{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,10]],"date-time":"2026-01-10T18:25:34Z","timestamp":1768069534539,"version":"3.49.0"},"reference-count":21,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[1999,10,1]],"date-time":"1999-10-01T00:00:00Z","timestamp":938736000000},"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":[[1999,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The current mainstay of treatment in glycogen storage disease type I (GSD I) is dietary management that includes providing a frequent source of glucose to prevent hypoglycaemia. To ensure compliance, routine follow\u2010up by a health care team, including a dietitian, experienced in the treatment of GSD is necessary. We describe an adolescent patient with GSD Ib in good metabolic control who was admitted with a 3\u2010month history of weakness, depression, vomiting, decreased appetite and a 11.4\u2010kg weight loss. He had a recent onset of unsteady gait, inability to write, and sore mouth. After an extensive work\u2010up, the patient was found to have vitamin B<jats:sub>12<\/jats:sub>, folate, iron and other nutritional deficiencies, which explained his symptoms. The patient improved within 72h of initiation of total parenteral nutrition and therapeutic doses of deficient micronutrients, with a complete recovery in 2 months. Dietary restrictions, dependence on non\u2010food products (e.g. cornstarch in GSD I), and social and developmental issues place individuals with metabolic disorders at a high risk for developing an array of nutritional deficiencies. This case highlights the importance of both close follow\u2010up of the metabolic control and close monitoring of growth and nutritional intake in individuals with inborn errors of metabolism. This case also illustrates the importance of daily supplementation with appropriate multivitamins, calcium and other minerals needed to meet the Recommended Dietary Allowances (RDAs) in these patients.<\/jats:p>","DOI":"10.1023\/a:1005549823146","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T22:31:09Z","timestamp":1040509869000},"page":"795-801","source":"Crossref","is-referenced-by-count":17,"title":["Nutritional deficiencies in a patient with glycogen storage disease type Ib"],"prefix":"10.1002","volume":"22","author":[{"given":"P. S.","family":"Kishnani","sequence":"first","affiliation":[{"name":"Department of Pediatrics Duke University Medical Center  Durham North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Boney","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Duke University Medical Center  Durham North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Y.\u2010T.","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Duke University Medical Center  Durham North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1999,10]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1994.tb05110.x"},{"key":"e_1_2_1_3_1","first-page":"935","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Chen Y\u2013T","year":"1995"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198401193100306"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(87)80124-5"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02072090"},{"key":"e_1_2_1_7_1","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/S0002-8223(21)07779-8","article-title":"Diet and growth of children with glycogen storage diseases types I and III","volume":"3","author":"Daeschel IE","year":"1983","journal-title":"J Am Diet Assoc"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442683"},{"key":"e_1_2_1_9_1","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/S0002-8223(21)08145-1","article-title":"Dietary management of type I glycogen storage disease","volume":"84","author":"Folk CC","year":"1984","journal-title":"J Am Diet Assoc"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(97)01463-4"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-8223(93)92246-T"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197602192940805"},{"key":"e_1_2_1_13_1","volume-title":"Recommended Dietary Allowances","author":"Havel R","year":"1989"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02034750"},{"key":"e_1_2_1_15_1","first-page":"149","article-title":"Characterizing nutrient intakes of adolescents by sociodemographic factors","volume":"15","author":"Johnson RK","year":"1994","journal-title":"Soc Adolesc Med"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.8211187"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.100.3.323"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-8223(98)00329-0"},{"key":"e_1_2_1_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1991.tb11793.x"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1995.tb13748.x"},{"key":"e_1_2_1_21_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.102.4.913"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1990.tb11366.x"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1023%2FA%3A1005549823146","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1023\/A%3A1005549823146","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T04:45:55Z","timestamp":1694753155000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1023\/A%3A1005549823146"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1999,10]]},"references-count":21,"journal-issue":{"issue":"7","published-print":{"date-parts":[[1999,10]]}},"alternative-id":["10.1023\/A:1005549823146"],"URL":"https:\/\/doi.org\/10.1023\/a:1005549823146","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1999,10]]}}}