Neonatal hemochromatosis

A hereditary autosomal recessive disorder characterized by LIVER FAILURE in the neonatal period and heavy iron staining in the liver. There are also iron deposits in extrahepatic tissues, including the heart and pancreas. OMIM: 231100

Also Known As:

Networked: 387 relevant articles (11 outcomes, 23 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Biliary Atresia
2.	Cholestasis
3.	Autoimmune Hemolytic Anemia (Cold Agglutinin Disease)
4.	Liver Diseases (Liver Disease)
5.	Neonatal Systemic lupus erythematosus

Experts

1.	Whitington, Peter F: 7 articles (10/2012 - 09/2003)
2.	Lomas, David A: 5 articles (05/2013 - 03/2002)
3.	Knisely, A S: 4 articles (01/2022 - 09/2003)
4.	Mieli-Vergani, Giorgina: 4 articles (11/2007 - 09/2003)
5.	Kobayashi, Keiko: 4 articles (01/2005 - 01/2002)
6.	Saheki, Takeyori: 4 articles (01/2005 - 01/2002)
7.	Maggiore, Giuseppe: 3 articles (01/2022 - 03/2013)
8.	Hata, Toshiyuki: 3 articles (01/2020 - 12/2011)
9.	Tanaka, Hirokazu: 3 articles (01/2020 - 12/2011)
10.	Kasahara, Mureo: 3 articles (11/2016 - 09/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neonatal hemochromatosis:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 06/01/2008 - "Outcome of pregnancies at risk for neonatal hemochromatosis is improved by treatment with high-dose intravenous immunoglobulin." 04/01/2021 - "The Effect of Prenatal and Postnatal Treatment with Intravenous Immunoglobulin on Severity of Neonatal Hemochromatosis: The Tale of Two Brothers (Case Report)." 07/01/2011 - "[Prenatal treatment of neonatal hemochromatosis with maternal administration of intravenous immunoglobulins (about four cases)]." 10/01/2009 - "Treatment of neonatal hemochromatosis with exchange transfusion and intravenous immunoglobulin." 06/01/2008 - "Neonatal hemochromatosis seems to be the result of a gestational alloimmune disease, and occurrence of severe neonatal hemochromatosis in at-risk pregnancies can be significantly reduced by treatment with high-dose intravenous immunoglobulin during gestation."
2.	Methionine (L-Methionine)FDA Link 11/01/1989 - "These results suggested that the cytotoxicity resulted from the immaturity of suckling rat hepatocytes and therefore dietary restriction of galactose, tyrosine and methionine might be a useful treatment for patients with neonatal hepatitis." 09/01/2001 - "Compared with those of age-matched cholestatic disease controls, idiopathic neonatal hepatitis (n=9) and biliary atresia (n=14), plasma levels of three amino acids, citrulline, methionine, and threonine, were significantly greater, respectively (P<0.01). " 02/01/2017 - "Levels of citrulline, threonine, methionine, tyrosine and arginine and the threonine-to-serine ratio were higher in children with neonatal intrahepatic cholestasis caused by NICCD compared with that in patients with idiopathic neonatal hepatitis (INH). "
3.	Immunoglobulins (Immunoglobulin)IBA 01/01/1987 - "Based on these data, the efficacy of using anti-HBs immunoglobulin for prevention of neonatal hepatitis B is discussed." 01/01/2020 - "The Japanese experience and pharmacokinetics of antenatal maternal high-dose immunoglobulin treatment as a prophylaxis for neonatal hemochromatosis in siblings." 10/01/2016 - "Antenatal immunoglobulin for prevention of neonatal hemochromatosis." 09/01/2015 - "Another great success has been the preventive use of immunoglobulin in pregnant women with a past history of neonatal hemochromatosis, thus decreasing the rate of disease recurrence up to 70%. " 06/01/2013 - "Compliance with administration of neonatal hepatitis B immunoglobulin and birth-dose vaccination was high (>90%). "
4.	Phenobarbital (Luminal)FDA Link 03/01/1984 - "Hepatobiliary scintigraphy after phenobarbital pretreatment in patients with neonatal hepatitis demonstrates normal hepatic extraction and delayed tracer excretion into the gastrointestinal tract. " 04/01/1994 - "Administration of phenobarbital for 5 days before serial US examination is very useful in the diagnosis of neonatal hepatitis when differentiation between neonatal hepatitis and biliary atresia is impossible because of inability to identify the gallbladder by US alone." 07/01/1981 - "The results of this study show that administration of phenobarbital in a dose of 5 mg/kg/day for at least 5 days prior to the examination enhances and accelerates biliary excretion of IDA compounds and thereby significantly increases the accuracy of 99mTc-IDA scintigraphy in differentiating extrahepatic biliary atresia from neonatal hepatitis. " 04/01/1991 - "Phenobarbital-augmented cholescintigraphy is used as a means of increasing the accuracy of hepatobiliary scintigraphy in differentiating neonatal hepatitis from biliary atresia. " 01/01/1996 - "Phenobarbital premedication (5 mg/kg per day for a minimum of 5 days in divided doses) is used in infants who are being examined for neonatal jaundice to increase the accuracy of 99mTc-IDA scintigraphy in differentiating extrahepatic biliary atresia from neonatal hepatitis. "
