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Clinical Presentation

Chief Complaint: abdominal distention

Prenatal/Birth History: Unremarkable

Family History: Not contributory

Pre-biopsy Physical Examination and Laboratory Findings:

• Hypoglycemia and metabolic acidosis
• Mildly elevated liver enzymes
• Abdominal ultrasound: hepatomegaly

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Light Microscopic Findings

• The hepatocytes were diffusely enlarged and demonstrated pale cytoplasm in a mosaic pattern lined by a prominent “plant-cell” like cell membrane.
• No significant inflammation, cholestasis, steatosis or fibrosis was identified

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Ultrastructural Findings

• The hepatocytes were enlarged and contain abundant glycogen particles.
• The cell organelles were dislocated to the periphery of the cells.
• The dislocated cell organelles contributed for the hepatocytes to have a histologic feature of prominent “plant-cell” like cell membrane.

Diagnosis?

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