engleski

GLYCOGENIC HEPATOPATHY IN TYPE 1 DIABETES: AN ACUTE COMPLICATION?

Objectives and Study: Glycogenic hepatopathy (GH) key feature is glycogen accumulation in the liver causing hepatomegaly and raised serum transaminases in patients with type 1 diabetes (DM1) and poor glycemic control. Wide fluctuations in both glucose and insulin levels are held responsible for its occurence which may happen at any age and anytime during the course of the disease. We present 4 patients with GH. Methods: Liver biopsy specimens were obtained from patients and histologic features of large hepatocytes with pale cytoplasm and periodic acid Schiff (PAS) positive staining were observed. Results: A 12-year old female with 1 year history of poorly regulated DM1 presented with diabetic ketoacidosis (DKA) and severe abdominal pain. Besides hepatomegaly her physical examination was unremarkable. Elevated liver enzymes were noted (AST 630 U/L, ALT 548 U/L, GGT 87 U/L). Coagulation studies, protein electrophoresis and abdominal ultrasound (US) were normal. EBV and CMV serology were negative. Transaminase level normalized during following 2 weeks. Two months later she had new episode of abdominal pain and elevated liver enzymes (10xULN). Alpha-1 AT deficiency, Wilson disease, autoimmune and viral hepatitis (CMV, EBV, hepatitis B, C, parvoB19, adenovirus) were excluded. US revealed moderate hepatomegaly. Liver biopsy showed normal liver architecture, with no evidence of inflammation or fibrosis. Hepatocytes were large with pale cytoplasm. PAS staining showed abundant glycogen accumulation. 20% of hepatocytes showed macrovesicular steatosis. The diagnosis of GH was made. The remaining 3 patients were longtime diabetics who had at least one episode of severe DKA, and had developed the same clinical symptoms. The increase in transaminases was 10-30XULN. They had the same diagnostic procedure performed as the first patient. Important differences were detected in their liver tissue specimens. The first of them (15 years, 10 years duration of DM1, Graves' disease) had a typical GH pathohistological finding. Another (23 years, 4 years duration of DM1) additionally showed significant fibrosis.The third patient (16 years, 13 years duration of DM1, hyperlipidemia) had typical GH and perportal fibrosis and steatosis resembling non-alcocholic steatohepatitis (NASH). Improvement of glycemic control within subsequent weeks led to normalisation of transaminases in all 4 patients. Conclusion: Although GH appears as acute and fully reversible complication of DM1 at least in our patients, we lack sufficent data for that conclusion. GH may also be the first step in possible subclinical progression and development of chronic liver disease. Therefore we need to actively search for this complication and develop strategies for its long-term follow up.

Glycogenic hepatopathy; type 1 diabetes;

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