{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T19:49:24Z","timestamp":1787600964242,"version":"build-2736575974"},"reference-count":45,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2003,6,1]],"date-time":"2003-06-01T00:00:00Z","timestamp":1054425600000},"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":[[2003,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The occurrence of (symptoms related to) osteopenia is a known complication in glycogen storage disease type Ia (GSD Ia) patients. However, only limited information is available about bone mineral density (BMD). Using dual energy x\u2010ray absorptiometry, we studied both cross\u2010sectional and longitudinal lumbar spine areal BMD (BMD<jats:sub>areal<\/jats:sub> in g\/cm<jats:sup>2<\/jats:sup>), areal BMD corrected for delayed bone maturation (BMD<jats:sub>bone age<\/jats:sub> in g\/cm<jats:sup>2<\/jats:sup>), and volumetric BMD (BMD<jats:sub>vol<\/jats:sub> in g\/cm<jats:sup>3<\/jats:sup>). Prepubertal GSD Ia patients (<jats:italic>n<\/jats:italic> = 8) had normal BMD (median <jats:italic>z<\/jats:italic>\u2010scores BMD<jats:sub>areal<\/jats:sub> \u20100.6, BMD<jats:sub>bone age<\/jats:sub> \u20100.5 and BMD<jats:sub>vol<\/jats:sub> \u2010 0.5), whereas adolescent patients (<jats:italic>n<\/jats:italic> = 12) and adult patients (<jats:italic>n<\/jats:italic> = 9) had significantly reduced BMD (BMD<jats:sub>areal<\/jats:sub> \u20102.3, BMD<jats:sub>bone age<\/jats:sub> \u20101.6, BMD<jats:sub>vol<\/jats:sub> \u20102.0, and BMD<jats:sub>areal<\/jats:sub> \u20101.9, BMD<jats:sub>vol<\/jats:sub>\u20101.5, respectively). Our longitudinal study, showing a stable BMD<jats:sub>areal<\/jats:sub> but a trend to a decrease in BMD<jats:sub>vol<\/jats:sub> in prepubertal patients during follow\u2010up, did not clarify whether the difference in BMD between prepubertal and adolescent\/adult patients reflects a diminished accretion of BMD during childhood or reflects historica l differences in treatment. In adolescent and adult GSD Ia patients, BMD<jats:sub>areal<\/jats:sub> and BMD<jats:sub>vol<\/jats:sub> were reduced but stable during follow\u2010up. Especially patients with delayed bone maturation were at risk for reduced BMD. No correlation between parameters of metabolic control and BMD could be detected. Daily calcium intake was within recommended allowances ranges. Abnormal biochemical results included hypomagnesaemia (29%), hypercalciuria (34%) and reduced tubular resorption of phosphate (21%).Although the underlying pathophysiology of reduced BMD in GSD Ia remains unsolved, metabolic control should be optimized to correct as much as possible metabolic and endocrine abnormalitie s that may influence both bone matrix formation and bone mineral accretion.<\/jats:p>","DOI":"10.1023\/a:1025111220095","type":"journal-article","created":{"date-parts":[[2003,9,16]],"date-time":"2003-09-16T22:25:03Z","timestamp":1063751103000},"page":"371-384","source":"Crossref","is-referenced-by-count":29,"title":["Bone mineral density in children, adolescents and adults with glycogen storage disease type Ia: A cross\u2010sectional and longitudinal study"],"prefix":"10.1002","volume":"26","author":[{"given":"J. P.","family":"Rake","sequence":"first","affiliation":[{"name":"Division of Metabolic Diseases, Department of Paediatrics Beatrix Children's Hospital Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Visser","sequence":"additional","affiliation":[{"name":"Division of Metabolic Diseases, Department of Paediatrics Beatrix Children's Hospital Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Huismans","sequence":"additional","affiliation":[{"name":"Division of Metabolic Diseases, Department of Paediatrics Beatrix Children's Hospital Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Huitema","sequence":"additional","affiliation":[{"name":"Division of Metabolic Diseases, Department of Paediatrics Beatrix Children's Hospital Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Van Der Veer","sequence":"additional","affiliation":[{"name":"Laboratory of Bone Metabolism, Department of Paediatrics Beatrix Children's Hospital Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. A.","family":"Piers","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine University Hospital Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. P. A.","family":"Smit","sequence":"additional","affiliation":[{"name":"Division of Metabolic Diseases, Department of Paediatrics Beatrix Children's Hospital Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2003,6]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"69","volume-title":"Osteoporosis","author":"Bachrach L","year":"1994"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1210\/jcem-73-3-555"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.82.1.57"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.me.42.020191.000313"},{"key":"e_1_2_1_6_1","first-page":"1521","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Chen