Pinto, Maria João Rodrigues Ferreira and Melo, Nuno and Flores, Luís and Cunha, Francisco (2018) Mauriac Syndrome: A Rare Complication of Type 1 Diabetes Mellitus. European Journal of Case Reports in Internal Medicine, 5 (12). ISSN 2284-2594
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Abstract
Mauriac syndrome, first described in 1930, is typically diagnosed in young patients with poorly controlled type 1 diabetes mellitus and growth retardation, delayed puberty, Cushingoid features, hypercholesterolaemia and hepatomegaly. However, the sole presenting feature of Mauriac syndrome can be hepatic glycogenosis in both adults and children. The mainstay of treatment for hepatic glycogenosis is strict control of glucose levels, with an excellent prognosis with improved glycaemic control. The authors present the case of a 22-year-old female patient with type 1 diabetes mellitus and a history of poor glycaemic control who was admitted with diabetic ketoacidosis (DKA). She complained of episodes of right upper quadrant abdominal pain associated with nausea and vomiting for the last 2 months with worsening in the last 48 hours. Physical examination was remarkable for short stature and tenderness over the hepatic area with a mildly enlarged liver. The patient had elevated liver enzymes and persistent hyperlactacidaemia despite DKA resolution. Liver imaging suggested diffuse fat infiltration. The clinical suspicion of hepatic glycogenosis was confirmed by liver biopsy. After glycaemic control was improved, liver enzymes normalized and the episodes of abdominal pain, nausea and vomiting subsided.
| Item Type: | Article |
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| Uncontrolled Keywords: | Mauriac syndrome, hepatic glycogenosis, chronic liver disease, type 1 diabetes mellitus |
| Subjects: | 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) > 616 Malattie (classificare qui la Clinica medica, la medicina basata sull'evidenza, la Medicina interna, la Medicina sperimentale) |
| Depositing User: | Greta Di Blasio |
| Date Deposited: | 10 Jun 2019 16:15 |
| Last Modified: | 10 Jun 2019 16:15 |
| URI: | http://eprints.bice.rm.cnr.it/id/eprint/18813 |
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