Liver transplantation for type Ib glycogenosis with reversal of cyclic neutropenia

We describe a case of glycogen storage disease type lb in 32-year old male patient with poor metabolic control in spite of medical and nutritional management and the use of recombinant granulocyte stimulating factor. Because of this, liver transplantation was considered as a definitive treatment. We comment on the metabolic results of liver transplantation performed, with reversal of hypoglycemia, hyperuricemia, hypertrigliceridemia and cyclic neutropenia, all of which persist 4 years post-transplant. In view of this case, we believe that liver transplantation is a feasible option to consider in patients with type lb glycogenosis as a definitive therapeutic procedure. (C) 2001 Harcourt Publishers Ltd.