علاج داء تخزين الغليكوجين من النوع الأول مع الورم الحميد الكبدي

References

DOI: 10.1038/gim.2014.128

Initially, the management of liver adenomas in the GSD I population should be conservative (Box 3).

Transplantation should be reserved for patients who have not had success with medical management, have a history of recurrent adenomas despite liver resection, have a rapid increase in the size and number of liver adenomas, and are at high risk for liver cancer.

After liver transplantation, all GSD I patients have achieved resolution of their metabolic derangement, including correction of hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia.