期刊
ACTA PAEDIATRICA
卷 95, 期 12, 页码 1526-1532出版社
WILEY
DOI: 10.1080/08035250601087607
关键词
antibacterial peptides; granulocyte colony-stimulating factor; Kostmann syndrome; periodontitis; severe congenital neutropenia
类别
Congenital neutropenia in man was first reported 50 years ago by the Swedish paediatrician Rolf Kostmann. He coined the term infantile genetic agranulocytosis'' for this condition, which is now known as Kostmann syndrome. Recent studies have demonstrated a lack of antibacterial peptides and severe periodontitis in these patients despite recombinant growth factor treatment. Moreover, an increased degree of apoptosis of myeloid progenitor cells in the bone marrow has been shown.
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