title: Pathology teach and tell: neonatal hemochromatosis with massive hepatic necrosis.
abstract: Neonatal hemochromatosis (NH) is an uncommon disorder clinicopathologically defined by severe liver disease of intrauterine onset associated with extrahepatic siderosis that spares reticuloendothelial elements. NH phenotypically is a similar disorder to hereditary hemochromatosis. However, its extremely early onset of liver failure makes it notably unique. Massive liver necrosis in the newborn is a rare occurrence, but whenever present hemochromatosis should be considered in the differential diagnosis. Herein, we report a case of neonatal hemochromatosis that had massive hepatic necrosis with sparing only little parenchyma. The outcome was fatal within the first month of life.
authors.affiliation: Department of Pathology and Paediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Richa
authors.initials: R
authors.lastname: Goyal
authors.forename: Sanjib Kumar
authors.initials: SK
authors.lastname: Pattari
authors.forename: Nandita
authors.initials: N
authors.lastname: Kakkar
authors.forename: B D
authors.initials: BD
authors.lastname: Radotra
authors.forename: Anil
authors.initials: A
authors.lastname: Narang
mesh_terms: D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006432:Hemochromatosis / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases; D007501:Iron / Q000378:metabolism; D008099:Liver / Q000473:pathology*; D008107:Liver Diseases; D008297:Male; D009336:Necrosis / Q000473:pathology*; D010179:Pancreas / Q000166:cytology; D010641:Phenotype
chemical_list: D007501:Iron