ArticleItalian journal of pediatrics2019
Hepatomegaly and type 1 diabetes: a clinical case of Mauriac's syndrome.
Article in Italian journal of pediatrics, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 22 citations in OpenAlex.
- Hepatomegaly in an Adult With Type 1 Diabetes Mellitus: Mauriac Syndrome Still Exists in a Developed Country.JCEM case reports · 2025Article
- CXCL12 as a Potential Hub Gene for N-Acetylcysteine Treatment of T1DM Liver Disease.Biomolecules · 2025Article
- Mauriac Syndrome in Sudanese Children: An Old Syndrome Still Existing in Resource-Limited Countries.Pediatric diabetes · 2025Article
- Glycogenic hepatopathy - An underrecognised cause of transaminitis in primary care settings: A case report.Malaysian family physician : the official journal of the Academy of Family Physicians of Malaysia · 2025Article
- Article
- Persistent Lactatemia in Mauriac Syndrome.Case reports in endocrinology · 2024Article
- Mauriac Syndrome Still Exists in Poorly Controlled Type 1 Diabetes: A Report of Two Cases and Literature Review.Cureus · 2021Article
- Hepatopathy Associated With Type 1 Diabetes: Distinguishing Non-alcoholic Fatty Liver Disease From Glycogenic Hepatopathy.Frontiers in pharmacology · 2021Review
- Glycogenic hepatopathy in children with poorly controlled type 1 diabetes mellitus.Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology · 2021Article
- Advances in paediatrics in 2019: current practices and challenges in allergy, endocrinology, gastroenterology, public health, neonatology, nutrition, nephrology, neurology, respiratory diseases and rheumatic diseases.Italian journal of pediatrics · 2020Review
- Improvement of Glycogenic Hepatopathy With Minimal Corresponding Improvement of Glycemic Control in a Person With Type 1 Diabetes: Case Report and Literature Review.AACE clinical case reportsArticle
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHepatic glycogenosis is characterized by excessive glycogen accumulation in hepatocytes and represents a complication of poor controlled type 1 diabetes. It can be caused by excessive insulin doses or recurrent ketoacidosis episodes. Mauriac's syndrome is a rare disease, which includes short stature, growth maturation delay, dyslipidemia, moon facies, protuberant abdomen, hepatomegaly with transaminase elevation. It has become even less common after the emergence of advances on diabetes treatment, but still exists. Recent reports described glycogenosis without the full spectrum of Mauriac's syndrome in both adults and children with brittle diabetes. Clinical, laboratory and histological abnormalities are reversible with appropriate glycemic control. CASE PRESENTATION: We hereby report a case of 11-year-old male who presented with hepatic glycogenosis mimicking Mauriac's syndrome. The patient was admitted at our Pediatric Diabetes Clinic for marked hepatomegaly, short stature and for the poor metabolic control. Blood investigations and liver tests excluded most of major causes of hepatopathy. A liver biopsy allowed us to make diagnosis of hepatic glycogenosis. To control hyperglycaemia, initially we titrated daily insulin dosage, and then intravenous insulin treatment was practiced with the consequent normalization of liver enzymes.
conclusionMauriac's syndrome should be considered in subjects with brittle type 1 diabetes and hepatomegaly.
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