title: Granulocyte colony-stimulating factor corrects the neutropenia associated with glycogen storage disease type Ib.
abstract: A young woman with glycogen storage disease, type Ib, and chronic neutropenia had severe recurrent infections. In a life-threatening situation, treatment with granulocyte colony-stimulating factor (G-CSF) resulted in the prompt correction of neutropenia. Subsequently, daily G-CSF therapy has allowed the maintenance of a normal neutrophil count and marked clinical improvement over a period of 18 months. The spectrum of neutropenic conditions which are responsive to G-CSF should include this inherited metabolic disorder.
authors.affiliation: Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, TN 38101.
authors.forename: W C
authors.initials: WC
authors.lastname: Wang
authors.forename: W M
authors.initials: WM
authors.lastname: Crist
authors.forename: J N
authors.initials: JN
authors.lastname: Ihle
authors.forename: B A
authors.initials: BA
authors.lastname: Arnold
authors.forename: J P
authors.initials: JP
authors.lastname: Keating
mesh_terms: D000328:Adult; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D009503:Neutropenia / Q000188:drug therapy* / Q000209:etiology; D011994:Recombinant Proteins / Q000627:therapeutic use
chemical_list: D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor