FRUCTOSE AND GLUCAGON LOADING IN SIBLINGS WITH FRUCTOSE-1,6-DIPHOSPHATASE DEFICIENCY IN FED STATE

被引：5

作者：

NAGAI, T ^{[1
]}

YOKOYAMA, T ^{[1
]}

HASEGAWA, T ^{[1
]}

TSUCHIYA, Y ^{[1
]}

MATSUO, N ^{[1
]}

机构：

[1] KEIO UNIV, SCH MED, DEPT PEDIAT, TOKYO 108, JAPAN

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1992年 / 15卷 / 05期

关键词：

D O I：

10.1007/BF01800012

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：720 / 722

页数：3

共 8 条

[1]

BAERLOCHER K, 1971, HELV PAEDIATR ACTA, V26, P489

[2]

BAKER L, 1970, LANCET, V2, P13

[3]

Gitzelmann R, 1989, METABOLIC BASIS INHE, P399

[4] INHIBITION OF PHOSPHORYLASE-A BY FRUCTOSE-1-PHOSPHATE, ALPHA-GLYCEROPHOSPHATE AND FRUCTOSE-1,6-DIPHOSPHATE - EXPLANATION FOR FRUCTOSE-INDUCED HYPOGLYCEMIA IN HEREDITARY FRUCTOSE INTOLERANCE AND FRUCTOSE-1,6-DIPHOSPHATASE DEFICIENCY [J].