A patient with diabetes mellitus may develop accumulate excessive amounts of glycogen within hepatocytes with hepatomegaly, a condition referred to as glycogenic hepatopathy.


Clinical and laboratory features:

(1) long-standing diabetes mellitus that is poorly controlled, with elevated hemoglobin A1c levels

(2) almost all episodes have been in patients taking insulin

(3) abdominal pain

(4) variable nausea and vomiting

(5) marked elevation of liver transaminases

(6) hepatomegaly

(7) reversal of changes on improved glucose management, included pancreas transplant


Findings in the liver biopsy:

(1) accumulation of glycogen within the cytoplasm and nuclei of hepatocytes with diffuse pallor on H+E stains

(2) usually little or no steatosis (fat accumulation)

(3) absence of significant inflammation


Differential diagnosis:

(1) steatohepatitis (alcoholic or non-alcoholic)

(2) hereditary glycogen storage disease


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