Neonatal hemochromatosis as one of the manifestations of gestational alloimmune liver disease in fetomaternal isoimmunization
Year: 2025
Type: article
Abstract: Fetomaternal isoimmunization (alloimmunization) predominantly results in the development of hematological disorders in the neonate, such as hemolytic disease of the Rh blood group and ABO system, as well as alloimmune thrombocytopenia and neutropenia. These pathologies are mediated by maternal IgG antibodies directed against paternally inherited fetal antigens. Gestational alloimmune liver disease (GALD), in contrast, represents a distinct form of fetomaternal isoimmunization, targeting fetal hepatocytes independently of paternal antigenic inheritance In GALD, the transplacental passage of specific maternal antibodies, typically after 18 weeks of gestation, leads to immune- mediated injury of fetal hepatocytes. The most prevalent clinical manifestation of GALD is neonatal hemochromatosis, characterized by acute hepatic failure in the newborn and extrahepatic siderosis. GALD generally presents with nonspecific clinical features, which may include hypoglycemia, severe coagulopathy commonly manifesting as bleeding from various anatomical sites, generalized edema with or without ascites, and oliguria. Recent advances in the understanding of GALD's alloimmune etiology have fundamentally altered therapeutic approaches The use of intravenous immunoglobulin and exchange transfusion has significantly improved survival outcomes, increasing the survival rate without liver transplantation from approximately 20% to 75%. It is imperative to enhance physician awareness across disciplines regarding the alloimmune pathogenesis, diverse clinical presentations, and current treatment tactics for GALD, as well as prophylactic treatment protocols for pregnant women sensitized to fetal hepatocyte antigens. KEYWORDS: neonatal hemochromatosis, gestational alloimmune liver disease, congenital alloimmune hepatitis, fetomaternal isoimmunization, alloimmunization, neonatal acute liver failure, newborns. FOR CITATION: Bem E.V., Chumakova G.N., Panchenko A.S., Myznikova I.V., Tretyakova K.D., Fedorova L.A., Pavlova S.E. Neonatal hemochromatosis as one of the manifestations of gestational alloimmune liver disease in fetomaternal isoimmunization. Russian Journal of Woman and Child Health. 2025;8(2):149–155 (in Russ.). DOI: 10.32364/2618-8430-2025-8-2-9
Language: English
Topic: Blood groups and transfusion
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4411653175