Periodontal status in two siblings with severe congenital neutropenia: Diagnosis and mutational analysis of the cases

hakkı s., Aprikyan A., Yildirim S., Aydinbelge M., Gokalp A., Ucar C., ...More

JOURNAL OF PERIODONTOLOGY, vol.76, no.5, pp.837-844, 2005 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 76 Issue: 5
  • Publication Date: 2005
  • Doi Number: 10.1902/jop.2005.76.5.837
  • Journal Name: JOURNAL OF PERIODONTOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.837-844
  • Keywords: autosomal recessive syndrome, delivery of dental care, Kostmann syndrome/diagnosis, neutropenia, congenital/diagnosis
  • Erciyes University Affiliated: No

Abstract

Background: Severe congenital neutropenia (SCN), also known as Kostmann syndrome, was originally reported as an autosomal recessive disease of neutrophil production. The disease is characterized by a maturation arrest of neutrophil precursors at the promyelocytic stage of differentiation and by extremely low levels of mature neutrophils in peripheral blood.