Fructose and glucagon loading in siblings with fructose-1,6-diphosphatase deficiency in fed state

T. Nagai, T. Yokoyama, T. Hasegawa, Y. Tsuchiya, N. Matsuo

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5 Citations (Scopus)


Hypoglycaemia induced by fructose administration is one of the diagnostic clues to fructose-1,6-diphosphatase (FDPase) deficiency (McKusick 229700). However, the pathological mechanism of this reactive hypoglycaemia is not fully known. This paper describes two siblings with FDPase deficiency, diagnosed enzymatically in leukocytes, who failed to correct reactive hypoglycaemia after glucagon administration even in the fed state, supporting a possibility that disturbed hepatic phosphorylase activity may be a main cause of reactive hypoglycaemia.

Original languageEnglish
Pages (from-to)720-722
Number of pages3
JournalJournal of Inherited Metabolic Disease
Issue number5
Publication statusPublished - 1992 Sep 1
Externally publishedYes


ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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