Hepatic Glycogenosis: A Rare Complication of Type 1 Diabetes Mellitus.

Type 1 diabetes mellitus (T1DM) is a common autoimmune pathology requiring lifelong insulin therapy. We report the case of a 12-year-old girl with T1DM admitted to Department C of the National Institute of Nutrition of Tunis for diabetic ketosis. She had suffered from T1DM for five years, with poor glycemic control (hemoglobin A1C = 10%) and poor therapeutic adherence. On examination, she had abdominal bloating with homogeneous hepatomegaly. Her height was 146 cm (less than the second percentile), her weight was 38 kg (less than the second percentile), and her body mass index was 17.8 kg/m². Tanner's stage was S1P1A1. Biological investigations showed mixed dyslipidemia, normal liver and renal functions, and normal thyroid-stimulating hormone levels. The aspartate aminotransferase/alanine aminotransferase ratio was 1.35. Ultrasound of the abdomen revealed hepatomegaly with a liver span of 19 cm. Based on the clinical history and investigations, Mauriac syndrome was the most likely diagnosis of our patient. A holistic multidisciplinary approach, in collaboration with the child psychiatrist, was opted to optimize diabetes management and reduce hepatic metabolic overload. Further investigations were conducted to rule out differential diagnoses, especially viral and autoimmune hepatitis. Poor acceptance of type 1 diabetes leads to non-compliance with insulin therapy. Then, energy metabolism becomes defective with growth retardation and pubertal delay. Glucose accumulates in the liver leading to metabolic liver disease. Liver damage could be irreversible. Therapeutic education, a good doctor-patient relationship, and family support are the cornerstones of managing T1DM diabetes complicated by Mauriac syndrome.