Affiliations:
    1. Federal State Budgetary Institution Research Centre for Medical Genetics, Moscow, Russian Federation
    2. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation
    3. Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences», Moscow, Russian Federation 4 Pirogov Russian National Research Medical University, Moscow, Russian Federation
    4. LLC group of clinics Crede Expert, Moscow, Russian Federation
    5. National Research Center — Institute of Immunology Federal Medical-Biological Agency of Russia
    6. 3 Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences», Moscow, Russian Federation
    7. National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia, Moscow, Russian Federation
    8. National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia
    9. Moscow State University of Medicine and Dentistry named after A.I. Evdokimov
    10. Russian Medical Academy of Continuous Professional Education of the Ministry of Health of the Russian Federation
    11. The Russian National Research Medical University named after N.I. Pirogov
    12. Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences», Moscow, Russian Federation Pirogov Russian National Research Medical University, Moscow, Russian Federation The State Education Institution of Higher Professional Training The First Sechenov Moscow State Medical University under Ministry of Health of the Russian Federation, Moscow, Russian Federation
    13. Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology
  • Section: Help to the practitioner
  • URL: https://rusalljournal.ru/raj/article/view/1447
  • DOI: https://doi.org/10.36691/RJA1447
  • Cite item

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Abstract

Hereditary angioedema (HAE) is a rare, potentially life-threatening, genetic disease which manifests with the skin and mucous / submucosal swelling that occurs under the influence of bradykinin (BC). Clinical guidelines for Hereditary angioedema: code for the International Statistical Classification of Diseases and Related Health Problems D84.1. In December 2020, were developed by Russian Assotiation of Allergology and Clinical Immunology, Association of Medical Geneticists, National Association of Experts in the Field of Primary Immunodeficiencies, Union of Pediatricians of Russia. In December 2020, the project was approved at a meeting of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (December 10, 2020 No. 743/12).

The presented clinical guidelines are devoted to the optimizing of clinical care for patients with HAE. The relevance of the topic is due to the underdiagnosis of Hereditary angioedema and insufficient awareness of doctors of various specialties about this problem, as well as the numerous difficulties in managing this category of patients.
The clinical guidelines contain information about HAE epidemiology, etiology, classification, pathogenesis and common clinical manifestations. Actual diagnostic approaches and differential diagnosis of HAE are particularly described, and diagnostic criteria have been established. Medical care algorithms to patients with Hereditary angioedema at the prehospital and hospital stages are described. The principles of HAE pharmacotherapy, including long-term prophylaxis, short-term prophylaxis, and acute therapy are described. Treatment regimens are stratified according to patients’ age and gender. Non-drug measures for the prevention HAE attacks are considered.
Information for patients and HAE patient passport in the new clinical guidelines’ edition, sections  have been added.
Clinical guidelines for Hereditary Angioedema are intended for practicing doctors of all specialties, students and teachers of medical universities, residents, graduate students of medical universities.

 

About the authors

Elena A. Bliznetz

Federal State Budgetary Institution Research Centre for Medical Genetics, Moscow, Russian Federation

Email: bliznetzelena@mail.ru
ORCID iD: 0000-0002-5339-5566
SPIN-code: 8451-3075

Cand. Sci. (Med.),

Russian Federation

Ekaterina A. Viktorova

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation

Email: vikat.10@mail.ru
ORCID iD: 0000-0002-2427-1417

Elena A. Vishneva

Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences», Moscow, Russian Federation
4 Pirogov Russian National Research Medical University, Moscow, Russian Federation

Email: vishneva.e@yandex.ru
ORCID iD: 0000-0001-7398-0562
SPIN-code: 1109-2810

Dr. Sci. (Med.),

Eliso M. Dzhobava

LLC group of clinics Crede Expert, Moscow, Russian Federation

Email: super.lis9@yandex.ru
SPIN-code: 8866-4534

Dr. Sci. (Med.);

Russian Federation

Natalya I. Il'ina

National Research Center — Institute of Immunology Federal Medical-Biological Agency of Russia

Email: instimmun@yandex.ru
ORCID iD: 0000-0002-3556-969X
SPIN-code: 6715-5650

MD, Dr. Sci. (Med.), Professor

Russian Federation, 115522, 24 Kashirskoe shosse, Moscow

Natalia B. Kuzmenko

3 Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences», Moscow, Russian Federation

Email: plunge@list.ru
ORCID iD: 0000-0002-1669-8621

Cand. Sci.(Med.)

Elena A. Latysheva

National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia

National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia, Moscow, Russian Federation

Email: ealat@mail.ru
ORCID iD: 0000-0002-1606-205X
SPIN-code: 2063-7973

MD, Dr. Sci. (Med.)

Russian Federation, 24, Kashirskoye shosse, Moscow, 115522

Tatiana V. Latysheva

National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia; Moscow State University of Medicine and Dentistry named after A.I. Evdokimov

Email: tvlat@mail.ru
ORCID iD: 0000-0003-1508-0640
SPIN-code: 8929-7644

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow; Moscow

Irina A. Manto

National Research Center – Institute of Immunology Federal Medical-Biological Agency of Russia

Author for correspondence.
Email: irina.manto@yandex.ru
ORCID iD: 0000-0001-6432-394X
SPIN-code: 7944-5159
Russian Federation, 24, Kashirskoye shosse, Moscow, 115522

Leyla S. Namazova-Baranova

Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences», Moscow, Russian Federation
4 Pirogov Russian National Research Medical University, Moscow, Russian Federation

Email: leyla.s.namazova@gmail.com
ORCID iD: 0000-0002-2209-7531
SPIN-code: 1312-2147

Dr. Sci. (Med.),

Natalia M. Nenasheva

Russian Medical Academy of Continuous Professional Education of the Ministry of Health of the Russian Federation

Email: 1444031@gmail.com
ORCID iD: 0000-0002-3162-2510
SPIN-code: 3363-6170

 Professor, Doctor of Medical Sciences

Russian Federation, 2/1 bld 1, Barrikadnaya ul., Moscow, 125993

Alexander N. Pampura

The Russian National Research Medical University named after N.I. Pirogov

Email: apampura1@mail.ru
ORCID iD: 0000-0001-5039-8473
SPIN-code: 9722-7961

MD, Dr. Sci. (Med.), Professor

Russian Federation, Moscow

Aleksander V. Polyakov

Federal State Budgetary Institution Research Centre for Medical Genetics, Moscow, Russian Federation

Email: apol@dnalab.ru
ORCID iD: 0000-0002-0105-1833
SPIN-code: 6453-3097

Dr. Sci. (Bio), Professor,

Lilia R. Selimzianova

Research Institute of Pediatrics and Children’s Health in «Central Clinical Hospital of the Russian Academy of Sciences», Moscow, Russian Federation
Pirogov Russian National Research Medical University, Moscow, Russian Federation
The State Education Institution of Higher Professional Training The First Sechenov Moscow State Medical University under Ministry of Health of the Russian Federation, Moscow, Russian Federation

Email: lilysir@mail.ru
ORCID iD: 0000-0002-3678-7939
SPIN-code: 5508-1689

Cand. Sci.(Med.)

Anna Yu. Shcherbina

Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology

Email: shcher26@hotmail.com
ORCID iD: 0000-0002-3113-4939
SPIN-code: 6759-0031

head of Immunology Department Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID), PhD, professor

Russian Federation, Moscow

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