Abstract #1404927: Glycogenic Hepatopathy is a Rare Reversible Condition with Optimal Glycemic Control in Type 1 Diabetes Mellitus

Abstract

Glycogenic hepatopathy (GH) is a rare complication of type 1 DM due to over-insulin use to achieve glycemic control due to excessive calorie intake. GH developed due to the accumulation of glycogen in hepatocytes predominantly seen in the pediatric population with poorly controlled DM type 1 and rarely seen in DM 2 and characterized by transient transaminitis and hepatomegaly. Liver biopsy is the gold standard and non-invasive tests are having a limited role. 18-year-old Hispanic male with poorly controlled type 1 DM, dyslipidemia, and post-ablative hypothyroidism. The patient was non-compliant with medical treatment leading to multiple hospitalizations for diabetes ketoacidosis (DKA) and also hypoglycemic events. The patient was clinically asymptomatic (denied any nausea/vomiting or abdominal pain) and was never been diagnosed with liver dysfunction in the past. Physical examination was significant for the enlarged liver but no splenomegaly, otherwise unremarkable. Initial routine labs revealed elevated aminotransferases ALT 1329 IU/L and AST 1987 IU/L otherwise CBC and CMP, TSH were unremarkable. HbA1c was 13.7. A liver ultrasound revealed diffuse fatty infiltration and hepatomegaly (20.5 cm). No signs of biliary stones or obstruction. Serology for viral Hepatitis A’’, ‘B’ and ‘C’ were negative. Autoimmune hepatitis workup (ANA, ASMA, AMA) and celiac serology were negative. The patient underwent a liver biopsy and showed diffuse hepatocyte swelling with frequent glycogenated nuclei consistent with glycogenic hepatopathy with minimal inflammation and no evidence of steatosis. Minimal sub-sinusoidal fibrosis (Fibrosis stage 0/6). The patient was counseled for strict glycemic control with a low-carb diet (portion control) and proper use of insulin with close office follow-up and regular monitoring of blood sugar at home. Within 6 months, HbA1C improved from 13.7% to 9.8% and ALT improved from 1329 to 51 IU/L and AST decreased from 1987 to 57 IU/L. During a nine-month follow-up visit, AST and ALT remained low at 44 and 31 IU/L respectively. Patient followed the The patient underwent a repeat liver biopsy which revealed minimal accumulation of glycogen deposits and minimal hepatocyte inflammation and no evidence of fibrosis. GH and NAFLD are indistinguishable clinically and NAFLD is more prevalent in DM although GH is a revertible disease within 2-14 weeks with better glycemic control. The pathophysiology of GH is incompletely understood. It is believed to be the consequence of recurrent fluctuations of glucose level with hyperglycemia and hypoglycemia and hyperinsulinism. Supra-physiological doses of insulin to manage blood sugar are believed to be the cause of this condition. Awareness of Glycogenic hepatopathy is essential, and the proper management of elevated liver enzymes is to be addressed in uncontrolled type 1 DM patients. Recurrence of GH has been reported with repeated episodes of DKA. Further research is needed to investigate this phenomenon.