nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.17650/2311-1267-2017-4-3-51-57nodgo-315Research ArticleОбзоры литературыКлассификация первичных иммунодефицитов как отражение современных представлений об их патогенезе и терапевтических подходахClassification of primary immunodeficiencies as a reflection of modern ideas about their pathogenesis and therapeutic approachesКузьменкоН. Б.KuzmenkoN. B.

Россия, 117997, Москва, ул. Саморы Машела, 1

1 Samory Mashela St., Moscow, 117997, Russia

plunge@list.ruЩербинаА. Ю.ShcherbinaA. Yu.

Россия, 117997, Москва, ул. Саморы Машела, 1

1 Samory Mashela St., Moscow, 117997, Russia

ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaRussian Federation201721092017435157Copyright © Кузьменко Н.Б., Щербина А.Ю., 20172017Кузьменко Н.Б., Щербина А.Ю.Kuzmenko N.B., Shcherbina A.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nodgo.org/jour/article/view/315

Первичные иммунодефициты (ПИД) представляют собой разнородную группу генетически обусловленных заболеваний, вызван- ных дефектами более чем 300 различных генов. Современная классификация ПИД построена на основном патогенетическом механизме заболеваний, однако в большой мере опирается на наличие того или иного генетического дефекта. Таким образом, роль молекулярно-генетических методов обследования пациентов выходит на первый план и нередко определяет подходы к таргетной консервативной и куративной терапии ПИД. Несмотря на успешное использование новых терапевтических подходов, терапия внутривенными иммуноглобулинами остается актуальной для подавляющего большинства пациентов с ПИД.

 

Primary immune deficiencies (PID) are the heterogeneous group of genetically associated disorders developed due to defects of more than 300 different genes. Modern classification of PID based on main pathogenic mechanism of disease, but to a large extent relies on presence of any genetic defect. Thus, role of molecular and genetic methods of investigation of patients comes to the fore and often determines approaches to a target conservative or curative therapy of PIDs. Despite of successful application of new therapeutic approaches, therapy with the help of intravenous immune globulins remains actual for majority of patients with PID.

 

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The authors declare that there are no conflicts of interest present.