Kostmann syndrome, or severe congenital neutropenia, is a rare condition in children marked by a neutrophil count of less than 500/mm3. This congenital agranulocytosis, an autosomal recessive genetic disorder, is ofte...Kostmann syndrome, or severe congenital neutropenia, is a rare condition in children marked by a neutrophil count of less than 500/mm3. This congenital agranulocytosis, an autosomal recessive genetic disorder, is often first identified by a neonatal infectious syndrome. The deficiency in neutrophils increases susceptibility to bacterial and fungal infections. Prior to the availability of hematopoietic growth factors, the disease was associated with significant morbidity and early mortality. We present the case of a 17-month-old boy who was admitted to the pediatric emergency department at Hassan II University Hospital in Fes with skin abscesses.展开更多
文摘Kostmann syndrome, or severe congenital neutropenia, is a rare condition in children marked by a neutrophil count of less than 500/mm3. This congenital agranulocytosis, an autosomal recessive genetic disorder, is often first identified by a neonatal infectious syndrome. The deficiency in neutrophils increases susceptibility to bacterial and fungal infections. Prior to the availability of hematopoietic growth factors, the disease was associated with significant morbidity and early mortality. We present the case of a 17-month-old boy who was admitted to the pediatric emergency department at Hassan II University Hospital in Fes with skin abscesses.
