nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.36485/1561-6274-2023-27-3-23-31BDGFNWnefr-2237Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEWS AND LECTURESВторичный АА-амилоидоз у педиатрических и взрослых пациентов: классификация, этиология, патогенез, диагностика, лечениеSecondary AA-amyloidosis in pediatric and adult patients: classification, etiology, pathogenesis, diagnosis, treatmenthttps://orcid.org/0000-0002-4890-9120СтепановаА. А.StepanovaA. A.

Степанова Арина Александровна, канд. мед. наук, кафедра факультетской педиатрии, ассистент,

19400, Санкт-Петербург, Литовская, д. 2

Arina A. Stepanova, MD, PhD, Department of faculty pediatrics,

194100, Saint-Petersburg, Litovskaya st., 2

ariwka@list.ruhttps://orcid.org/0000-0002-9415-4785СавенковаН. Д.SavenkovaN. D.

Проф. Савенкова Надежда Дмитриевна, д-р мед. наук, кафедра факультетской педиатрии, заведующая кафедрой 2,

19400, Санкт-Петербург, Литовская, д. 2

Prof. Nadezhda D. Savenkova, MD, PhD, DMedSci, Head of the Department of faculty pediatrics,

194100 Saint-Petersburg, Litovskaya st., 2

savenkova.n.spb@mail.ruФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет» Минздрава РоссииРоссияMinistry of Healthcare of the Russian Federation Saint Petersburg State Pediatric Medical UniversityRussian Federation2023110920232732331Copyright © Степанова А.А., Савенкова Н.Д., 20232023Степанова А.А., Савенкова Н.Д.Stepanova A.A., Savenkova N.D.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephrolog.ru/jour/article/view/2237

В обзоре литературы обобщены сведения о современной номенклатуре и классификации амилоидоза, особенностях этиологии, патогенеза, течения, диагностики и лечения системного вторичного АА-амилоидоза у детей и взрослых пациентов. Среди системных форм амилоидоза вторичный АА-амилоидоз составляет 40–60 % случаев. Приведены данные литературы об особенностях развития вторичного АА-амилоидоза при семейной средиземноморской лихорадке (периодической болезни) вследствие гомозиготной или гетерозиготной мутации гена MEFV у детей.

The literature review summarizes information about the current nomenclature and classification of amyloidosis, the features of the etiology, pathogenesis, course, diagnosis and treatment of systemic secondary AA-amyloidosis in children and adult patients. Among the systemic forms of amyloidosis, secondary AA-amyloidosis accounts for 40–60 % of cases. The literature data on the features of the development of secondary AA-amyloidosis in familial Mediterranean fever (periodic illness) due to homozygous or heterozygous mutation of the MEFV gene in children are presented.

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