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

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

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

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
Volume15
Issue number5
DOIs
Publication statusPublished - 1992 Sep
Externally publishedYes

Fingerprint

Fructose-1,6-Diphosphatase Deficiency
Fructose
Glucagon
Hypoglycemia
Phosphorylases
Leukocytes
Liver

ASJC Scopus subject areas

  • Genetics(clinical)
  • Genetics
  • Endocrinology

Cite this

Fructose and glucagon loading in siblings with fructose-1,6-diphosphatase deficiency in fed state. / Nagai, T.; Yokoyama, T.; Hasegawa, Tomonobu; Tsuchiya, Y.; Matsuo, N.

In: Journal of Inherited Metabolic Disease, Vol. 15, No. 5, 09.1992, p. 720-722.

Research output: Contribution to journalArticle

@article{474f41c589174d90ad529001524ccd8c,
title = "Fructose and glucagon loading in siblings with fructose-1,6-diphosphatase deficiency in fed state",
abstract = "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.",
author = "T. Nagai and T. Yokoyama and Tomonobu Hasegawa and Y. Tsuchiya and N. Matsuo",
year = "1992",
month = "9",
doi = "10.1007/BF01800012",
language = "English",
volume = "15",
pages = "720--722",
journal = "Journal of Inherited Metabolic Disease",
issn = "0141-8955",
publisher = "Springer Netherlands",
number = "5",

}

TY - JOUR

T1 - Fructose and glucagon loading in siblings with fructose-1,6-diphosphatase deficiency in fed state

AU - Nagai, T.

AU - Yokoyama, T.

AU - Hasegawa, Tomonobu

AU - Tsuchiya, Y.

AU - Matsuo, N.

PY - 1992/9

Y1 - 1992/9

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=0026687746&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0026687746&partnerID=8YFLogxK

U2 - 10.1007/BF01800012

DO - 10.1007/BF01800012

M3 - Article

C2 - 1434510

AN - SCOPUS:0026687746

VL - 15

SP - 720

EP - 722

JO - Journal of Inherited Metabolic Disease

JF - Journal of Inherited Metabolic Disease

SN - 0141-8955

IS - 5

ER -