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J Pediatr Endocrinol Metab. 2007 Apr;20(4):545-9.

Orthotopic liver transplantation in glucose-6-phosphatase deficiency--Von Gierke disease--with multiple hepatic adenomas and concomitant focal nodular hyperplasia.

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  • 1Hepatology, Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, Brazil.


Von Gierke disease is occasionally complicated by hepatic adenomas (HAs) causing great concern owing to the current difficulties in monitoring them regarding malignant transformation. Orthotopic liver transplantation (OLT) is proposed as a therapeutic tool when multiple HAs and poor metabolic control are present, owing to the lack of a clear-cut criterion to detect early malignant transformation, whether or not associated with growth retardation. Focal nodular hyperplasia (FNH) has never been described together with multiple adenomas in von Gierke disease. We report a 26-year-old woman with von Gierke disease complicated by multiple HAs concomitant with FNH who underwent OLT and is now free from disease symptoms with good long-term outcome. In conclusion, although FNH could have been managed clinically, when multiple adenomas are present, OLT should be planned for some patients, mainly for those with poor metabolic control.

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