{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T00:12:58Z","timestamp":1793059978142,"version":"4.5.0"},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,8,21]],"date-time":"2021-08-21T00:00:00Z","timestamp":1629504000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,8,21]],"date-time":"2021-08-21T00:00:00Z","timestamp":1629504000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Endocr Disord"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>The Mauriac syndrome was described in 1930 as a peculiar combination of poorly controlled diabetes mellitus type 1, stunted growth and glycogenic hepatopathy. More recently, lactic acidosis was recognized as an additional feature, often induced by insulin treatment.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 17-year old girl known for diabetes type 1A and Mauriac syndrome was admitted to the emergency room with hyperglycemia of &gt;\u200941 mmol\/l without ketoacidosis. Under a standard insulin regimen, hyperglycemia was rapidly corrected but marked hyperlactatemia occurred.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>The mechanism of impaired glucose utilization and lactate elevation independent of ketoacidosis in Mauriac syndrome is intriguing. The rarity of Mauriac syndrome and its resemblance to glycogen storage diseases suggest the presence of a specific metabolic or genetic predisposition that remains to be identified.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12902-021-00835-1","type":"journal-article","created":{"date-parts":[[2021,8,21]],"date-time":"2021-08-21T10:02:56Z","timestamp":1629540176000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Elevated lactate in Mauriac syndrome: still a mystery"],"prefix":"10.1186","volume":"21","author":[{"given":"Brice","family":"Touilloux","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henri","family":"Lu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Belinda","family":"Campos-Xavier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Superti-Furga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Hauschild","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Th\u00e9r\u00e8se","family":"Bouthors","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4158-536X","authenticated-orcid":false,"given":"Christel","family":"Tran","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,8,21]]},"reference":[{"key":"835_CR1","first-page":"402","volume":"26","author":"P Mauriac","year":"1930","unstructured":"Mauriac P. Gros ventre, h\u00e9patom\u00e9galie, troubles de la croissance chez les enfants diab\u00e9tiques trait\u00e9s depuis plusieurs ann\u00e9es par l'insuline. Gaz Hebd Med Bourdeaux. 1930;26:402\u201310.","journal-title":"Gaz Hebd Med Bourdeaux"},{"issue":"2","key":"835_CR2","doi-asserted-by":"publisher","first-page":"e11","DOI":"10.2337\/dc14-2205","volume":"38","author":"MC Brouwers","year":"2015","unstructured":"Brouwers MC, Ham JC, Wisse E, et al. Elevated lactate levels in patients with poorly regulated type 1 diabetes and glycogenic hepatopathy: a new feature of Mauriac syndrome. Diabetes Care. 2015;38(2):e11\u20132. https:\/\/doi.org\/10.2337\/dc14-2205.","journal-title":"Diabetes Care"},{"key":"835_CR3","first-page":"6072909","volume":"2016","author":"KS Deemer","year":"2016","unstructured":"Deemer KS, Alvarez GF. A rare case of persistent lactic acidosis in the ICU: glycogenic Hepatopathy and Mauriac syndrome. 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Written consent for publication of this case study and accompanying data was obtained from the patient after she turned 18\u2009years.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"172"}}