Mauriac's Syndrome is still among us: A case report
DOI:
https://doi.org/10.33448/rsd-v14i7.49271Keywords:
Hyperglycemia, Type 1 diabetes mellitus, Hepatopathy, Hyperinsulinemia, Mauriac's Syndrome.Abstract
Mauriac's Syndrome (MS) is a rare complication of Type 1 Diabetes Mellitus (T1DM) triggered by inadequate glycemic control. The objective of this article is to present a classic case of MS, manifested in an insulin-dependent pre-pubescent diabetic, with typical symptoms and findings. Although its incidence has decreased with advances in T1DM management, MS remains clinically relevant. This study presents a case report of a 14-year-old male patient diagnosed with this condition after being referred to a specialized center, describing the findings observed during clinical evaluation and physical examination, laboratory and imaging tests, and the results of the liver biopsy performed, which confirmed the diagnosis. The presented case illustrates the persistence of MS in patients with poorly controlled T1DM, highlighting the need for healthcare professionals to be attentive to this rare but clinically significant complication. Early diagnosis and optimization of glycemic control are fundamental for the reversal of hepatic and systemic changes, as is the relevance of guiding the patient and their guardians for therapeutic success in this age group.
References
Azariadis, K., Gatselis, N. K., Koukoulis, G. K., & Dalekos, G. N. (2019). Glycogenic hepatopathy as a cause of severe deranged liver enzymes in a young patient with type 1 diabetes mellitus. BMJ Case Reports, 12(12), e228524.
Azhar, M., Hammami, M., Musmar, A., & Bromer, M. (2021). Glycogen Hepatopathy: A Reversible yet Relapsing Cause of Hepatitis in Type 1 Diabetics. Cureus, 13(2), e13441. https://doi.org/10.7759/cureus.13441
Brahmbhatt, K. J., Doshi, M. J., Dave, M., Contractor, H., & Shah, K. (2024). Mauriac Syndrome: A Rare Condition With Cushingoid Feature and Hepatomegaly in Poorly Controlled Diabetes Mellitus Type 1. Cureus, 16(7), e65057. https://doi.org/10.7759/cureus.65057
Chandel, A., Scarpato, B., Camacho, J., McFarland, M., & Mok, S. (2017). Glycogenic hepatopathy: Resolution with minimal glucose control. Case Reports in Hepatology, 7651387. https://doi.org/10.1155/2017/7651387
Fitzpatrick, E., Coley, C., Quaglia, A., Karani, J., Dhawan, A., & Champion, M. (2014). Hepatopathy of Mauriac syndrome: A retrospective review from a tertiary liver centre. Archives of Disease in Childhood, 99(4), 354–357.
Gil, A. C. (2017). Como elaborar projetos de pesquisa. (6ed). Editora Atlas.
Hudacko, R. M., Manoukian, A. V., Schneider, S. H., & Fyfe, B. (2008). Clinical resolution of glycogenic hepatopathy following improved glycemic control. Journal of Diabetes Complications, 22(5), 329–330.
Imtiaz, K. E., Healy, C., Sharif, S., & Hussaini, S. H. (2013). Glycogenic hepatopathy in type 1 diabetes: An underrecognized condition. Diabetes Care, 36(1), e6–e7.
Khoury, J., Zohar, Y., Shehadeh, N., & Saadi, T. (2018). Glycogenic hepatopathy. Hepatobiliary & Pancreatic Diseases International, 17(2), 113–118.
Kleint, A., Dussan, M., Chandran, A., & Salameh, M. (2024). A Case of Mauriac Syndrome: A Teenage Girl With Poorly Controlled Diabetes. Cureus, 16(7), e64748. https://doi.org/10.7759/cureus.64748
Koren, D. (2022). Growth and development in type 1 diabetes. Current Opinion in Endocrinology, Diabetes and Obesity, 29(1), 57–64. https://doi.org/10.1097/MED.0000000000000694
Marble, A. (1938). Enlargement of the liver in diabetic children. Archives of Internal Medicine, 62(5), 740–750. https://doi.org/10.1001/archinte.1938.00180160019002
Mauriac, P. (1930). Gros ventre, hepatomegalie, troubles de la croissance chez les enfants diabetiques traites depuis plusieurs annees par l’insuline. Gazette hebdomadaire des sciences médicales de Bordeaux, 26, 402–410.
Moreno Tirado, A., González Lázaro, P., Montero Benítez, M. Z., & Jiménez Torrecilla, P. (2023). Hepatopathy of Mauriac syndrome. The importance of therapeutic adherence. Revista Española de Enfermedades Digestivas, 115(11), 659–660. https://doi.org/10.17235/reed.2023.9513/2023
Mukewar, S., Sharma, A., Lackore, K. A., Dierkhising, R. A., & Angulo, P. (2017). Clinical, biochemical, and histopathology features of patients with glycogenic hepatopathy. Clinical Gastroenterology and Hepatology, 15(6), 927–933.
Parmar, N., Atiq, M., Austin, L., Khatiwada, P., & Al-Hawari, A. (2015). Glycogenic hepatopathy: Thinking outside the box. Case Reports in Gastroenterology, 9(2), 221–226.
Pereira A. S. et al. (2018). Metodologia da pesquisa científica. [free e-book]. Editora UAB/NTE/UFSM.
Plaza Enriquez, L., Konindala, N., Yeh, H., Khatiwada, P., Sanchez Valenzuela, M., & Askari, K. (2022). A Case Report about Glycogenic Hepatopathy. Case Reports in Endocrinology, 5134049. https://doi.org/10.1155/2022/5134049
Silva, M., Marques, M., Cardoso, H., Gomes, S., Ribeiro, A. M., & Dias, C. (2016). Glycogenic hepatopathy in young adults: A case series. Revista Española de Enfermedades Digestivas, 108(11), 673–676.
Singh, Y., Gurung, S., & Gogtay, M. (2022). Glycogen hepatopathy in type-1 diabetes mellitus: A case report. World Journal of Hepatology, 14(2), 471–478.
Toassi, R. F. C. & Petry, P. C. (2021). Metodologia científica aplicada à área da Saúde. (2ed). Editora da UFRGS.
Torbenson, M., Chen, Y. Y., Brunt, E., Brunt, L., Slezak, L., & Miller, J. (2006). Glycogenic hepatopathy: An underrecognized hepatic complication of diabetes mellitus. American Journal of Surgical Pathology, 30(4), 508–513.
Tsujimoto, T., Takano, M., Nishiofuku, M., Yoshiji, H., Matsumura, Y., Kuriyama, S., et al. (2006). Rapid onset of glycogen storage hepatomegaly in a type-2 diabetic patient after a massive dose of long-acting insulin and large doses of glucose. Internal Medicine, 45(8), 469–473.
Yin, R. K. (2024). Case Study Research and Applications: Design and Methods (7th ed.). Sage Publications.
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Copyright (c) 2025 Leonardo Davi Kallaur Grangeiro; Julio Cesar Silva Camargo de Souza; Vivian Mota Guimarães; Drielle Rezende Pavanitto Silveira
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