FRUCTOSE-1,6-DIPHOSPHATASE DEFICIENCY

被引：0
|
作者：
PINKAS, M
PINCHON, TM
机构：
来源：
SEMAINE DES HOPITAUX | 1992年 / 68卷 / 1-2期
关键词：
FRUCTOSE 1,6 DIPHOSPHATASE DEFICIENCY; HYPOGLYCEMIA; FRUCTOSE; SUCROSE; GLYCEROL; SORBITOL;
D O I：
暂无
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
Fructose-1,6-diphosphatase deficiency, first described in 1970, is a severe inherited disease responsible for life-threatening events in neonates and young infants. Patients have reduced tolerance to fructose, sorbitol and glycerol: after oral ingestion of these products, hypoglycemia and acidosis develop. Treatment is essentially preventive and includes a fructose and sucrose-free diet and avoidance of fasting. Can only be prescribed medicines without fructose, sucrose, sorbitol and glycerol.
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页码：12 / 13
页数：2
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