title: Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment.
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.
authors: Neonatal Intensive Care Unit, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 49202 Petah Tikva, Israel.
authors: Ayelet
authors: A
authors: Machtei
authors: Neonatal Intensive Care Unit, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 49202 Petah Tikva, Israel ; The Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors: Gil
authors: G
authors: Klinger
authors: The Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel ; Institute of Gastroenterology, Nutrition and Liver Diseases, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.
authors: Rivka
authors: R
authors: Shapiro
authors: The Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel ; Radiology Department, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.
authors: Osnat
authors: O
authors: Konen
authors: Neonatal Intensive Care Unit, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 49202 Petah Tikva, Israel ; The Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors: Lea
authors: L
authors: Sirota