Sickle Cell Disease

Which of the following is the primary mechanism of hemolysis in sickle cell disease?

What is the molecular basis of sickle cell disease?

A 3-year-old child with known sickle cell disease presents with sudden onset of severe bone pain, fever, and elevated reticulocyte count. The clinical team suspects acute vaso-occlusive crisis with possible osteomyelitis. Which investigation would be most appropriate to differentiate between bone infarction and osteomyelitis?

A 6-year-old boy of African descent presents with acute chest pain, fever, and shortness of breath. Clinical examination reveals hepatosplenomegaly and bone tenderness. A peripheral blood smear shows sickle-shaped RBCs. Which investigation is most appropriate to confirm the diagnosis of sickle cell disease?

A 32-year-old man with sickle cell disease presents with acute chest syndrome (fever, chest pain, pulmonary infiltrate). Which agent is the first-line treatment to reduce mortality and organ damage in this life-threatening complication?