Neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report

Neonatal hemochromatosis (NH) is a rare fatal liver disease accompanied by hepatic and extrahepatic iron overload.1-3 Gestational alloimmune liver disease (GALD) is a materno-fetal alloimmune disorder and leading cause of NH.2,4,5 This condition allows an interplay between the maternal adaptive immu...

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Autores principales:	Adhi Teguh Perma Iskandar, Vini Jamarin, Kamajaya Mulyana
Formato:	article
Lenguaje:	EN
Publicado:	Indonesian Pediatric Society Publishing House 2021
Materias:	neonatal hemochromatosis gestational alloimmune liver disease intravenous immunoglobulin exchange transfusion Medicine R Pediatrics RJ1-570
Acceso en línea:	https://doaj.org/article/d5a05cb01b844afda580d74c7514dc47
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spelling	oai:doaj.org-article:d5a05cb01b844afda580d74c7514dc472021-11-29T02:29:22ZNeonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report0030-93112338-476X10.14238/pi61.6.2021.350-5https://doaj.org/article/d5a05cb01b844afda580d74c7514dc472021-11-01T00:00:00Zhttps://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/2383https://doaj.org/toc/0030-9311https://doaj.org/toc/2338-476XNeonatal hemochromatosis (NH) is a rare fatal liver disease accompanied by hepatic and extrahepatic iron overload.1-3 Gestational alloimmune liver disease (GALD) is a materno-fetal alloimmune disorder and leading cause of NH.2,4,5 This condition allows an interplay between the maternal adaptive immune system and the fetus, resulting in an allograft to the mother. The mother becomes sensitized to an alloantigen expressed by the fetus and forms specific reactive antibodies. Immunoglobulin G (IgG) is transported through the placenta and attacks the fetal hepatocytes, resulting in severe loss of hepatocytes and fetal iron overload.3,6 Liver transplantation has been the only definitive treatment for NH for many years, with a survival rate of ±35%. Conventional therapy containing antioxidants and chelation agents reportedly have very poor success, with survival rate of only 10-20%. A new treatment paradigm involving intravenous immunoglobulin (IVIG) and exchange transfusion (ET) therapy has shown significant success in survival rate in NH, decreasing the need for liver transplantation.3,7,8 Here we present a case of NH caused by GALD and treated successfully with a combination of IVIG therapy and ET. We also aimed to evaluate the efficacy of IVIG and ET therapy for NH.Adhi Teguh Perma IskandarVini JamarinKamajaya MulyanaIndonesian Pediatric Society Publishing Housearticleneonatal hemochromatosisgestational alloimmune liver diseaseintravenous immunoglobulinexchange transfusionMedicineRPediatricsRJ1-570ENPaediatrica Indonesiana, Vol 61, Iss 6, Pp 350-5 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	neonatal hemochromatosis gestational alloimmune liver disease intravenous immunoglobulin exchange transfusion Medicine R Pediatrics RJ1-570
spellingShingle	neonatal hemochromatosis gestational alloimmune liver disease intravenous immunoglobulin exchange transfusion Medicine R Pediatrics RJ1-570 Adhi Teguh Perma Iskandar Vini Jamarin Kamajaya Mulyana Neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report
description	Neonatal hemochromatosis (NH) is a rare fatal liver disease accompanied by hepatic and extrahepatic iron overload.1-3 Gestational alloimmune liver disease (GALD) is a materno-fetal alloimmune disorder and leading cause of NH.2,4,5 This condition allows an interplay between the maternal adaptive immune system and the fetus, resulting in an allograft to the mother. The mother becomes sensitized to an alloantigen expressed by the fetus and forms specific reactive antibodies. Immunoglobulin G (IgG) is transported through the placenta and attacks the fetal hepatocytes, resulting in severe loss of hepatocytes and fetal iron overload.3,6 Liver transplantation has been the only definitive treatment for NH for many years, with a survival rate of ±35%. Conventional therapy containing antioxidants and chelation agents reportedly have very poor success, with survival rate of only 10-20%. A new treatment paradigm involving intravenous immunoglobulin (IVIG) and exchange transfusion (ET) therapy has shown significant success in survival rate in NH, decreasing the need for liver transplantation.3,7,8 Here we present a case of NH caused by GALD and treated successfully with a combination of IVIG therapy and ET. We also aimed to evaluate the efficacy of IVIG and ET therapy for NH.
format	article
author	Adhi Teguh Perma Iskandar Vini Jamarin Kamajaya Mulyana
author_facet	Adhi Teguh Perma Iskandar Vini Jamarin Kamajaya Mulyana
author_sort	Adhi Teguh Perma Iskandar
title	Neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report
title_short	Neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report
title_full	Neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report
title_fullStr	Neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report
title_full_unstemmed	Neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report
title_sort	neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report
publisher	Indonesian Pediatric Society Publishing House
publishDate	2021
url	https://doaj.org/article/d5a05cb01b844afda580d74c7514dc47
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Neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report

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