title: Type I glycogenosis with renal tubular dysfunction (presentation of two cases).
abstract: 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.
authors.affiliation: Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.
authors.forename: A
authors.initials: A
authors.lastname: Yüce
authors.forename: T
authors.initials: T
authors.lastname: Coşkun
authors.forename: N
authors.initials: N
authors.lastname: Koçak
authors.forename: S
authors.initials: S
authors.lastname: Ozsoylu
authors.forename: I
authors.initials: I
authors.lastname: Ozalp
authors.forename: S
authors.initials: S
authors.lastname: Göğüş
mesh_terms: D000284:Administration, Oral; D000469:Alkaline Phosphatase / Q000627:therapeutic use; D002675:Child, Preschool; D005198:Fanconi Syndrome / Q000150:complications* / Q000628:therapy; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D010758:Phosphorus / Q000627:therapeutic use; D012996:Solutions; D013213:Starch / Q000627:therapeutic use
chemical_list: D012996:Solutions; D010758:Phosphorus; D013213:Starch; D000469:Alkaline Phosphatase