{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,21]],"date-time":"2026-02-21T08:23:00Z","timestamp":1771662180490,"version":"3.50.1"},"reference-count":23,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2005,12,1]],"date-time":"2005-12-01T00:00:00Z","timestamp":1133395200000},"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":[[2005,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Despite highly increased blood lipids, patients with glycogen storage disease type Ia (GSD Ia) do not develop premature vascular complications. Since this could be due to changes of coagulation factors, coagulation tests (including von Willebrand factor (VWF) antigen (VWF:Ag) ELISA, VWF:collagen binding activity (VWF:CB) and VWF multimer analysis) were performed in 10 GSD Ia patients, single cases of other GSD types, and in both healthy and hyperlipidaemic controls. In 60% of GSD Ia patients we found abnormal results, with a decrease of VWF:Ag and multimer analysis showing reduced intensity of individual oligomers in the presence of all multimers with a normal triplet structure. We interpret these findings as an aquired \u2018von Willebrand syndrome type I\u2019 in GSD Ia. The underlying metabolic mechanism and a potential role in the protection from vascular complication still needs to be evaluated.<\/jats:p>","DOI":"10.1007\/s10545-005-0184-9","type":"journal-article","created":{"date-parts":[[2006,1,25]],"date-time":"2006-01-25T09:35:29Z","timestamp":1138181729000},"page":"945-950","source":"Crossref","is-referenced-by-count":26,"title":["Decreased plasma concentration of von Willebrand factor antigen (VWF:Ag) in patients with glycogen storage disease type Ia"],"prefix":"10.1002","volume":"28","author":[{"given":"C.","family":"M\u00dcHlhausen","sequence":"first","affiliation":[{"name":"Departments of Pediatrics University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Schneppenheim","sequence":"additional","affiliation":[{"name":"Departments of Pediatric Hematology and Oncology University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"U.","family":"Budde","sequence":"additional","affiliation":[{"name":"Prof Arndt &amp; Partners Laboratory University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Merkel","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Muschol","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Ullrich","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Santer","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics University Medical Center Hamburg\u2010Eppendorf Hamburg Germany"},{"name":"Department of Pediatrics University Medical Center Martinistr. 52 Hamburg D\u201020246 Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,12]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(86)90150-7"},{"key":"e_1_2_1_3_1","first-page":"1521","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Chen YT","year":"2001"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(74)80288-X"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V41.2.235.235"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1754-4505.1998.tb01641.x"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198904000-00002"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)82052-9"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442739"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.51.1.49"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199611000-00017"},{"issue":"2","key":"e_1_2_1_12_1","first-page":"106","article-title":"Description of an in vitro platelet function analyser \u2014 PFA\u2010100","volume":"21","author":"Kundu SK","year":"1995","journal-title":"Semin Thromb Hemost"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0021-9150(94)90072-8"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V68.1.180.180"},{"key":"e_1_2_1_15_1","first-page":"18","article-title":"Enzyme\u2010linked immunoabsorbent assay of factor VIII\u2010related antigen. 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