{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T09:04:08Z","timestamp":1789376648055,"version":"build-2803163510"},"reference-count":10,"publisher":"Wiley","issue":"S3","license":[{"start":{"date-parts":[[2011,1,16]],"date-time":"2011-01-16T00:00:00Z","timestamp":1295136000000},"content-version":"vor","delay-in-days":46,"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":[[2010,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>MPI\u2010CDG (formally called CDG 1b), caused by phosphomannose isomerase (MPI) deficiency, leads to hypoglycaemia, protein losing enteropathy, hepatopathy, and thrombotic events, whereas neurologic development remains unaffected. Dietary supplementation of mannose can reverse clinical symptoms by entering the N\u2010glycosylation pathway downstream of MPI. When oral intake of mannose in patients with MPI\u2010CDG is not possible, e.g. due to surgery, mannose has to be given intravenously. We report a patient with MPI\u2010CDG on intravenous mannose therapy that showed severe depression of consciousness and seizures without apparent cause. EEG and cranial MRI findings were compatible with metabolic coma whereas extended laboratory examinations including repeated blood glucose measurements were normal. Importantly, an intravenous bolus of glucose immediately led to clinical recovery and EEG improvement. Mannose did not interfere with glucose measurement in our assay. We suggest that in patients with MPI\u2010CDG, intravenous mannose infusion can lead to intracellular ATP deprivation due to several mechanisms: (1) in MPI deficiency, mannose 6\u2010P cannot be isomerised to fructose 6\u2010P and therefore is unavailable for glycolysis; (2) animal data has shown that accumulating intracellular mannose 6\u2010P inhibits glycolysis; and (3) elevated intracellular mannose 6\u2010P may induce an ATP wasting cycle of dephosphorylation and rephosphorylation (\u201choney bee effect\u201d). The mannose\u2010induced metabolic inhibition may be overcome by high\u2010dose glucose treatment. We caution that, in patients with MPI\u2010CDG, life\u2010threatening central nervous system disturbances may occur with intravenous mannose treatment. These may be due to intracellular energy failure. Clinical symptoms of energy deficiency should be treated early and aggressively with intravenous glucose regardless of blood glucose levels.<\/jats:p>","DOI":"10.1007\/s10545-010-9252-x","type":"journal-article","created":{"date-parts":[[2011,1,15]],"date-time":"2011-01-15T05:46:18Z","timestamp":1295070378000},"page":"497-502","source":"Crossref","is-referenced-by-count":18,"title":["Seizures and stupor during intravenous mannose therapy in a patient with CDG syndrome type 1b (MPI\u2010CDG)"],"prefix":"10.1002","volume":"33","author":[{"given":"A. Sebastian","family":"Schroeder","sequence":"first","affiliation":[{"name":"Dr. von Hauner's Childrens Hospital Ludwig\u2010Maximilians\u2010University Munich Lindwurmstr. 4 Munich 80337 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Kappler","sequence":"additional","affiliation":[{"name":"Dr. von Hauner's Childrens Hospital Ludwig\u2010Maximilians\u2010University Munich Lindwurmstr. 4 Munich 80337 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Bonfert","sequence":"additional","affiliation":[{"name":"Dr. von Hauner's Childrens Hospital Ludwig\u2010Maximilians\u2010University Munich Lindwurmstr. 4 Munich 80337 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingo","family":"Borggraefe","sequence":"additional","affiliation":[{"name":"Dr. von Hauner's Childrens Hospital Ludwig\u2010Maximilians\u2010University Munich Lindwurmstr. 4 Munich 80337 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carola","family":"Schoen","sequence":"additional","affiliation":[{"name":"Dr. von Hauner's Childrens Hospital Ludwig\u2010Maximilians\u2010University Munich Lindwurmstr. 4 Munich 80337 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karl","family":"Reiter","sequence":"additional","affiliation":[{"name":"Dr. von Hauner's Childrens Hospital Ludwig\u2010Maximilians\u2010University Munich Lindwurmstr. 4 Munich 80337 Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2011,1,16]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(86)91056-9"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2008.11.012"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70139-3"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M511982200"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198401263100404"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2002.tb00101.x"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI2350"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.08.2257"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.131.3396.297"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI104814"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-010-9252-x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T06:23:30Z","timestamp":1694759010000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-010-9252-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,12]]},"references-count":10,"journal-issue":{"issue":"S3","published-print":{"date-parts":[[2010,12]]}},"alternative-id":["10.1007\/s10545-010-9252-x"],"URL":"https:\/\/doi.org\/10.1007\/s10545-010-9252-x","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,12]]}}}