Hypertransaminasemia in poorly-controlled type-1 diabetes mellitus

C. Merino Palacios, J. Primo Vera¹, J. Fernández Chinchilla¹, J. Ferrando Marco², M. Aragó Galindo and L. García Ferrer

Service of Internal Medicine, ¹Division of Gastroenterology and ²Department of Pathology. Hospital de Sagunto. Valencia. Spain

A 16-year-old boy had been diagnosed with type-1 diabetes mellitus at the age of 9 with no subsequent appropriate metabolic control (HbA1c: 10-11%). He presented frequent hypoglycemic episodes and required hospitalization in several occasions because of vomiting and ketoacidosis. His weight and height were in low percentiles (BMI: 21.1 kg/m²) for his age and laboratory data did not disclosed any abnormality except those related with the diabetes. While remaining asymptomatic, an ordinary laboratory test revealed abnormal liver function parameters (AST/ALT: 578/526 U/l, GGT: 284 U/l) and a raised lipid profile (cholesterol/triglycerides 225/223: mg/dl). An initial study yielded negative results (virus serologic tests, autoantibodies, electrophoresis, complement, alpha1 antitrypsin, and celiac disease screening tests). For the following 8 months, the patients remained asymptomatic, although with a bad control of the diabetes and elevated serum levels of aminotransferases (AST 59-227 IU/l, ALT 68-120 IU/l y GGT 53-120 IU/l). At that moment, physical examination revealed the presence of a hepatomegaly and a new investigation demonstrated positive ANA (1/640), but other autoantibodies were (SMA, LKM-1, AMA) negative. His HLA haplotype was DR4. Abdominal ultrasonography demonstrated a diffuse hyperechogenic liver suggestive of fatty infiltration. A liver biopsy revealed a normal liver architecture with mild inflammatory infiltrates in some portal tracts and no piecemeal necrosis. Liver cells showed a pale and microvacuolated cytoplasm (arrow in Fig. 1, hematoxylin and eosin x 200) without additional hepatic injury. These cytoplasmic deposits were PAS-positive (Fig. 2) and not visible following diastase digestion indicating the presence of deposits of liver glycogen and excluding hepatic steatosis. In conclusion, this patient with poor controlled type-1 diabetes mellitus developed a hepatic glycogenosis resembling Mauriac's syndrome (1). This complication can revert following optimal control of the glycemia (2).

REFERENCES

1. Chatila R, West AB. Hepatomegaly and abnormal liver test due to glycogenosis in adults with diabetes. Medicine (Baltimore) 1996; 75: 327-33.

2. Munns CF, McCrossin RB, Thomsett MJ, Batch J. Hepatic glycogenosis: reversible hetomegaly in type 1 diabetes. J Paediatr Child Health 2000; 36: 449-52.