zdorПроблемы здоровья и экологииHealth and Ecology Issues2220-09672708-6011Gomel State Medical University10.51523/2708-6011.2026-23-1-16zdor-2931Research ArticleСЛУЧАЙ ИЗ КЛИНИЧЕСКОЙ ПРАКТИКИCLINICAL CASESВрожденный цирроз печени как проявление неонатального гемохроматозаCongenital liver cirrhosis as a manifestation of neonatal hemochromatosishttps://orcid.org/0000-0003-2500-4297КривицкаяЛ. В.KrivitskayaL. V.

Кривицкая Людмила Васильевна, к.м.н., доцент, доцент кафедры педиатрии с курсом ФПКиП 

г. Гомель 

Lyudmila V. Krivitskaya, Candidate of Medical Sciences, Associate Professor, Associate Professor at the Department of Pediatrics with a course of Advanced Training and Retraining 

Gomel 

pediatrics@gsmu.byГомельский государственный медицинский университетБеларусьGomel State Medical UniversityBelarus202616042026231134140Copyright © Кривицкая Л.В., 20262026Кривицкая Л.В.Krivitskaya L.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.gsmu.by/jour/article/view/2931

Неонатальный гемохроматоз (НГ) является одним из фенотипических проявлений гестационного аллоиммунного повреждения печени (ГАПП).

При этой патологии вследствие фетоматеринской изоиммунизации происходит выработка антител IgG против гепатоцитов плода. Вследствие взаимодействия антигенов печени плода и антител матери развивается повреждение гепатоцитов и их апоптоз.

Клинические симптомы ГАПП разнообразны и чаще всего проявляются в виде неонатального гемохроматоза. Отмечается клинико-лабораторная картина острой печеночной недостаточности с явлениями геморрагического синдрома вследствие коагулопатии, гипогликемии, гипоальбуминемии с развитием отечного синдрома. Открытие аллоиммунного характера этой патологии позволило разработать более эффективный метод лечения с использованием заменного переливания крови и внутривенного введения IgG в дозе 1 г/кг, что позволило увеличить выживаемость новорожденных до 75 %. В связи с этим важным является осведомленность врачей акушеров-гинекологов и неонатологов о механизмах развития данной патологии, клинической картине и возможных методах лечения.

Neonatal hemochromatosis (NH) is one of the phenotypic manifestations of gestational alloimmune liver disease (GALD). In this pathology, due to fetomaternal isoimmunization, the production of IgG antibodies against fetal hepatocytes occurs. As a result of the interaction between fetal liver antigens and mothers antibodies, hepatocyte damage and apoptosis develop. The clinical symptoms of GALD are diverse and most often manifest in a form of neonatal hemochromatosis. There is a clinical and laboratory picture of acute liver failure with hemorrhagic syndrome due to coagulopathy, hypoglycemia, hypoalbuminemia with the development of edema syndrome. The discovery of the alloimmune nature of this pathology has made it possible to develop a more effective treatment method using exchange blood transfusion and intravenous administration of immunoglobulin at a dose of 1g/kg, which has increased the survival rate of newborns to 75%. In this regard, it is important for obstetricians, gynecologists and neonatologists to be aware of the mechanisms of this pathology, its clinical picture, and possible treatment methods.

