Atransferrinemia

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Atransferrinemia

Atransferrinemia (pronounced as a-trans-fer-ri-ne-mia) is a rare, autosomal recessive disorder characterized by an absence of transferrin, a plasma protein that transports iron throughout the body.

Etymology

The term "Atransferrinemia" is derived from the Greek prefix 'a-' meaning 'without', 'trans-' meaning 'across', 'ferrum' meaning 'iron', and '-emia' meaning 'in the blood'. Thus, it literally means 'without transferrin in the blood'.

Symptoms

The main symptoms of Atransferrinemia include anemia, iron overload, and recurrent infections due to impaired immune system function.

Diagnosis

Diagnosis of Atransferrinemia is typically made through blood tests showing very low or absent transferrin levels, high serum iron levels, and other signs of iron overload. Genetic testing can confirm the diagnosis.

Treatment

Treatment for Atransferrinemia is primarily supportive and includes iron chelation therapy to reduce iron overload, and blood transfusions to treat anemia.

Prognosis

The prognosis for individuals with Atransferrinemia varies. With early diagnosis and appropriate treatment, many individuals can lead normal lives. However, complications such as heart disease and liver damage due to iron overload can affect life expectancy.

See also

External links

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