Kostmann&;s syndrome-treatment is
High-Yield Explanation
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