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What is Kostmann&;s syndrome-treatment is and explain in detail?
kostmann syndrome characterized by hereditary neutropenia and treatment of choice is administration of granulocyte colony stimulating factor. Severe Congenital Neutropenia (Kostmann&;s Syndrome) * Clinical manifestations: Chronic severe neutropenia present at bih Accumulation of granulocytic precursors in the bone marrow Recurrent infections * Treatment with G-CSF -- Reduces infections and improves survival * Marked propensity to develop acute myeloid leukemia or myelodysplasia Reference: GHAI Essential pediatrics, 8th edition