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

Turk J Pediatr. 1993 Jul-Sep;35(3):201-4.

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.

Publication types

  • Case Reports

MeSH terms

  • Administration, Oral
  • Alkaline Phosphatase / therapeutic use
  • Child, Preschool
  • Fanconi Syndrome / complications*
  • Fanconi Syndrome / therapy
  • Female
  • Glycogen Storage Disease Type I / complications*
  • Glycogen Storage Disease Type I / therapy
  • Humans
  • Infant
  • Male
  • Phosphorus / therapeutic use
  • Solutions
  • Starch / therapeutic use

Substances

  • Solutions
  • Phosphorus
  • Starch
  • Alkaline Phosphatase