title: Long term G-CSF-induced remission of ulcerative colitis-like inflammatory bowel disease in a patient with glycogen storage disease Ib and evaluation of associated neutrophil function.
abstract: We present a 23-year-old female with Glycogen storage disease Ib (GSD Ib) who was diagnosed with ulcerative colitis-like inflammatory bowel disease (IBD) at 7 years of age. G-CSF therapy reversed the IBD, was required to maintain IBD remission and was well tolerated. Neutrophil functions at time of diagnosis showed impaired chemotaxis but normal superoxide anion production and bactericidal activity. Ulcerative colitis-like IBD may also be seen in GSD Ib and is responsive to G-CSF therapy. Neutrophil dysfunction is variable among patients with GSD Ib.
authors.affiliation: Department of Pediatrics and Pathology, University of Colorado Denver, Aurora, Colorado, USA.
authors.forename: Abdulrahman
authors.initials: A
authors.lastname: Alsultan
authors.forename: Ronald J
authors.initials: RJ
authors.lastname: Sokol
authors.forename: Mark A
authors.initials: MA
authors.lastname: Lovell
authors.forename: Gail
authors.initials: G
authors.lastname: Thurman
authors.forename: Daniel R
authors.initials: DR
authors.lastname: Ambruso
mesh_terms: D000328:Adult; D001707:Biopsy, Needle; D001770:Blood Bactericidal Activity; D002634:Chemotaxis, Leukocyte; D003093:Colitis, Ulcerative / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D003106:Colon / Q000473:pathology; D003113:Colonoscopy; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000150:complications*; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D009504:Neutrophils / Q000502:physiology*; D012074:Remission Induction; D013481:Superoxides / Q000378:metabolism; D055815:Young Adult
chemical_list: D013481:Superoxides; D016179:Granulocyte Colony-Stimulating Factor