All issues > Volume 32(11); 1989

Original Article

J Korean Pediatr Soc. 1989;32(11):1568-1573. Published online November 30, 1989.

A case of Kostmann syndrome.

Won Sup Shin¹, Sang Woo Kim¹, In Kee Paik²

¹Department of Pediatrics, Paik Hospital in Seoul, In Je Medical Collage, Seoul, Korea
²Department of Pathology, Paik Hospital in Seoul, In Je Medical Collage, Seoul, Korea

Received: March 13, 1989;  Accepted: July 7, 1989.

Abstract

Kostmann Syndrome is an uncommon disorder, which is characterized by recurring infections, begining soon after birth, arid by abscence of granulocyte in the peripheral blood. The authors experienced a case of Kostmann Syndrome in a 5 months old boy who had suffered from recurrent infection since 7 days of age. We diagnosed this case by history, physical examina- tions, culture of bone marrow stem cell and other lab. findings. Bone marrow study in this study disclosed morphologic details of Kostmann Syndrome. The authors reported a case of Kostmann Syndrome with a brief review of related literatures.

Keywords :Kostmann Syndrome, Agranulocytosis