{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T03:56:19Z","timestamp":1782186979043,"version":"3.54.5"},"reference-count":13,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2010,7,1]],"date-time":"2010-07-01T00:00:00Z","timestamp":1277942400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2010,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Objectives:<\/jats:title><jats:p>Glycogen storage disease (GSD) type Ib is a congenital disorder of glycogen metabolism that is associated with neutropenia, neutrophil and monocyte dysfunction, and an inflammatory bowel disease (IBD) that mimics a Crohn disease phenotype. The enteric microflora is implicated in the pathogenesis of IBD; however, its role in the development of GSD\u2010associated IBD is unknown. Antibody reactivity to <jats:italic>Saccharomyces cerevisiae<\/jats:italic> antibodies (ASCA), <jats:italic>Escherichia coli<\/jats:italic> outer membrane porin C (anti\u2010OmpC), and bacterial flagellin (anti\u2010CBir1) have been associated with Crohn disease in the general population, but they have an undetermined association in children and adults with GSD\u2010Ib. Our goal was to examine the association of ASCA, anti\u2010OmpC, and anti\u2010CBir1 with the clinical features of GSD\u2010Ib enterocolitis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods:<\/jats:title><jats:p>A retrospective review identified 19 patients with GSD\u2010Ib with or without a known diagnosis of enterocolitis. Radiographic, endoscopic, and serologic data were collected and assays for ASCA, anti\u2010OmpC, and anti\u2010CBir1 obtained.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>Seven patients had combined radiographic, endoscopic, and histologic evidence of intestinal inflammation; the majority had ileocolonic involvement. Seventeen of 19 (89%) patients had elevated anti\u2010CBir1 levels (6\/7 in the IBD group and 11\/12 in the no clinical evidence of IBD group). Thirteen of 19 (68%) had elevated anti\u2010OmpC levels (5\/7 in the IBD group and 8\/12 in the no clinical evidence of IBD group). Eleven of 19 (58%) patients had elevated ASCA IgA levels (4\/7 in the IBD group and 7\/12 in the no clinical evidence of IBD group).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>Nearly all of the patients with GSD\u2010Ib had elevated anti\u2010CBir1 levels. The antibody did not differentiate those with and without a diagnosis of GSD\u2010Ib\u2010associated IBD. Seroreactivity to flagellin may represent immune dysfunction rather than active enterocolitis in this patient population. Long\u2010term follow\u2010up of the group without known IBD is required to determine whether these antibodies can predict intestinal inflammation.<\/jats:p><\/jats:sec>","DOI":"10.1097\/mpg.0b013e3181c15f78","type":"journal-article","created":{"date-parts":[[2010,4,20]],"date-time":"2010-04-20T06:47:05Z","timestamp":1271746025000},"page":"14-18","source":"Crossref","is-referenced-by-count":13,"title":["Antibodies to CBir1 Are Associated With Glycogen Storage Disease Type Ib"],"prefix":"10.1002","volume":"51","author":[{"given":"Michael K","family":"Davis","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology Department of Pediatrics USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John F","family":"Valentine","sequence":"additional","affiliation":[{"name":"Inflammatory Bowel Diseases Program Division of Gastroenterology, Hepatology, and Nutrition Department of Medicine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A","family":"Weinstein","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology Department of Pediatrics USA"},{"name":"Glycogen Storage Disease Program and Division of Pediatric Endocrinology Department of Pediatrics University of Florida Gainesville USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven","family":"Polyak","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology Department of Pediatrics USA"},{"name":"Glycogen Storage Disease Program and Division of Pediatric Endocrinology Department of Pediatrics University of Florida Gainesville USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,7]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"1521","volume-title":"The Metabolic Basis of Inherited Disease.","author":"Chen Y\u2010T","year":"2001"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02072085"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2000.105232"},{"key":"e_1_2_6_5_1","doi-asserted-by":"crossref","unstructured":"DavisMK RufoPA PolyakSF WeinsteinDA.Adalimumab for the treatment of Crohn\u2010like colitis and enteritis in glycogen storage disease type Ib. J Inherit Metab Dis. 2008 Jan 5 [Epub ahead of print].","DOI":"10.1007\/s10545-007-0774-9"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2005.03.046"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1053\/gast.2002.35379"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI200420295"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2008.04.032"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2003.tb00825.x"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)61212-4"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mpg.0000166801.61708.60"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ibd.20106"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ibd.20336"}],"container-title":["Journal of Pediatric Gastroenterology and Nutrition"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1097\/MPG.0b013e3181c15f78","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,18]],"date-time":"2024-01-18T13:30:34Z","timestamp":1705584634000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1097\/MPG.0b013e3181c15f78"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,7]]},"references-count":13,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2010,7]]}},"alternative-id":["10.1097\/MPG.0b013e3181c15f78"],"URL":"https:\/\/doi.org\/10.1097\/mpg.0b013e3181c15f78","archive":["Portico"],"relation":{},"ISSN":["0277-2116","1536-4801"],"issn-type":[{"value":"0277-2116","type":"print"},{"value":"1536-4801","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,7]]}}}