A 3-year-old girl is diagnosed with thalassemia major. The long-term complications seen in a child with thalassemia major are related to which of the following?


•Thalassemia major is a genetic disorder that causes a decrease in hemoglobin formation. Thalassemia causes an abnormal production of a protein responsible for iron absorption, leading to lethal iron overload.

• Thalassemia major or Cooley's anemia is a disorder caused by defects in both beta-chains of the hemoglobin molecule, result in a severe microcytic anemia. These patients develop facial deformities from expansion of the marrow of the facial bones, including maxillary hyperplasia and frontal bossing. Thalassemia is found most often in Black, Mediterranean, and Southeast Asian ethnic groups. Management requires regular blood transfusions to maintain HGB levels at 9-10 g/dL. Chelation therapy with Deferoxamine (Desferal) and monitoring for signs of chelator-related toxicity is required.  

•Thalassemia major in children can cause symptoms of anemia, such as pallor, fatigue, weakness, irritability, and dyspnea.

•Incorrect: Bleeding, increased reflexes, and cerebral edema are not associated with thalassemia.

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