неонатальный гемохроматозгестационное аллоиммунное повреждение печениострая печеночная недостаточностьноворожденныеneonatal hemochromatosisgestational alloimmune liver damageacute liver failurenewbornsReferencesWhitington PF. Neonatal hemochromatosis: a congenital alloimmune hepatitis. Semin Liver Dis. 2007;27(3):243-250. DOI: https://doi.org/10.1055/s-2007-985069Whitington PF. Neonatal hemochromatosis: a congenital alloimmune hepatitis. Semin Liver Dis. 2007;27(3):243-250. DOI: https://doi.org/10.1055/s-2007-985069Whitington PF. Gestational alloimmune liver disease and neonatal hemochromatosis. Semin Liver Dis. 2012;32(4):325- 332. DOI: https://doi.org/10.1055/s-0032-1329901Whitington PF. Gestational alloimmune liver disease and neonatal hemochromatosis. Semin Liver Dis. 2012;32(4):325- 332. DOI: https://doi.org/10.1055/s-0032-1329901Smyk D, Grammatikopoulos T, Daponte A. Rigopoulou EI, Bogdanos DP. Fetomaternal alloimmunity as a cause of liver disease. Auto Immun Highlights. 2011;23;2(l):21-28. DOI: https://doi.org/10.1007/s13317-011-0019-7Smyk D, Grammatikopoulos T, Daponte A. Rigopoulou EI, Bogdanos DP. Fetomaternal alloimmunity as a cause of liver disease. Auto Immun Highlights. 2011;23;2(l):21-28. DOI: https://doi.org/10.1007/s13317-011-0019-7Garcia Victori E, Manas R, Castilla Fernandez Y. Félix Castillo Salinas. Hemocromatosis neonatal. Diez anos en un cambio De paradigma [Neonatal haemochromatosis: 10 years into a paradigm shift]. An Pediatr (Engl Ed). 2019;91(2):124-126. Spanish. DOI: https://doi.org/10.1016/j.anpedi.2018.06.016Garcia Victori E, Manas R, Castilla Fernandez Y. Félix Castillo Salinas. Hemocromatosis neonatal. Diez anos en un cambio De paradigma [Neonatal haemochromatosis: 10 years into a paradigm shift]. An Pediatr (Engl Ed). 2019;91(2):124-126. Spanish. DOI: https://doi.org/10.1016/j.anpedi.2018.06.016Bonilla S, Prozialeck JD, Malladi P,Pan X, Yu S, et al. Neonatal iron overload and tissue siderosis due to gestational alloimmune liver disease. Hepatol. 2012;56(6):1351-1355. DOI: https://doi.org/10.1016/j.jhep.2012.01.010Bonilla S, Prozialeck JD, Malladi P,Pan X, Yu S, et al. Neonatal iron overload and tissue siderosis due to gestational alloimmune liver disease. Hepatol. 2012;56(6):1351-1355. DOI: https://doi.org/10.1016/j.jhep.2012.01.010Feldman AG., Whitington PF. Neonatal hemochromatosis. / Clin Exp Hepatol. 2013;3(4):313-320. DOI: https://doi.org/10.1016/j.jceh.2013.10.004Feldman AG., Whitington PF. Neonatal hemochromatosis. / Clin Exp Hepatol. 2013;3(4):313-320. DOI: https://doi.org/10.1016/j.jceh.2013.10.004Lopriore E, Mearin ML, Oepkes D, Devlieger R, Whitington PF. Neonatal hemochromatosis: management, outcome, and prevention. Prenat Diagn. 2013;33(13):1221-1225. DOI: https://doi.org/10.1002/pd.4232Lopriore E, Mearin ML, Oepkes D, Devlieger R, Whitington PF. Neonatal hemochromatosis: management, outcome, and prevention. Prenat Diagn. 2013;33(13):1221-1225. DOI: https://doi.org/10.1002/pd.4232Ciocca M, Álvarez F. Neonatal acute liver failure: a diagnosis challenge. Arch Argent Pediatr. 2017 Apr 1;115(2):175- 180. English, Spanish. DOI: https://doi.org/10.5546/aap.2017. eng.175Ciocca M, Álvarez F. Neonatal acute liver failure: a diagnosis challenge. Arch Argent Pediatr. 2017 Apr 1;115(2):175- 180. English, Spanish. DOI: https://doi.org/10.5546/aap.2017. eng.175Debray FG, de Halleux V, Guidi O, Detrembleur N, Gaillez S, et al. Neonatal liver cirrhosis without iron overload caused by gestational alloimmune liver disease. Pediatrics. 2012 Apr;129(4):e1076-e1079. doi: https://doi.org/10.1542/peds.2011-0568Debray FG, de Halleux V, Guidi O, Detrembleur N, Gaillez S, et al. Neonatal liver cirrhosis without iron overload caused by gestational alloimmune liver disease. Pediatrics. 2012 Apr;129(4):e1076-e1079. doi: https://doi.org/10.1542/peds.2011-0568Whitington PF. Gestational alloimmune liver disease and neonatal hemochromatosis. Semin Liver Dis. 2012;32(4):325- 332. DOI: https://doi.org/10.1055/s-0032-1329901Whitington PF. Gestational alloimmune liver disease and neonatal hemochromatosis. Semin Liver Dis. 2012;32(4):325- 332. DOI: https://doi.org/10.1055/s-0032-1329901Tауlог SA, Whitington PF. Neonatal acute liver failure. Liver Transp 2016;22(5):677-685. DOI: https://doi.org/10.1002/lt.24433Tауlог SA, Whitington PF. Neonatal acute liver failure. Liver Transp 2016;22(5):677-685. DOI: https://doi.org/10.1002/lt.24433Rand EB, Karpen SJ, Kelly S, Mack CL, Malatack JJ, et al. Treatment of neonatal hemochromatosis with exchange transfusion and intravenous immunoglobulin. J Pediatr. 2009 Oct;155(4):566-571. DOI: https://doi.org/10.1016/j.jpeds.2009.04.012Rand EB, Karpen SJ, Kelly S, Mack CL, Malatack JJ, et al. Treatment of neonatal hemochromatosis with exchange transfusion and intravenous immunoglobulin. J Pediatr. 2009 Oct;155(4):566-571. DOI: https://doi.org/10.1016/j.jpeds.2009.04.012Mulzer LM, Reutter H, Jüngert J, Knisely AS, Schmid M, et al. Premature birth associated with a favorable course in gestational alloimmune liver disease (GALD): A case report. Front Pediatr. 2023 Mar 16;11:1104530. DOI: https://doi.org/10.3389/fped.2023.1104530Mulzer LM, Reutter H, Jüngert J, Knisely AS, Schmid M, et al. Premature birth associated with a favorable course in gestational alloimmune liver disease (GALD): A case report. Front Pediatr. 2023 Mar 16;11:1104530. DOI: https://doi.org/10.3389/fped.2023.1104530Whitington PF. Fetal and infantile hemochromatosis. Hepatology. 2006 Apr;43(4):654-660. DOI: https://doi.org/10.1002/hep.21129Whitington PF. Fetal and infantile hemochromatosis. Hepatology. 2006 Apr;43(4):654-660. DOI: https://doi.org/10.1002/hep.21129Bem EV, Chumakova GN, Panchenko AS, Myznikova IV, Tretyakova KD, et al. Neonatal Hemochromatosis as One of the Manifestations of Gestational Alloimmune liver disease in fetomaternal Isoimmunization. RMJ. Mother and Child. 2025;8(2):149- 155. DOI: https://doi.org/10.32364/2618-8430-2025-8-2-9Bem EV, Chumakova GN, Panchenko AS, Myznikova IV, Tretyakova KD, et al. Neonatal Hemochromatosis as One of the Manifestations of Gestational Alloimmune liver disease in fetomaternal Isoimmunization. RMJ. Mother and Child. 2025;8(2):149- 155. DOI: https://doi.org/10.32364/2618-8430-2025-8-2-9

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