zabmedvestnikЗабайкальский медицинский вестникTransbaikalian Medical Bulletin1998-6173Читинская государственная медицинская академия10.52485/19986173_2024_2_152zabmedvestnik-44Research ArticleНАУЧНЫЕ ОБЗОРЫ, ИЛЛЮСТРИРОВАННЫЕ КЛИНИЧЕСКИМ НАБЛЮДЕНИЕМSCIENTIFIC REVIEWS ILLUSTRATED WITH CLINICAL CASE STUDIESГИПЕРАММОНИЕМИЯ В НЕОНАТОЛОГИИ: СЛОЖНОСТИ ДИАГНОСТИКИ, ОСОБЕННОСТИ ВЕДЕНИЯHYPERAMMONEMIA IN NEONATOLOGY: DIAGNOSTIC DIFFICULTIES, MANAGEMENT FEATURESПоповаН. Г.PopovaN. G.

672000, г. Чита, ул. Горького, 39 А; 

672038, г. Чита, ул. Коханского, 16

39 a Gorky str., Chita, 672000; 

16 Kohanskogo str., Chita, 672038

КочероваВ. В.KocherovaV. V.

672000, г. Чита, ул. Горького, 39 А; 

672038, г. Чита, ул. Коханского, 16

39 a Gorky str., Chita, 672000; 

16 Kohanskogo str., Chita, 672038

КонтееваМ. В.KonteevaM. V.

672038, г. Чита, ул. Коханского, 16

16 Kohanskogo str., Chita, 672038

ФГБОУ ВО «Читинская государственная медицинская академия» Минздрава России; ГБУЗ "Забайкальский краевой перинатальный центр"РоссияChita State Medical Academy; Chita Zabaikalsky regional Perinatal CenterRussian FederationГБУЗ "Забайкальский краевой перинатальный центр"РоссияChita Zabaikalsky regional Perinatal CenterRussian Federation20242407202402152160Copyright © Попова Н.Г., Кочерова В.В., Контеева М.В., 20242024Попова Н.Г., Кочерова В.В., Контеева М.В.Popova N.G., Kocherova V.V., Konteeva M.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.zabmedvestnik.ru/jour/article/view/44

Различают первичные и вторичные гипераммониемии. Первичные гипераммониемии – наследственные нарушения цикла образования мочевины. Вторичные гипераммониемии могут возникать как при наследственных заболеваниях (митохондриальных болезнях, органических ацидуриях/аминоацидопатиях), так и при перенесенной в родах асфиксии, поражении печени различной этиологии (токсическом, инфекционном и т. д.), внепеченочной портальной гипертензии, почечной недостаточности, инфекциях, вызванных уреазо-продуцирующими микроорганизмами, заболеваниях, сопровождающихся повышенным катаболизмом, и носить транзиторный характер.

Независимо от этиологии, гипераммониемия может стать причиной необратимого поражения клеток головного мозга, что определяет исключительную важность ранней диагностики и своевременной терапии.

There are primary and secondary hyperammonemia. Primary hyperammonemia is a hereditary disorder of the urea formation cycle. Secondary hyperammonemia can occur both in hereditary diseases (mitochondrial diseases, organic aciduria/aminoacidopathies) and in asphyxia suffered in childbirth, liver damage of various etiologies (toxic, infectious, etc.), extrahepatic portal hypertension, renal failure, infections caused by urease-producing microorganisms, diseases accompanied by increased catabolism, etc. transient nature. Regardless of the etiology, hyperammonemia can cause irreversible damage to brain cells, which determines the exceptional importance of early diagnosis and timely therapy.

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