YT","year":"2001"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199008303230907"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00441207"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-662-04285-4_6"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002239900138"},{"key":"e_1_2_1_11_1","first-page":"s78","article-title":"Catabolism in uremia: the impact of metabolic acidosis","volume":"9","author":"Franch HA","year":"1998","journal-title":"J Am Soc Nephrol"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1515\/JPEM.2000.13.6.571"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00369209"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1210\/jcem-70-5-1330"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1991.2"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(96)70424-9"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.79.3.691"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(91)90483-2"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1073-4449(12)80070-7"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199406233302510"},{"key":"e_1_2_1_21_1","first-page":"F442","article-title":"Acidosis inhibits osteoblastic and stimulates osteoclastic activity in vitro","volume":"262","author":"Krieger NS","year":"1992","journal-title":"Am J Physiol"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/0169-6009(92)90712-M"},{"key":"e_1_2_1_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0169-6009(08)80094-3"},{"key":"e_1_2_1_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S8756-3282(95)00162-X"},{"key":"e_1_2_1_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02029361"},{"key":"e_1_2_1_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70020-X"},{"key":"e_1_2_1_27_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.132.3.379"},{"key":"e_1_2_1_28_1","first-page":"200 202","article-title":"Case report 765. Von Gierke's disease","volume":"22","author":"Miller TT","year":"1993","journal-title":"SkeletalRadiol"},{"key":"e_1_2_1_29_1","first-page":"545","article-title":"Glycogen storage disease type b: microsomal glucose\u20106\u2010phosphatase system in two patients with different clinical findings","volume":"17","author":"Narisawa K","year":"1983","journal-title":"Pediatrics"},{"key":"e_1_2_1_30_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.107.4.840"},{"key":"e_1_2_1_31_1","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1997.443"},{"key":"e_1_2_1_32_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004310051281"},{"key":"e_1_2_1_33_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-002-0999-4"},{"key":"e_1_2_1_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)83422-5"},{"key":"e_1_2_1_35_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004670050479"},{"key":"e_1_2_1_36_1","first-page":"27","article-title":"The development of the skeletal system in children and the influence of muscular strength","volume":"49","author":"Sch6nau E","year":"1998","journal-title":"Horm Res"},{"key":"e_1_2_1_37_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2002.126456"},{"key":"e_1_2_1_38_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02072089"},{"key":"e_1_2_1_39_1","doi-asserted-by":"publisher","DOI":"10.3109\/15513818509078791"},{"key":"e_1_2_1_40_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.179.3.2027984"},{"key":"e_1_2_1_41_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-120-3-199402010-00008"},{"key":"e_1_2_1_42_1","volume-title":"Assessment of Skeletal Maturity and Prediction of Adult Height (TW2 Method)","author":"Tanner JM","year":"1983"},{"key":"e_1_2_1_43_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1016572706488"},{"key":"e_1_2_1_44_1","first-page":"127","article-title":"Metabolic and endocrine effects of metabolic acidosis in humans","volume":"131","author":"Wiederkehr M","year":"2001","journal-title":"Swiss Med Wkly"},{"key":"e_1_2_1_45_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2002.127504"},{"key":"e_1_2_1_46_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/52.6.1051"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1023%2FA%3A1025111220095","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1023\/A%3A1025111220095","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T13:20:40Z","timestamp":1694784040000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1023\/A%3A1025111220095"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2003,6]]},"references-count":45,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2003,6]]}},"alternative-id":["10.1023\/A:1025111220095"],"URL":"https:\/\/doi.org\/10.1023\/a:1025111220095","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2003,6]]}}}