{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T15:08:45Z","timestamp":1784128125427,"version":"3.55.0"},"reference-count":12,"publisher":"The Endocrine Society","issue":"5","license":[{"start":{"date-parts":[[2025,4,15]],"date-time":"2025-04-15T00:00:00Z","timestamp":1744675200000},"content-version":"vor","delay-in-days":19,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,3,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>We report a case of a 19-year-old male with type 1 diabetes mellitus (T1DM) diagnosed at age 2 years, childhood growth retardation, and multiple admissions for diabetic ketoacidosis, presenting with hepatomegaly and elevated liver transaminase. His hemoglobin A1c (HbA1c) was 13.1% (reference range, &amp;lt; 5.7%). Massive hepatomegaly without splenomegaly was noted and accompanied by significant liver enzyme derangement, and lactatemia. Extensive viral, serologic, genetic, and metabolic tests to identify the etiology of hepatomegaly were unrevealing. A liver biopsy showed microvesicular and macrovesicular steatosis with periportal and lobular inflammation consistent with glycogenic hepatopathy (GH) of Mauriac syndrome. A continuous subcutaneous insulin infusion therapy was initiated and gradually titrated. With an improvement in HbA1c down to 9.2% over 9 months, liver transaminase levels became normalized. The current report includes a thorough evaluation of causes of hepatomegaly in an adult with T1DM and highlights the importance of glycemic control in ameliorating GH.<\/jats:p>","DOI":"10.1210\/jcemcr\/luaf070","type":"journal-article","created":{"date-parts":[[2025,4,15]],"date-time":"2025-04-15T09:34:36Z","timestamp":1744709676000},"source":"Crossref","is-referenced-by-count":1,"title":["Hepatomegaly in an Adult With Type 1 Diabetes Mellitus: Mauriac Syndrome Still Exists in a Developed Country"],"prefix":"10.1210","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8928-8287","authenticated-orcid":false,"given":"Kaveh","family":"Fekri","sequence":"first","affiliation":[{"name":"Department of Medicine, Division of Endocrinology, Diabetes and Metabolism, University of Toledo College of Medicine and Life Sciences , Toledo, OH 43614 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yalda","family":"Soleimanifard","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Toledo College of Medicine and Life Sciences , Toledo 43614, OH ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0926-5721","authenticated-orcid":false,"given":"John Y","family":"Jun","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Endocrinology, Diabetes and Metabolism, University of Toledo College of Medicine and Life Sciences , Toledo, OH 43614 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2025,4,15]]},"reference":[{"issue":"1","key":"2025041509341248000_luaf070-B1","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/s13052-018-0598-2","article-title":"Hepatomegaly and type 1 diabetes: a clinical case of Mauriac's syndrome","volume":"45","author":"Lombardo","year":"2019","journal-title":"Ital J Pediatr"},{"issue":"2","key":"2025041509341248000_luaf070-B2","doi-asserted-by":"crossref","first-page":"172","DOI":"10.4254\/wjh.v10.i2.172","article-title":"Glycogenic hepatopathy: a narrative review","volume":"10","author":"Sherigar","year":"2018","journal-title":"World J Hepatol"},{"issue":"7","key":"2025041509341248000_luaf070-B3","doi-asserted-by":"crossref","first-page":"1545","DOI":"10.1111\/liv.14827","article-title":"Liver chemistries in glycogenic hepatopathy associated with type 1 diabetes mellitus: a systematic review and pooled analysis","volume":"41","author":"Haffar","year":"2021","journal-title":"Liver Int"},{"issue":"Suppl 1","key":"2025041509341248000_luaf070-B4","doi-asserted-by":"crossref","first-page":"S59","DOI":"10.2337\/dc25-S004","article-title":"4. Comprehensive medical evaluation and assessment of comorbidities: standards of care in diabetes-2025","volume":"48","author":"American Diabetes Association Professional Practice Committee","year":"2025","journal-title":"Diabetes Care"},{"issue":"1","key":"2025041509341248000_luaf070-B5","doi-asserted-by":"crossref","first-page":"1257","DOI":"10.1038\/s41598-022-04825-8","article-title":"Simple diameter measurement as predictor of liver volume and liver parenchymal disease","volume":"12","author":"Seppelt","year":"2022","journal-title":"Sci Rep"},{"issue":"3","key":"2025041509341248000_luaf070-B6","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1007\/s10545-014-9744-1","article-title":"Evaluation of glycogen storage disease as a cause of ketotic hypoglycemia in children","volume":"38","author":"Brown","year":"2015","journal-title":"J Inherit Metab Dis"},{"key":"2025041509341248000_luaf070-B7","first-page":"402","article-title":"Gros ventre, hepatomegalie, troubles de las croissance chez les enfants diabetiques traits depuis plusieurs annes par l'insuline","volume":"26","author":"Mauriac","year":"1930","journal-title":"Gax Hebd Med Bordeaux"},{"key":"2025041509341248000_luaf070-B8","doi-asserted-by":"crossref","first-page":"2050313X18785510","DOI":"10.1177\/2050313X18785510","article-title":"Old syndrome-new approach: Mauriac syndrome treated with continuous insulin delivery","volume":"6","author":"Kocova","year":"2018","journal-title":"SAGE Open Med Case Rep"},{"issue":"7","key":"2025041509341248000_luaf070-B9","doi-asserted-by":"crossref","first-page":"2051","DOI":"10.2337\/db16-0099","article-title":"Discovery of a genetic metabolic cause for mauriac syndrome in type 1 diabetes","volume":"65","author":"MacDonald","year":"2016","journal-title":"Diabetes"},{"issue":"4","key":"2025041509341248000_luaf070-B10","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1136\/archdischild-2013-304426","article-title":"Hepatopathy of mauriac syndrome: a retrospective review from a tertiary liver centre","volume":"99","author":"Fitzpatrick","year":"2014","journal-title":"Arch Dis Child"},{"issue":"12","key":"2025041509341248000_luaf070-B11","doi-asserted-by":"crossref","first-page":"3860","DOI":"10.2337\/dc13-2088","article-title":"Thirty years of research on the Dawn phenomenon: lessons to optimize blood glucose control in diabetes","volume":"36","author":"Porcellati","year":"2013","journal-title":"Diabetes Care"},{"issue":"6","key":"2025041509341248000_luaf070-B12","doi-asserted-by":"crossref","first-page":"1262","DOI":"10.1177\/1932296820949939","article-title":"Patients with type 1 diabetes treated with insulin pumps need widely heterogeneous basal rate profiles ranging from negligible to pronounced diurnal variability","volume":"15","author":"Lindmeyer","year":"2021","journal-title":"J Diabetes Sci Technol"}],"container-title":["JCEM Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/3\/5\/luaf070\/62928911\/luaf070.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/3\/5\/luaf070\/62928911\/luaf070.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,15]],"date-time":"2025-04-15T09:34:38Z","timestamp":1744709678000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jcemcr\/article\/doi\/10.1210\/jcemcr\/luaf070\/8113489"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3,27]]},"references-count":12,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2025,3,27]]}},"URL":"https:\/\/doi.org\/10.1210\/jcemcr\/luaf070","relation":{},"ISSN":["2755-1520"],"issn-type":[{"value":"2755-1520","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2025,5]]},"published":{"date-parts":[[2025,3,27]]},"article-number":"luaf070"}}