{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T08:46:10Z","timestamp":1791103570164,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2002,6,1]],"date-time":"2002-06-01T00:00:00Z","timestamp":1022889600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2002,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Glycogen storage disease type I (GSD I) (McKusick 232200) is caused by inherited defects of the glucose\u20106\u2010phosphatase complex. Patients with GSD Ia as well as patients with GSD Ib may suffer from intermittent diarrhoea, which seems to worsen with age. The cause of this diarrhoea is unknown. This study describes the results of investigations of intestinal functions and morphology in patients with GSD Ia and GSD Ib, which were performed to detect a common cause for chronic diarrhoea in GSD I. The following were investigated: faecal fat excretion, faecal \u03b1<jats:sub>1<\/jats:sub>\u2010antitrypsin and faecal chymotrypsin, expiratory H<jats:sub>2<\/jats:sub> concentrations, persorption of cornstarch in urine and colonic biopsies. With the investigations presented in this study, no common cause for diarrhoea in GSD I was found. In GSD Ib loss of mucosal barrier function due to inflammation, documented by increased faecal \u03b1<jats:sub>1<\/jats:sub>\u2010antitrypsin excretion (3.5\u20139.6\u2009mg\/g dry faeces) and inflammation in the colonic biopsies, seems to be the main cause. The inflammation is most likely related to disturbed neutrophil function, which is often found in GSD Ib. Whether another cause is involved in GSD Ia and in GSD Ib, related to the disturbed function of glucose\u20106\u2010phosphatase in the enterocyte, remains to be investigated.<\/jats:p>","DOI":"10.1023\/a:1016572706488","type":"journal-article","created":{"date-parts":[[2002,12,29]],"date-time":"2002-12-29T03:32:49Z","timestamp":1041132769000},"page":"261-267","source":"Crossref","is-referenced-by-count":33,"title":["Intestinal function in glycogen storage disease type I"],"prefix":"10.1002","volume":"25","author":[{"given":"G.","family":"Visser","sequence":"first","affiliation":[{"name":"Wilhelmina Children's Hospital University Hospital Utrecht  Utrecht"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. P.","family":"Rake","sequence":"additional","affiliation":[{"name":"Beatrix Children's Hospital, Department of Pediatrics University Hospital Groningen  Groningen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. T. M.","family":"Kokke","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Rijnstate Hospital  Arnhem"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. G. J.","family":"Nikkels","sequence":"additional","affiliation":[{"name":"Department of Pathology University Hospital Utrecht  The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. J. J.","family":"Sauer","sequence":"additional","affiliation":[{"name":"Beatrix Children's Hospital, Department of Pediatrics University Hospital Groningen  Groningen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. P. 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