{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T00:12:41Z","timestamp":1793059961889,"version":"4.5.0"},"reference-count":13,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2022,6,2]],"date-time":"2022-06-02T00:00:00Z","timestamp":1654128000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,6,2]],"date-time":"2022-06-02T00:00:00Z","timestamp":1654128000000},"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":["J Med Case Reports"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>We describe a case of Mauriac syndrome, which is a rare complication of poorly controlled type I diabetes that combines glycogenosis, hepatomegaly, growth retardation with a Cushingoid appearance that is most often present in children but also in young adults. Here we also describe another finding with this syndrome, which is hyperlactatemia.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>The case is of\u00a0a 16-year-old female of North African ethnicity with history of poorly controlled type I diabetes who was brought to the emergency department for dyspnea and tachycardia, treated initially for diabetic ketoacidosis. Her persistent hyperlactatemia helped to reveal a more subtle condition known as Mauriac syndrome after multiple examinations and follow-up.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>This case reports shows that Mauriac syndrome is a rare condition that should be considered in a setting of poorly controlled type I diabetes, hepatomegaly, Cushingoid appearance, and hyperlactatemia. The current treatment of this condition is a\u00a0strict control of blood glucose levels with an attempt to achieve an acceptable glycated hemoglobin value.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13256-022-03416-5","type":"journal-article","created":{"date-parts":[[2022,6,2]],"date-time":"2022-06-02T15:06:15Z","timestamp":1654182375000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Persistent hyperlactatemia in decompensated type I diabetes with hepatic glycogenosis and hepatomegaly: Mauriac syndrome: a case report"],"prefix":"10.1186","volume":"16","author":[{"given":"Waheed","family":"Dolip","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Bourmanne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte","family":"Van Homwegen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Van Nuffelen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,6,2]]},"reference":[{"key":"3416_CR1","doi-asserted-by":"publisher","first-page":"132","DOI":"10.1016\/j.jcrc.2011.07.071","volume":"27","author":"C Kristin","year":"2012","unstructured":"Kristin C, Michael N, Cocchi MD, Justin D. Prevalence and significance of lactic acidosis in diabetic ketoacidosis. J Crit Care. 2012;27:132\u20137.","journal-title":"J Crit Care"},{"key":"3416_CR2","doi-asserted-by":"crossref","unstructured":"Jeffrey A.Kraut and N.E.Madias. Lactic acidosis. N Engl J Med 2015 vol.371, no. 24, pp. 615-624.","DOI":"10.1056\/NEJMra1309483"},{"issue":"9","key":"3416_CR3","doi-asserted-by":"publisher","first-page":"615","DOI":"10.3949\/ccjm.82a.14098","volume":"82","author":"AJ Reddy","year":"2015","unstructured":"Reddy AJ, Lam SW, Bauer SR, Guzman JA. Lactic acidosis: clinical implications and management strategies. Clevel Clin J Med. 2015;82(9):615\u201324.","journal-title":"Clevel Clin J Med"},{"key":"3416_CR4","doi-asserted-by":"publisher","first-page":"e11","DOI":"10.2337\/dc14-2205","volume":"38","author":"CG Martijn","year":"2015","unstructured":"Martijn CG, Brouwers J, Ham JC, Eddie W, Shivani M. Elevated lactate levels in patients with poorly regulated type 1 diabetes and glycogenic hepatopathy: a new feature of Mauriac syndrome. Diabetes Care. 2015;38:e11\u20132.","journal-title":"Diabetes Care"},{"key":"3416_CR5","first-page":"6072909","volume":"3","author":"SD Kisten","year":"2016","unstructured":"Kisten SD, Alvarez GF. A rare case of persistent lactic acidosis in the ICU: glycogenic hepatopathy and Mauriac syndrome. Case Rep Crit Care. 2016;3:6072909.","journal-title":"Case Rep Crit Care"},{"key":"3416_CR6","first-page":"402","volume":"26","author":"P Mauriac","year":"1930","unstructured":"Mauriac P. Gros ventre, hepatomegaly, growth disorder in diabetic children treated for several years with insulin. Gaz Hebdo Med Bordeaux. 