Neonatal hemochromatosis

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Neonatal Hemochromatosis

Neonatal hemochromatosis (pronunciation: nee-oh-nay-tal hee-moh-kroh-muh-toh-sis) is a rare and severe liver disease that affects newborns. It is characterized by the excessive accumulation of iron in the liver and other organs, leading to liver failure.

Etymology

The term "neonatal hemochromatosis" is derived from three words: "neonatal", from the Latin neonatus meaning "newly born"; "hemo", from the Greek haima meaning "blood"; and "chromatosis", from the Greek chroma meaning "color". This refers to the characteristic rusty color of the organs affected by the disease due to iron overload.

Symptoms

The symptoms of neonatal hemochromatosis may include jaundice, low birth weight, hepatomegaly (enlarged liver), and coagulopathy (blood clotting disorder). In severe cases, it can lead to liver failure and death.

Causes

The exact cause of neonatal hemochromatosis is unknown. However, it is believed to be an alloimmune disease, where the mother's immune system attacks the liver of the fetus. It is not considered a hereditary form of hemochromatosis.

Diagnosis

Diagnosis of neonatal hemochromatosis is often made based on clinical symptoms and confirmed by liver biopsy, which shows excessive iron accumulation in the liver.

Treatment

Treatment for neonatal hemochromatosis includes supportive care, medications to remove excess iron from the body, and in severe cases, liver transplantation.

Prognosis

The prognosis for neonatal hemochromatosis has improved significantly in recent years with the advent of new treatments. However, it remains a serious condition with a high mortality rate.

See also

External links

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