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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
Author(s) -
Alsultan Abdulrahman,
Sokol Ronald J.,
Lovell Mark A.,
Thurman Gail,
Ambruso Daniel R.
Publication year - 2010
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22706
Subject(s) - ulcerative colitis , medicine , inflammatory bowel disease , glycogen storage disease , gastroenterology , disease , colitis , immunology
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. Pediatr Blood Cancer. 2010;55:1410–1413. © 2010 Wiley‐Liss, Inc.