title: Granulocyte colony-stimulating factor-responsive chronic neutropenia in cartilage-hair hypoplasia.
abstract: : Chronic neutropenia is common in children with cartilage-hair hypoplasia (CHH). The authors describe a 6-year-old with severe CHH, moderate neutropenia associated with serum IgG antibodies directed against Fcgamma-RIIIb (NA1/2), and frequent bacterial infections. In this patient, long-term administration of granulocyte colony-stimulating factor increased peripheral neutrophil counts and prevented recurrent hospitalizations for bacterial lower respiratory tract infections.
authors.affiliation: Department of Pediatrics, University of Bern, Bern, Switzerland.
authors.forename: Roland A
authors.initials: RA
authors.lastname: Ammann
authors.forename: Andrea
authors.initials: A
authors.lastname: Duppenthaler
authors.forename: Juergen
authors.initials: J
authors.lastname: Bux
authors.forename: Christoph
authors.initials: C
authors.lastname: Aebi
mesh_terms: D000130:Achondroplasia / Q000150:complications / Q000188:drug therapy*; D002648:Child; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006201:Hair Diseases / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007958:Leukocyte Count; D008297:Male; D009503:Neutropenia / Q000097:blood / Q000188:drug therapy* / Q000209:etiology*; D011994:Recombinant Proteins
chemical_list: D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor