Kostmann&;s syndrome-treatment is: (A) Anti-thymocyte globulin + cyclosporin, (B) Anti-thymocyte globuline + cyclosporine + GM-CSF, (C) G-CSF, (D) GM-CSF
Answer is C. 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