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

研究成果: Article査読

5 被引用数 (Scopus)

抄録

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.

本文言語English
ページ(範囲)720-722
ページ数3
ジャーナルJournal of Inherited Metabolic Disease
15
5
DOI
出版ステータスPublished - 1992 9月
外部発表はい

ASJC Scopus subject areas

  • 遺伝学
  • 遺伝学(臨床)

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