Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous Immunoglobulin

Authors

Ehab Mohamed Hantash^{1, *,} , Abdulrahman Al Zahrani¹, Mohamed Abdoun^1, , Syeda Naqvi¹, Georges E. Nawfal^2,

¹Neonatal Intensive Care Unit, Arryan Hospital, Dr. Suliman Al Habib Medical Group, Khurais Road, P.O. Box 100266, Riyadh 11635, Saudi Arabia

²Department of Pediatric Gastroenterology, Arryan Hospital, Dr. Suliman Al Habib Medical Group, Riyadh, Saudi Arabia

^*Corresponding author. Email: ehab_hantash@yahoo.com

DOI

10.2991/dsahmj.k.210715.001How to use a DOI?

Keywords

Hemochromatosis; liver failure; gestational alloimmune liver disease; immunoglobulin; exchange transfusion

Abstract

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.

Copyright

© 2021 Dr. Sulaiman Al Habib Medical Group. Publishing services by Atlantis Press International B.V.

Open Access

This is an open access article distributed under the CC BY-NC 4.0 license (http://creativecommons.org/licenses/by-nc/4.0/).

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TY  - JOUR
AU  - Ehab Mohamed Hantash
AU  - Abdulrahman Al Zahrani
AU  - Mohamed Abdoun
AU  - Syeda Naqvi
AU  - Georges E. Nawfal
PY  - 2021
DA  - 2021/07/27
TI  - Neonatal Hemochromatosis: Treatment with Exchange Transfusion and Intravenous Immunoglobulin
JO  - Dr. Sulaiman Al Habib Medical Journal
SP  - 99
EP  - 103
VL  - 3
IS  - 3
SN  - 2590-3349
UR  - https://doi.org/10.2991/dsahmj.k.210715.001
DO  - 10.2991/dsahmj.k.210715.001
ID  - Hantash2021
ER  -