HEPATIC GLYCOGENOSIS ASSOCIATED WITH TYPE 1 DIABETES (A CASE REPORT)

Background:insulin deficiency in diabetes leads to many complications. Hepatocyte glycogen overload, previously known as Mauriac syndrome, is one of them. It is a rare syndrome initially described in type 1 diabetic children in whom significant hyperglycemia is followed by administration of high doses of insulin.

Case Presentation:We present the case of a 17-year-old girl withuncontrolled type 1 diabetes. Admitted for growth retardation in addition to impuberism. The clinical examination revealed a weight delay associated with a distended abdomen and hepatomegaly. The laboratory work-up showed cytolysis and anicteric cholestasis with negative viral serologies and immunological assessment. Hepatic ultrasound revealed a 19 cm hepatomegaly without any focal signs or steatosis, as confirmed by a bili-MRI that proved the same finding. A management based on insulin therapy was introduced. The etiological investigation of the hepatic disturbances was negative.

Conclusions:The course was satisfactory under appropriate insulin therapy. The diagnosis of hepatic glycogenosis was based on a combination of anamnestic, clinical and histological findings, given the absence of other abnormalities responsible for the liver disturbances.