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KMID : 0361719960070020188
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Korean Journal of perinatology
1996 Volume.7 No. 2 p.188 ~ p.193
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A Case of Kostmann Syndrome Treated with Recombinant Human Graulocyte Colony-Stimulating Factor
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Abstract
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Kostmann syndrome is characterized by persistent and severe neutropenia presented on the first week of life and a maturation arrest of the myeloid series at the promyelecyte myelocyte stages in bone marrow, Since G-CSF has been pururified, it
demonstrated to be a potent stimuil for myeloid proliferation and differentiation, thus it improves disease morbidity and mortality.
We experienced a case of Kostmann syndrome with skin infectionand acute otitis media due to S. aureus in neonatal period. The peripheral blood smear disclosed no granulocyte and the bone marrow revealed few granulocyte precursors. His absolute
neutrophil counts were increased by administration with rhG-CSF(5μg/kg/day) and the infectious process was terminated successfully with increased neutrophil counts.
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KEYWORD
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