Neonatal hemochromatosis: Radiographical and histological signs

Neonatal hemochromatosis.

Minor salivary gland biopsy in neonatal hemochromatosis.

BACKGROUND Neonatal hemochromatosis (NH), a rare disorder seen in newborns, is defined as liver failure with extrahepatic iron deposition that spares the reticuloendothelial elements. This disorder is considered the pathologic end point of a variety of diseases that result in prenatal liver failure, and mortality without aggressive treatment is common. However, ready diagnosis remains a problem...

Neonatal hemochromatosis--report of an autopsy case.

A case of neonatal hemochromatosis in a 3-hour-old male is described. He presented with hypotonia, mild jaundice, and respiratory difficulty immediately after birth. He had no evidence of congenital infection, immune-related hemolysis or exogenous iron uptake. Postmortem examination revealed abnormal facial features. The organs were of normal weight for his age except a small liver and lungs, a...

Neonatal hemochromatosis: a congenital alloimmune hepatitis.

Neonatal hemochromatosis (NH) is a rare and enigmatic disease that has been clinically defined as severe neonatal liver disease in association with extrahepatic siderosis. It recurs at an alarming rate in the offspring of certain women; the rate and pattern of recurrence led us to hypothesize that maternal alloimmunity is the likely cause at least of recurrent cases. This hypothesis led to a tr...

Neonatal hemochromatosis. Case series from Bahrain.

OBJECTIVE To review clinical presentations, diagnosis, response to treatment, and outcome of infants with neonatal hemochromatosis (NH). METHODS This is a retrospective review of all cases admitted to the Pediatric Department at Salmaniya Medical Center, Manama, Bahrain between March 2008 and May 2011. The diagnosis was based on serum iron and ferritin, alpha-fetoprotein levels (AFP), liver a...