# Successful IVIG Treatment in Neonatal Hemochromatosis Without Extrahepatic Siderosis: A Case Report

## TL;DR

A newborn with liver failure due to neonatal hemochromatosis recovered after IVIG treatment, even without extrahepatic iron buildup.

## Contribution

Demonstrates successful IVIG treatment in neonatal hemochromatosis without extrahepatic siderosis.

## Key findings

- IVIG therapy improved liver function in a neonate with hemochromatosis.
- Diagnosis can be made clinically and via MRI when biopsy is not feasible.
- Early treatment is critical for effective management of neonatal hemochromatosis.

## Abstract

Background and Clinical Significance: Neonatal hemochromatosis is a rare iron overload disorder that causes severe liver injury in newborns, typically with extrahepatic siderosis. Diagnosis of neonatal hemochromatosis is usually confirmed through a biopsy and MRI, demonstrating deposition of iron and liver failure. However, in severe patients who are not able to undergo biopsy, the diagnostic and management method remains unknown. Case Presentation: We present an unusual case of neonatal hemochromatosis without extrahepatic iron deposition in a 9-day-old male who showed signs of liver failure and respiratory distress. This case suggests that when the risks of biopsy outweigh its benefits, a diagnosis may be reached based on clinical evaluation and MRI findings. Early high-dose intravenous immunoglobulin therapy improved liver function and led to recovery, highlighting the need for early therapeutic intervention in neonatal hemochromatosis. Conclusions: This case highlights that the absence of extrahepatic siderosis cannot exclude a diagnosis of neonatal hemochromatosis, and high doses of IVIG should be administered promptly when neonatal hemochromatosis is suspected to maximize therapeutic effectiveness.