1930;26:402\u201310.","journal-title":"Gaz Hebdo Med Bordeaux"},{"key":"3416_CR7","doi-asserted-by":"publisher","first-page":"s283","DOI":"10.4103\/2230-8210.119611","volume":"17","author":"M Gutch","year":"2013","unstructured":"Gutch M, Philip R, Saran S. Re-emergence of a rare syndrome: a case of Mauriac syndrome. Indian J Endocr Metab. 2013;17:s283\u20135.","journal-title":"Indian J Endocr Metab"},{"key":"3416_CR8","doi-asserted-by":"publisher","first-page":"89","DOI":"10.15537\/smj.2017.3.17101","volume":"38","author":"A Ahmed","year":"2017","unstructured":"Ahmed A, AlSarkhy A, Zaidi ZA, Babiker AM. Glycogenic hepatopathy, an underdiagnosed cause of relapsing hepatitis in uncontrolled type 1 diabetes mellitus. Saudi Med J. 2017;38:89\u201392.","journal-title":"Saudi Med J"},{"key":"3416_CR9","doi-asserted-by":"publisher","first-page":"172","DOI":"10.4254\/wjh.v10.i2.172","volume":"10","author":"MS Jagannath","year":"2018","unstructured":"Jagannath MS, Joline DE, Yong MY. Glycogenic hepatopathy: a narrative review. World J Hepatol. 2018;10:172\u201385.","journal-title":"World J Hepatol"},{"key":"3416_CR10","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1097\/00005792-199611000-00003","volume":"75","author":"R Chatila","year":"1996","unstructured":"Chatila R, West AB. Hepatomegaly and abnormal liver tests due to glycogenosis in adults with diabetes. Medicine (Baltimore). 1996;75:327\u201333.","journal-title":"Medicine (Baltimore)"},{"key":"3416_CR11","doi-asserted-by":"publisher","first-page":"424","DOI":"10.4103\/0256-4947.81803","volume":"31","author":"KS Aljabri","year":"2011","unstructured":"Aljabri KS, Bokhari SA, Fageeh SM, Alharbi AM, Abaza MA. Glycogen hepatopathy in a 13-year-old male with type 1 diabetes. Saudi Med J. 2011;31:424\u20137.","journal-title":"Saudi Med J"},{"key":"3416_CR12","doi-asserted-by":"publisher","first-page":"2051","DOI":"10.2337\/db16-0099","volume":"65","author":"MJ MacDonald","year":"2016","unstructured":"MacDonald MJ, Hasan NM, Ansari IH, Longacre MJ, Kendrick MA, Stoker SW. Discovery of a genetic metabolic cause for Mauriac syndrome in type 1 diabetes. Diabetes. 2016;65:2051\u20139.","journal-title":"Diabetes"},{"key":"3416_CR13","volume-title":"The Bright Liver of Glycogenic Hepatopathy","author":"S Seth","year":"2021","unstructured":"Seth S, Kraichely RE. The Bright Liver of Glycogenic Hepatopathy. New York: Division of Gastroenterology and Hepatology Mayo Clinic College of Medicine Rochester; 2021."}],"container-title":["Journal of Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-022-03416-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13256-022-03416-5\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-022-03416-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,2]],"date-time":"2022-06-02T15:08:43Z","timestamp":1654182523000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmedicalcasereports.biomedcentral.com\/articles\/10.1186\/s13256-022-03416-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,2]]},"references-count":13,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022,12]]}},"alternative-id":["3416"],"URL":"https:\/\/doi.org\/10.1186\/s13256-022-03416-5","relation":{},"ISSN":["1752-1947"],"issn-type":[{"value":"1752-1947","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,2]]},"assertion":[{"value":"11 January 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 April 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 June 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient\u2019s legal guardian(s) for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"Not applicable.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}},{"value":"Brugmann hospital, Brussels, Belgium; data available on request.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Availability of data and materials"}}],"article-number":"232"}}