Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment
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 transfu...
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| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2014-01-01
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| Series: | Case Reports in Critical Care |
| Online Access: | http://dx.doi.org/10.1155/2014/650916 |
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| _version_ | 1850175721939402752 |
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| author | Ayelet Machtei Gil Klinger Rivka Shapiro Osnat Konen Lea Sirota |
| author_facet | Ayelet Machtei Gil Klinger Rivka Shapiro Osnat Konen Lea Sirota |
| author_sort | Ayelet Machtei |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-c07fea2797b64705a99e9501f2f6e382 |
| institution | OA Journals |
| issn | 2090-6420 2090-6439 |
| language | English |
| publishDate | 2014-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Critical Care |
| spelling | doaj-art-c07fea2797b64705a99e9501f2f6e3822025-08-20T02:19:23ZengWileyCase Reports in Critical Care2090-64202090-64392014-01-01201410.1155/2014/650916650916Clinical and Imaging Resolution of Neonatal Hemochromatosis following TreatmentAyelet Machtei0Gil Klinger1Rivka Shapiro2Osnat Konen3Lea Sirota4Neonatal Intensive Care Unit, Schneider Children’s Medical Center of Israel, 14 Kaplan Street, 49202 Petah Tikva, IsraelNeonatal Intensive Care Unit, Schneider Children’s Medical Center of Israel, 14 Kaplan Street, 49202 Petah Tikva, IsraelThe Sackler School of Medicine, Tel Aviv University, Tel Aviv, IsraelThe Sackler School of Medicine, Tel Aviv University, Tel Aviv, IsraelNeonatal Intensive Care Unit, Schneider Children’s Medical Center of Israel, 14 Kaplan Street, 49202 Petah Tikva, IsraelNeonatal 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.http://dx.doi.org/10.1155/2014/650916 |
| spellingShingle | Ayelet Machtei Gil Klinger Rivka Shapiro Osnat Konen Lea Sirota Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment Case Reports in Critical Care |
| title | Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment |
| title_full | Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment |
| title_fullStr | Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment |
| title_full_unstemmed | Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment |
| title_short | Clinical and Imaging Resolution of Neonatal Hemochromatosis following Treatment |
| title_sort | clinical and imaging resolution of neonatal hemochromatosis following treatment |
| url | http://dx.doi.org/10.1155/2014/650916 |
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