{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T10:09:37Z","timestamp":1780049377254,"version":"3.53.1"},"reference-count":13,"publisher":"The Endocrine Society","issue":"2","license":[{"start":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T00:00:00Z","timestamp":1768780800000},"content-version":"vor","delay-in-days":11,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,1,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Mauriac syndrome is a rare complication of type 1 diabetes mellitus (T1D) with chronically elevated hemoglobin A1C (HbA1c) that is characterized by short stature, delayed puberty, cushingoid features, and hepatic glycogenosis. We report a 14-year-old male patient with T1D managed with multiple daily insulin injections who presented with growth failure and delayed puberty in the setting of several years of HbA1c &amp;gt; 12% (SI: &amp;gt; 108\u2005mmol\/mol) (reference range, &amp;lt; 5.7% [SI: &amp;lt; 39\u2005mmol\/mol]). He was initially suspected to have growth hormone deficiency, failed a growth hormone stimulation test and received growth hormone treatment without an increase in height velocity. After several months, he presented with abdominal distention due to new hepatomegaly. Laboratory evaluation revealed transaminitis with normal synthetic function and absence of cholestasis. Liver biopsy confirmed hepatic glycogenosis. Treatment included T1D management re-education, psychosocial support, and transition to automated insulin delivery (AID). AID resulted in decreased HbA1c level, normalized liver enzymes, resolution of hepatomegaly, puberty progression, and increased linear growth in line with his mid-parental height. This patient demonstrated that growth failure and delayed puberty can precede hepatic glycogenosis and that AID is a safe and effective treatment option for patients with Mauriac syndrome.<\/jats:p>","DOI":"10.1210\/jcemcr\/luaf282","type":"journal-article","created":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T13:18:26Z","timestamp":1761830306000},"source":"Crossref","is-referenced-by-count":1,"title":["Mauriac Syndrome: Growth and Clinical Outcomes After 2.5 Years of Automated Insulin Delivery Treatment"],"prefix":"10.1210","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-0849-3889","authenticated-orcid":false,"given":"Hanine","family":"Alarab","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of Washington , Seattle, WA 98145 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4769-5626","authenticated-orcid":false,"given":"Lina","family":"Merjaneh","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology and Metabolism, Texas Children's Hospital, Baylor College of Medicine , Houston, TX 77030 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5310-1057","authenticated-orcid":false,"given":"Kelsey B","family":"Eitel","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Washington , Seattle, WA 98145 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2026,1,19]]},"reference":[{"key":"2026011902133235200_luaf282-B1","first-page":"402","article-title":"Gros ventre, h\u00e9patom\u00e9galie, troubles de la croissance chez les enfants diab\u00e9tiques trait\u00e9s depuis plusieurs ann\u00e9es par l\u2019insuline","volume":"26","author":"Mauriac","year":"1930","journal-title":"Gaz Hebd Med Bordeaux"},{"issue":"4","key":"2026011902133235200_luaf282-B2","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":"Suppl 4","key":"2026011902133235200_luaf282-B3","first-page":"989","article-title":"Mauriac syndrome: growth failure and type 1 diabetes mellitus","volume":"5","author":"Kim","year":"2008","journal-title":"Pediatr Endocrinol Rev"},{"issue":"2","key":"2026011902133235200_luaf282-B4","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 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meta-analysis","volume":"76","author":"Roos","year":"2024","journal-title":"EClinicalMedicine"},{"issue":"7","key":"2026011902133235200_luaf282-B8","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":"1","key":"2026011902133235200_luaf282-B9","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1097\/01.mpg.0000243434.54958.21","article-title":"High prevalence of alpha-1-antitrypsin heterozygosity in children with chronic liver disease","volume":"44","author":"Campbell","year":"2007","journal-title":"J Pediatr Gastroenterol 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Obes Metab"}],"container-title":["JCEM Case 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