5.	Ursodeoxycholic Acid (Urso)FDA LinkGeneric 10/01/2009 - "The outcome of infants with neonatal hepatitis with intake of ursodeoxycholic acid and those without was: 108 (44.8%) and 179 (70.2%) successful (P = 0.000), 11 (4.6%) and 13 (5.1%) improved (P = 0. " 10/01/2009 - "Files of 496 infants with neonatal hepatitis and 97 with intrahepatic bile duct paucity were included; of them 241 (48.6%) and 52 (46.4%) received 20-40 mg/kg/day ursodeoxycholic acid for 319.2 +/- 506.9 days and 480.3 +/- 583.3 days, respectively. " 10/01/2009 - "Ursodeoxycholic acid in neonatal hepatitis and infantile paucity of intrahepatic bile ducts: review of a historical cohort." 09/01/2002 - "We report here an infant who presented with neonatal hepatitis and who was being treated with ursodeoxycholic acid. " 09/01/2004 - "This study aimed at identifying the role of ursodeoxycholic acid (UDCA), a choleretic agent, in conjunction with hepatobiliary scintigraphy in differentiating extrahepatic biliary atresia from neonatal hepatitis. "
6.	Bile Acids and Salts (Bile Acids)IBA 01/01/2022 - "Bile acid synthesis disorders should be routinely included in the NC/"neonatal hepatitis" work-up. " 11/01/2000 - "Accordingly, immaturity of the bile acid synthetic pathway may render many otherwise normal infants vulnerable to transient "neonatal hepatitis" with GCT in a broad range of cholestatic disorders." 09/01/1997 - "Abnormal bile acid metabolism and neonatal hemochromatosis: a subset with poor prognosis." 04/01/1979 - "Measurements of individual serum bile acids failed to differentiate between the various neonatal hepatitis syndromes. " 09/21/2018 - "Bile acid deficiency caused by genetic defects in AKR1D1 leads to life-threatening neonatal hepatitis and cholestasis. "
7.	AntibodiesIBA 10/01/1994 - "Postinfantile giant cell hepatitis associated with anti-M2 mitochondrial antibodies." 01/01/1969 - "[Feto-maternal allo-immunization with antiplatelet antibodies and familial neonatal hepatitis]." 01/01/1969 - "[Feto-maternal allo-immunization with anti-platelet antibodies and familial neonatal hepatitis]." 12/01/1984 - "The results indicate that 62% of babies with extrahepatic biliary atresia and 52% of infants with idiopathic neonatal hepatitis have reovirus 3 antibodies. " 07/01/2017 - "Anti-smooth muscle antibodies and liver enzymes in differentiation of extrahepatic biliary atresia and idiopathic neonatal hepatitis: Concern in laboratory medicine view."
8.	Ferritins (Ferritin)IBA 12/01/2008 - "Two of these 4 patients had extensively high serum ferritin levels mimicking neonatal hemochromatosis. " 04/01/2003 - "Liver ferritin subunit ratios in neonatal hemochromatosis." 01/01/2000 - "Neonatal hemochromatosis should be suspected in the presence of cholestasis with liver failure of perinatal onset and with high blood level ferritin. " 01/01/2008 - "High serum ferritin concentrations, MRI findings compatible with tissue iron overload and no evidence for infectious disease or inborn errors of metabolism suggested possible neonatal hemochromatosis (NH). " 04/01/2003 - "The authors report the tissue iron, ferritin, and ferritin subunit content of a child who died at 5 weeks of neonatal hemochromatosis. "
9.	SteroidsIBA 10/01/1993 - "Early steroid therapy for the treatment of giant cell hepatitis with autoimmune hemolytic anemia." 11/03/2022 - "Neonatal Lupus presenting with neonatal hemochromatosis-like liver disease that responded to steroids: a case report." 11/03/2022 - "Neonatal lupus liver disease is a rare cause of neonatal cholestasis that can rarely present with neonatal hemochromatosis picture which unlike other causes of neonatal hemochromatosis can be reversed with steroid therapy." 03/01/1991 - "Coombs-positive giant cell hepatitis of infancy: effect of steroids and azathioprine therapy." 02/01/1994 - "From the biochemical point some diseases present alterations of the cholesterol side chain (Zellweger syndrome, pseudo-Zellweger syndrome, infantile Refsum's disease, neonatal adrenoleukodystrophy), other diseases present errors involving the steroid nucleus (familial giant cell hepatitis). "
10.	Ribavirin (Virazole)FDA LinkGeneric 09/01/2000 - "This observation clearly confirms the efficacy of ribavirin for the treatment of giant cell hepatitis, thus providing evidence for its viral origin." 05/01/2003 - "A few sporadic case reports of paramyxo-like virus related, postinfantile giant cell hepatitis have shown that ribavirin was quite effective treatment but further clinical evaluation is needed." 03/06/1993 - "Ribavirin for syncytial giant cell hepatitis." 09/01/2000 - "A case of giant cell hepatitis recurring after liver transplantation and treated with ribavirin." 03/01/1997 - "This observation also suggests that early administration of ribavirin in patients affected by acute syncytia; giant cell hepatitis of unknown origin could avoid liver transplantation."

