Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous Immunoglobulin
Neonatal Hemochromatosis (NH) is a rare phenotype of severe fetal/neonatal liver injury that is accompanied by extrahepatic siderosis. Current clinical evidence shows that NH is not a disease per se, but is the consequence of fetal liver injury. Gestational alloimmune liver disease is the cause of n...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2021-07-01
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| Series: | Dr. Sulaiman Al Habib Medical Journal |
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| Online Access: | https://journals.lww.com/10.2991/dsahmj.k.210715.001 |
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| author | Ehab Mohamed Hantash Abdulrahman Al Zahrani Mohamed Abdoun Syeda Naqvi Georges E. Nawfal |
| author_facet | Ehab Mohamed Hantash Abdulrahman Al Zahrani Mohamed Abdoun Syeda Naqvi Georges E. Nawfal |
| author_sort | Ehab Mohamed Hantash |
| collection | DOAJ |
| description | Neonatal Hemochromatosis (NH) is a rare phenotype of severe fetal/neonatal liver injury that is accompanied by extrahepatic siderosis. Current clinical evidence shows that NH is not a disease per se, but is the consequence of fetal liver injury. Gestational alloimmune liver disease is the cause of nearly all cases of NH. Affected babies may die in utero, or present postnatally with severe acute liver failure or decompensated congenital liver cirrhosis. Diagnosis depends on the demonstration of extrahepatic siderosis by Magnetic Resonance Imaging (MRI), buccal biopsy, or detection of complement C5b-9 complex on hepatocytes from liver biopsy. Prognosis is generally bad without treatment. Treatment with iron chelators and antioxidants is not helpful. The more recent treatment approach of exchange transfusion and Intravenous Immunoglobulin (IVIG) has shown favorable outcomes. In this report, we describe a case of NH that presented with liver cell failure and high serum ferritin. Diagnosis was confirmed by MRI by demonstration of siderosis in the liver and pancreas while sparing the spleen. The infant was successfully treated with a combination of exchange transfusion and IVIG, and discharged at age 30 days in good condition. |
| format | Article |
| id | doaj-art-b079bca0dbaf436b8531cf9a80c9a1ab |
| institution | Kabale University |
| issn | 2666-819X 2590-3349 |
| language | English |
| publishDate | 2021-07-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Dr. Sulaiman Al Habib Medical Journal |
| spelling | doaj-art-b079bca0dbaf436b8531cf9a80c9a1ab2025-08-20T03:36:45ZengWolters Kluwer Medknow PublicationsDr. Sulaiman Al Habib Medical Journal2666-819X2590-33492021-07-01339910310.2991/dsahmj.k.210715.001Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous ImmunoglobulinEhab Mohamed HantashAbdulrahman Al ZahraniMohamed AbdounSyeda NaqviGeorges E. NawfalNeonatal Hemochromatosis (NH) is a rare phenotype of severe fetal/neonatal liver injury that is accompanied by extrahepatic siderosis. Current clinical evidence shows that NH is not a disease per se, but is the consequence of fetal liver injury. Gestational alloimmune liver disease is the cause of nearly all cases of NH. Affected babies may die in utero, or present postnatally with severe acute liver failure or decompensated congenital liver cirrhosis. Diagnosis depends on the demonstration of extrahepatic siderosis by Magnetic Resonance Imaging (MRI), buccal biopsy, or detection of complement C5b-9 complex on hepatocytes from liver biopsy. Prognosis is generally bad without treatment. Treatment with iron chelators and antioxidants is not helpful. The more recent treatment approach of exchange transfusion and Intravenous Immunoglobulin (IVIG) has shown favorable outcomes. In this report, we describe a case of NH that presented with liver cell failure and high serum ferritin. Diagnosis was confirmed by MRI by demonstration of siderosis in the liver and pancreas while sparing the spleen. The infant was successfully treated with a combination of exchange transfusion and IVIG, and discharged at age 30 days in good condition.https://journals.lww.com/10.2991/dsahmj.k.210715.001hemochromatosisliver failuregestational alloimmune liver diseaseimmunoglobulinexchange transfusion |
| spellingShingle | Ehab Mohamed Hantash Abdulrahman Al Zahrani Mohamed Abdoun Syeda Naqvi Georges E. Nawfal Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous Immunoglobulin Dr. Sulaiman Al Habib Medical Journal hemochromatosis liver failure gestational alloimmune liver disease immunoglobulin exchange transfusion |
| title | Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous Immunoglobulin |
| title_full | Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous Immunoglobulin |
| title_fullStr | Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous Immunoglobulin |
| title_full_unstemmed | Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous Immunoglobulin |
| title_short | Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous Immunoglobulin |
| title_sort | neonatal hemochromatosis treatment with exchange transfusion and intravenous immunoglobulin |
| topic | hemochromatosis liver failure gestational alloimmune liver disease immunoglobulin exchange transfusion |
| url | https://journals.lww.com/10.2991/dsahmj.k.210715.001 |
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