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Case Reports in Critical Care
Volume 2014 (2014), Article ID 650916, 4 pages
http://dx.doi.org/10.1155/2014/650916
Case Report

Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment

1Neonatal Intensive Care Unit, Schneider Children’s Medical Center of Israel, 14 Kaplan Street, 49202 Petah Tikva, Israel
2The Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
3Institute of Gastroenterology, Nutrition and Liver Diseases, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel
4Radiology Department, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel

Received 14 January 2014; Revised 31 May 2014; Accepted 7 June 2014; Published 24 June 2014

Academic Editor: Ricardo J. Dinis-Oliveira

Copyright © 2014 Ayelet Machtei et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Neonatal hemochromatosis (NH) is an acute liver disease associated with both hepatic and extrahepatic iron deposition and is a leading cause of neonatal liver transplantation. The concept that NH is an alloimmune disease has led to the emergence of a new treatment approach utilizing exchange transfusion and intravenous immunoglobulin therapy. We present a two-day old neonate with progressive liver dysfunction who was diagnosed with NH. Magnetic resonance imaging confirmed tissue iron overload. Treatment with intravenous immunoglobulins and exchange transfusion led to rapid improvement in liver function. Follow-up physical examination at the age of 8 months showed normal development and near normal liver function. A repeat abdominal magnetic resonance scan at 8 months showed no signs of iron deposition in the liver, pancreas, or adrenal glands. The present report provides further support for the use of exchange transfusion and immunoglobulin therapy in NH and is the first to document resolution of typical iron deposition by magnetic resonance imaging.