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What is A child with recurrent severe bacterial infections is diagnosed of having Kostmann's syndrome. Treatment is: and explain in detail?
Kostmann syndrome is the severe neutropenia with maturation defect in the marrow progenitor cells. Recombinant G-CSF will increase neutrophil counts in most patients and has the potential to prolong life expectancy. Hematopoietic stem cell transplantation may be considered for patients with severe complications, especially those with severe congenital neutropenia. Ref: Ambruso D.R., Hays T., Goldenberg N.A., Nuss R. (2012). Chapter 30. Hematologic Disorders. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.