Kostmann’s Neutropenia: A Case Report  

作　　者：Khaoula Achir Ilham Tadmori Mounia Lakhdar Idrissi Fatima Zahra Souilmi Mohamed Hbibi Moustapha Hida Khaoula Achir;Ilham Tadmori;Mounia Lakhdar Idrissi;Fatima Zahra Souilmi;Mohamed Hbibi;Moustapha Hida(Pediatric Department of CHU Hassan II in Fez, Fez, Morocco)

机构地区：[1]Pediatric Department of CHU Hassan II in Fez, Fez, Morocco

出　　处：《Open Journal of Pediatrics》2024年第5期869-872,共4页儿科学期刊（英文）

摘　　要：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.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.

关 键 词：Kostmann Syndrome Severe Congenital Neutropenia Skin Abscesses 

分 类 号：R72[医药卫生—儿科]