title: Correction of neutropenia following treatment with granulocyte colony-stimulating factor results in a decreased frequency of infections in Shwachman's syndrome.
abstract: We report the clinical efficacy of granulocyte colony-stimulating factor (G-CSF) in a 17-year-old woman with Shwachman's syndrome who was repeatedly admitted with severe infections and neutropenia. Bone marrow culture showed no increase in granulocyte colony-forming units upon stimulation with granulocyte-macrophage colony-stimulating factor (GM-CSF). The patient was treated with G-CSF 300 micrograms 3 times a week subcutaneously. A complete and sustained response of the neutropenia was obtained without side-effects during 21 months of treatment. No infectious episodes were encountered after starting G-CSF. This observation demonstrates the beneficial effect of G-CSF in treating the severe neutropenia in patients with Shwachman's syndrome.
authors.affiliation: Department of Internal Medicine, Catharina Hospital, Eindhoven, Netherlands.
authors.forename: F M
authors.initials: FM
authors.lastname: van der Sande
authors.forename: H F
authors.initials: HF
authors.lastname: Hillen
mesh_terms: D000293:Adolescent; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D010188:Exocrine Pancreatic Insufficiency / Q000628:therapy*; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D007279:Injections, Subcutaneous; D009503:Neutropenia / Q000628:therapy*; D009894:Opportunistic Infections / Q000628:therapy*; D013577:Syndrome
chemical_list: D016179:Granulocyte Colony-Stimulating Factor