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Glycogenic Hepatopathy: A Rare Cause of Increased Aminotransferase Levels in a Diabetic Patient
Image of the Month Glycogenic Hepatopathy: A Rare Cause of Increased Aminotransferase Levels in a Diabetic Patient
JOHN T. BASSETT,* GANESH R. VEERAPPAN,‡ and DONG H. LEE* *Gastroenterology Department, National Naval Medical Center, Bethesda, Maryland; and the ‡Gastroenterology Service, Walter Reed Army Medical Center, Washington, District of Columbia
Liver biopsy showed normal liver architecture with no evidence of hepatocellular injury, inflammation, fibrosis, or steatosis. However, the hepatocytes were diffusely swollen with rarefaction of the cytoplasm and accentuation of the cell membranes, giving the appearance of plant-like cells (Figure A). The periodic acid–Schiff stain showed abundant glycogen deposits (Figure B). Increased aminotransferase levels in diabetic patients are often secondary to nonalcoholic fatty liver disease or steatohepatitis. We describe a rare case of increased transaminase levels in a diabetic patient caused by glycogenic hepatopathy (GH). GH is also known as hepatic glycogenosis, liver glycogenosis, liver glycogen storage, and diabetes mellitus–associated glycogen storage hepatomegaly, which is a unique pathologic condition in which glycogen accumulates in the hepatocytes of type I diabetic patients when prolonged hyperglycemia is treated with insulin.1,2 GH is differentiated from glycogen storage disease based on the clinical history of poorly controlled type I diabetes. Patients may present with hepatomegaly, abdominal pain, nausea, and vomiting. Aminotransferase levels are up to 10 times the upper limit of normal. On histology, liver architecture is preserved and hepatocytes are diffusely swollen and have glycogen deposits seen on periodic acid-Schiff stain (Figures A and B).2 Once a patient is diagnosed with GH, liver enzyme levels normalize with improved glycemic control as seen in our patient (aspartate aminotransferase level, 25 U/L; alanine aminotransferase level, 28 U/L; HgbA1C, 9.1%), with counseling from the primary provider.1 GH is a rare and likely underrecognized condition of poorly controlled diabetes. It is a clinicopathologic diagnosis with unique pathologic features that are reversible with good glycemic control exemplified by our patient. References
A
19-year-old woman with type 1 diabetes mellitus presented with an 18-month history of increased aminotransferase levels and poorly controlled diabetes (HgbA1C 10.1%). She took glargine and lispro insulin, with no significant past medical or alcohol history. Physical examination, vital signs, and body mass index (20.1 kg/m2) were within normal limits. A right upper-quadrant ultrasound had no fatty infiltration. Alanine aminotransferase and aspartate aminotransferase levels were increased to 147 U/L (reference, 7–56) and 143 U/L (17– 49), respective. Her alkaline phosphatase level was 137 U/L (36 –126) and her total bilirubin was normal. Initial investigations excluded all metabolic and infectious causes.
1. Cuthbertson DJ, Brennan G, Walsh S, et al. Hepatic glycogenosis: abnormal liver function tests in type 1 diabetes. Diabet Med 2007;24:322–328. 2. Torbenson M, Chen Y, Brunt E, et al. Glycogenic hepatopathy—an underrecognized hepatic complication of diabetes mellitus. Am J Surg Pathol 2006;30:508 –513.
The opinions are solely those of the authors and do not represent an endorsement by the Department of Defense. This is a US government work. There are no restrictions on its use. Published by Elsevier Inc. on behalf of the AGA Institute 1542-3565/08/$0.00 doi:10.1016/j.cgh.2008.04.034 CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2008;6:xxvi
JOHN T. BASSETT,* GANESH R. VEERAPPAN,‡ and DONG H. LEE* *Gastroenterology Department, National Naval Medical Center, Bethesda, Maryland; and the ‡Gastroenterology Service, Walter Reed Army Medical Center, Washington, District of Columbia
Liver biopsy showed normal liver architecture with no evidence of hepatocellular injury, inflammation, fibrosis, or steatosis. However, the hepatocytes were diffusely swollen with rarefaction of the cytoplasm and accentuation of the cell membranes, giving the appearance of plant-like cells (Figure A). The periodic acid–Schiff stain showed abundant glycogen deposits (Figure B). Increased aminotransferase levels in diabetic patients are often secondary to nonalcoholic fatty liver disease or steatohepatitis. We describe a rare case of increased transaminase levels in a diabetic patient caused by glycogenic hepatopathy (GH). GH is also known as hepatic glycogenosis, liver glycogenosis, liver glycogen storage, and diabetes mellitus–associated glycogen storage hepatomegaly, which is a unique pathologic condition in which glycogen accumulates in the hepatocytes of type I diabetic patients when prolonged hyperglycemia is treated with insulin.1,2 GH is differentiated from glycogen storage disease based on the clinical history of poorly controlled type I diabetes. Patients may present with hepatomegaly, abdominal pain, nausea, and vomiting. Aminotransferase levels are up to 10 times the upper limit of normal. On histology, liver architecture is preserved and hepatocytes are diffusely swollen and have glycogen deposits seen on periodic acid-Schiff stain (Figures A and B).2 Once a patient is diagnosed with GH, liver enzyme levels normalize with improved glycemic control as seen in our patient (aspartate aminotransferase level, 25 U/L; alanine aminotransferase level, 28 U/L; HgbA1C, 9.1%), with counseling from the primary provider.1 GH is a rare and likely underrecognized condition of poorly controlled diabetes. It is a clinicopathologic diagnosis with unique pathologic features that are reversible with good glycemic control exemplified by our patient. References
A
19-year-old woman with type 1 diabetes mellitus presented with an 18-month history of increased aminotransferase levels and poorly controlled diabetes (HgbA1C 10.1%). She took glargine and lispro insulin, with no significant past medical or alcohol history. Physical examination, vital signs, and body mass index (20.1 kg/m2) were within normal limits. A right upper-quadrant ultrasound had no fatty infiltration. Alanine aminotransferase and aspartate aminotransferase levels were increased to 147 U/L (reference, 7–56) and 143 U/L (17– 49), respective. Her alkaline phosphatase level was 137 U/L (36 –126) and her total bilirubin was normal. Initial investigations excluded all metabolic and infectious causes.
1. Cuthbertson DJ, Brennan G, Walsh S, et al. Hepatic glycogenosis: abnormal liver function tests in type 1 diabetes. Diabet Med 2007;24:322–328. 2. Torbenson M, Chen Y, Brunt E, et al. Glycogenic hepatopathy—an underrecognized hepatic complication of diabetes mellitus. Am J Surg Pathol 2006;30:508 –513.
The opinions are solely those of the authors and do not represent an endorsement by the Department of Defense. This is a US government work. There are no restrictions on its use. Published by Elsevier Inc. on behalf of the AGA Institute 1542-3565/08/$0.00 doi:10.1016/j.cgh.2008.04.034 CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2008;6:xxvi