Type I glycogenosis with renal tubular dysfunction (presentation of two cases)

Two patients with hepatic glycogenosis associated with Fanconi syndrome are presented. Both patients were treated with a neutral phosphorus solution, an oral alkaline solution, cholecalciferol and uncooked cornstarch. The proximal renal tubular functions were corrected in the patient who used cornstarch properly, which may indicate a causal relationship between Fanconi syndrome and glycogenosis.