This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Pediatrics
[{'content': 'In beta thalassemia, there is – \nSelect and explain. \nA. Increase in beta chain, decrease in alpha chain, B. Decrease in beta chain, increase in alpha chain, C. Decrease in beta chain, decrease in alpha chain, D. Increase in beta chain, increase in alpha chain', 'role': 'user'}, {'content': 'Answer: B \nExplanation: Beta - thalassemia \n\nThe abnormality common to all P-thalassemias is diminished synthesis of structurally normal β-globin chains, coupled with unimpaired synthesis of α-chain.\nThe reduced supply of (β-globin chain diminishes production of hemoglobin tetramers, causing hypochromic and microcytic anemia.\nThe haematological consequences of diminished synthesis of one globin chain stem not only from low intracellular hemoglobin, but also from a relative excess of the unimpaired chain.\n\nConsequences of thalassemia\n\nIn (β-thalassemia, anemia is produced by two mechanisms\n\n\nDeficit in HbA synthesis produce hypochromic microcytic anemia.\nExcess free α-chains aggregate into insoluble inclusions within red cells and their precursors (normoblasts). These inclusions cause membrane damage that results in apoptosis of normoblasts in the marrow → ineffective erythropoiesis. The red cells which escape death in bone marrow are prone to splenic sequestration and destruction due to cell membrane damage and decreased deformability, leading to extravascular hemolysis.\n\n\nThe second mechanism is more important in producing anemia.\nAnaemia leads to several additional problems -\n\n\nIncreased erythropoietin secretion in response to anemia leads to massive erythroid hyperplasia in bone marrow that results in expansion of bone marrow and skeletal deformities.\nErythropoietin also induces extramedullary erythropoiesis in liver and spleen -p Hepatosplenomegaly.\nExcessive absorption of iron (in response to ineffective erythropoiesis) along with repeated blood transfusion in severe β-thalassemia results in iron overload and secondary hemochromatosis → iron overload in heart, liver, endocrine glands, spleen -4 cardiac failure / endocrine deficiency → Death.', 'role': 'assistant'}]