Neonate with recurrent infection and abscess diagnosed of kostmann syndrome. Treatment include:
High-Yield Explanation
Kostmann syndrome is severe neutropenia with maturation defect in the marrow progenitor cells. Recombinant G-CSF will increase neutrophil counts in most patients. The dose should be regulated to keep the absolute neutrophil count below 10,000/L. 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.