Mauriac syndrome is defined as glycogenic hepatopathy (GH), having Cushing-like features, growth retardation, and delayed puberty, which might be more than previously reported. The syndrome diagnosis is usually by exclusion to rule out all the causes of liver disease despite the close link to diabetes (mainly type-1 diabetes, other types of diabetes were reported on rare occasions). The main diagnostic challenge is the differentiation between GH and metabolic-associated fatty liver disease (MAFLD) (formerly nonalcoholic fatty liver disease because GH is self-limited, unlike MAFLD, which is a major risk for hepatitis, cirrhosis, and hepatoma). The literature was extensively searched for articles published on Mauriac syndrome and gave a broader insight into Mauriac syndrome. Out of the 500 articles retrieved, 57 full texts were screened, 24 articles assessed Mauriac syndrome, and the rest were used to collect and present information in the introduction and discussion. It was assumed that Mauriac syndrome is not a syndrome in developing countries and the majority were reported in the developed world after insulin analog introduction; most of the investigations, including liver biopsy, can be delayed with substantial priority to glycemic control and insulin adherence; and addressing the socioeconomic factors is of prime importance. Finally, a genetic defect in glucose metabolism was suggested. A management plan is recommended for Mauriac syndrome based on the findings and the expert’s opinion.
Key words: Mauriac syndrome, glycogenic hepatopathy, etiology, management, systemic review
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