EC Gastroenterology and Digestive System

Case Report Volume 10 Issue 3 - 2023

Isolated Hepatomegaly Revealing a Mauriac Syndrome (Case Report)

F Haddad1, K Belhaj1*, FZ El Rhaoussi1, M Tahiri Joutei Hassani1, W Hliwa1, A Bellabah1, W Badre1, M Azim2, M Regragui2 and M Karkouri2

1Service de Gastro-entérologie, CHU Ibn Rochd, Casablanca, Morocco

2Laboratoire d`Anatomie Pathologie, CHU Ibn Rochd, Casablanca, Morocco

*Corresponding Author: K Belhaj, Service de Gastro-entérologie, CHU Ibn Rochd, Casablanca, Morocco.
Received: March 23, 2023; Published: May 03, 2023



Background: Poor insulin compliance exposes type 1 diabetic patients to several complications. Hepatic glycogenosis (HG) or Mauriac syndrome is one of them. HG results from a significant accumulation of glycogen in the hepatocytes. It’s manifested by short stature, growth maturation delay, dyslipidemia, protuberant abdomen with hepatomegaly. Its management is based on the appropriate control of diabetes.

Case Presentation: We report a case of 15-years-old female, with type 1 diabetes, who was admitted for marked hepatomegaly. Several causes of hepatopathy were excluded with blood investigations and liver tests. The diagnosis of hepatic glycogenosis was retained histologically after a liver biopsy.

Conclusion: Although hepatic glycogenosis is a rare entity, it should be considered in cases of hepatomegaly in a context of unbalanced type 1 diabetes.

Keywords: Type 1 Diabetes Mellitus; Hepatic Glycogenosis; Mauriac Syndrome; Liver Biopsy

  1. Giordano S., et al. “Diagnosis of hepatic glycogenosis in poorly controlled type 1 diabetes mellitus”. World Journal of Diabetes6 (2014): 882-888.
  2. Julián MT., et al. “Hepatic glycogenosis: an underdiagnosed complication of diabetes mellitus?” World Journal of Diabetes2 (2015): 321-325.
  3. Mahesh S., et al. “Mauriac syndrome in a 3-year-old boy”. Endocrine Practice 13 (2007): 63-66.
  4. Mauriac P. “Gros ventre, hépatomégalie, trouble de la croissance chez les enfants diabétiques : traités depuis plusieurs années par l’insuline”. Gaz Hebl Sci Med Bordeaux 26 (1930): 402-410.
  5. Brondani V., et al. “Mauriac’s syndrome: an uncommon and old complication of type 1 diabetes mellitus”. Diabetology and Metabolic Syndrome1 (2015): A25.
  6. Mahévas T., et al. “Œdème insulinique au cours d’une glycogénose hépatique”. Revue de Médecine Interne 38 (2017): 201-203.
  7. Nordlie RC., et al. “Regulation of glucose production by the liver”. Annual Review of Nutrition 19 (1999): 379-406.
  8. Bua J., et al. “Hepatic glycogenosis in an adolescent with diabetes”. The Journal of Pediatrics 157 (2010): 1042.
  9. Munns CF., et al. “Hepatic glycogenosis: reversible hepatomegaly in type 1 diabetes”. Journal of Paediatrics and Child Health 36 (2000): 449-452.
  10. Haller MJ., et al. “Type 1 diabetes in the child and adolescent”. In: Lifshitz F (edition) Pediatric endocrinology, 5th Informa Healthcare, Switzerland (2007): 70.
  11. Bril F and Cusi K. “Management of nonalcoholic fatty liver disease in patients with type 2 diabetes: a call to action”. Diabetes Care 40 (2017): 419-430.
  12. Manderson WG., et al. “Liver glycogen accumulation in unstable diabetes”. Diabetes 17 (1968): 13-16.

K Belhaj., et al. "Isolated Hepatomegaly Revealing a Mauriac Syndrome (Case Report)". EC Gastroenterology and Digestive System  10.3 (2023): 08-11.

ECronicon Journal

Our Services

Quick Links

Information

Creative Commons LicenseOpen Access by ECronicon is licensed under a Creative Commons Attribution 4.0 International License
Based on a work at www.ecronicon.net