S2490 Glycogenic Hepatopathy Masquerading as Acute Pancreatitis

Abstract

INTRODUCTION: Glycogenic hepatopathy (GH) is underrecognized amongst clinicians.1,2 We present a case of GH clinically appearing at first as acute pancreatitis. CASE DESCRIPTION/METHODS: A 20-year-old female with a medical history of poorly controlled T1DM and prior episode of pancreatitis, presented with sharp epigastric pain worsened by eating. The patient was tachycardic to 115 bpm and physical exam revealed tenderness in the RUQ-epigastric region with negative Murphy's. Lab work was significant for WBC count 12,900/ μL, lipase 353 U/L, amylase 264 U/L, BHBA 3.02 mmol/L, HbA1C 13.5, ALT and AST 34 U/L and later AST 51 U/L, ALK 191, TG 162 mg/dL, and lactate 5.4 mmol/L which increased to 8.2 mmol/L. CT abdomen revealed hepatomegaly of approximately 24–25 cm craniocaudally, hepatic steatosis, trace pelvic ascites, and normal pancreas. Abdominal U/S showed an enlarged liver with diffuse increase in parenchymal echogenicity, consistent with steatosis, without any focal lesions. Mesenteric vascular doppler showed normal vasculature and flow. Hepatitis panel, iron panel, ANA, anti-smooth muscle antibody, and ceruloplasmin were negative. Liver biopsy showed preserved parenchyma without fibrosis, focal mild macrovesicular steatosis, and occasional glycogenated nuclei. The hepatocytes had increased swelling and cytoplasmic clearing, suggestive of glycogen deposition. PAS stain revealed abundant hepatocyte glycogen deposits which were not visible after diastase digestion. The histological findings are consistent with a GH diagnosis. DISCUSSION: GH, characterized by tender hepatomegaly with elevated liver enzymes in the setting of DM, exists within the Mauriac Syndrome spectrum, but lacks the syndrome's additional cushingoid features and poor growth. GH almost always occurs with T1DM and has slight female predominance. The pathobiology of GH may involve oscillation of glucose levels with poor insulin management leading to hepatocyte glucose trapping and glycogenosis.1 Biochemical findings include elevated HbA1c, AST greater than ALT, and elevated ALK.1,2 Increased amylase and lipase levels are reported as in our patient.3 DKA and lactic acidosis is common.4 Ascites has been reported.1 On CT the liver is hyperdense, unlike a hypodense liver of NAFLD.5 Definitive diagnosis is by findings of swollen hepatocytes with glycogen accumulation on biopsy, with limited or absent fatty change, inflammation, necrosis or fibrosis.1 Unlike NAFLD which may progress to fibrosis or cirrhosis, GH resolves with dysglycemia control.6,7Figure 1.: Benign liver parenchyma with focal mild macrovascular steatosis. Many hepatocytes have increased swelling and cytoplasmic clearing.Figure 2.: Occasional glycogenated nuclei are seen, as indicated by the black arrow.Figure 3.: A PAS stain highlights abundant glycogen in the hepatocytes, as evidenced by the magenta staining, but is not seen in the PASD stain (PAS stain after diastase pretreatment).