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.
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.
Alsultan
Abdulrahman
A
Department of Pediatrics and Pathology, University of Colorado Denver, Aurora, Colorado, USA.
Sokol
Ronald J
RJ
Lovell
Mark A
MA
Thurman
Gail
G
Ambruso
Daniel R
DR
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
D013481:Superoxides; D016179:Granulocyte Colony-Stimulating Factor