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

hakkı, sema; Aprikyan, AAG; Yildirim, ŞAHİN; Aydinbelge, MUSTAFA; Gokalp, A; Ucar, C; Guran, S; Koseoglu, V; Ataoglu, T; Somerman, MJ

doi:10.1902/jop.2005.76.5.837

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

hakkı s., Aprikyan A., Yildirim Ş., Aydinbelge M., Gokalp A., Ucar C., ...Daha Fazla

JOURNAL OF PERIODONTOLOGY, cilt.76, sa.5, ss.837-844, 2005 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 76 Sayı: 5
Basım Tarihi: 2005
Doi Numarası: 10.1902/jop.2005.76.5.837
Dergi Adı: JOURNAL OF PERIODONTOLOGY
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Sayfa Sayıları: ss.837-844
Anahtar Kelimeler: autosomal recessive syndrome, delivery of dental care, Kostmann syndrome/diagnosis, neutropenia, congenital/diagnosis
Erciyes Üniversitesi Adresli: Evet

Özet

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.