Therapies and Procedures

1.	Therapeutics 06/01/2008 - "When compared on a per-woman or per-infant basis, the outcome of gestation at risk for neonatal hemochromatosis was improved by gestational therapy. " 06/01/2008 - "This study was designed to determine whether therapy directed at limiting the severity of gestational alloimmunity can reduce the occurrence of severe neonatal hemochromatosis in infants of women at risk. " 07/01/2021 - "Adjuvant therapies were required for patients with giant cell hepatitis and hemophagocytosis. " 05/01/2010 - "Exchange transfusion as a possible therapy for neonatal hemochromatosis." 01/01/2001 - "The patients presented here, whose livers were infected with the TTV and showed a favorable response to gammaglobulin therapy, may represent a subset of idiopathic neonatal hepatitis patients."
2.	Diet Therapy (Therapy, Diet) 11/01/1989 - "The purpose was to study the reasons for the clinical improvement of patients with neonatal hepatitis after dietary restriction of these nutrients. " 11/01/1989 - "These results suggested that the cytotoxicity resulted from the immaturity of suckling rat hepatocytes and therefore dietary restriction of galactose, tyrosine and methionine might be a useful treatment for patients with neonatal hepatitis."
3.	Splenectomy 08/01/1996 - "[Giant cell hepatitis and autoimmune hemolytic anemia: efficacy of splenectomy on hemolysis]."
4.	Liver Transplantation 11/01/2005 - "The aim of our study was to compare the outcome of medical treatment vs. liver transplantation in infants with neonatal hemochromatosis (NH) referred to King's College Hospital from 1990-2002. " 08/01/1994 - "This study examines the clinical and pathologic course of seven patients who developed giant cell hepatitis (GCH) after liver transplantation. " 06/01/2016 - "Living Related Liver Transplantation in an Infant with Neonatal Hemochromatosis." 03/01/2015 - "Liver transplantation for neonatal hemochromatosis: analysis of the UNOS database." 11/01/2011 - "The cumulative incidence of death without liver transplantation was higher in infants with viral ALF (64%) compared with those with neonatal hemochromatosis (16%) or indeterminate ALF (14%) (P = .0007). "
5.	Total Parenteral Nutrition 01/01/2004 - "The biliary tree was completely opacified in 5 infants with the following diagnosis: neonatal hepatitis (2), duplication of the gallbladder (1), choledochocele (1), total parenteral nutrition (TPN) cholestasis (1). " 08/01/1997 - "Among 13 of the 53 patients who had no evidence of biliary excretion and who also did not have extrahepatic biliary atresia, 8 had idiopathic neonatal hepatitis, 4 had interlobular bile duct paucity and 1 had total parenteral nutrition-associated cholestasis. " 11/01/2018 - "Causes that are discussed include biliary atresia, idiopathic neonatal hepatitis, gestational alloimmune liver disease, metabolic and genetic diseases, total parenteral nutrition cholestasis, and congenital infection. " 10/01/2013 - "Other diagnoses included neonatal hepatitis, total parenteral nutrition cholestasis, Alagille syndrome, cystic fibrosis, choledochal cyst, hypothyroidism, alpha-1 antitrypsin deficiency and persistent cholestasis of unknown etiology. " 12/01/2011 - "Among the 18 patients who underwent successful PCC, biliary atresia was excluded in 13, with diagnoses as follows: total parenteral nutrition-associated cholestasis (TPNAC) (n = 6), neonatal hepatitis (n = 4), congenital syphilis (n = 1), neonatal lupus (n = 1), and congenital cytomegalovirus hepatitis (n = 1). "