A SCREENING METHOD FOR LIVER GLYCOGEN DISEASES

被引:42
作者
FERNANDES, J
HUIJING, F
VANDEKAM.JH
机构
[1] Children's Hospital, University of Utrecht, Zeist
[2] Department of Medical Enzymology, Laboratory of Biochemistry, University of Amsterdam, Zeist
[3] Central Institute for Nutrition and Food Research TNO, Zeist
关键词
D O I
10.1136/adc.44.235.311
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Blood lactate levels were followed during oral hexose tolerance tests in 18 patients with different types of liver glycogenosis. 5 patients with glucose-6-phosphatase deficiency were given a glucose tolerance test, 5 patients with a deficiency of the debranching enzyme system, and 8 patients with a deficiency of the phosphorylase system were given glucose, fructose, and galactose tolerance tests. All patients were also given a glucagon tolerance test, with estimation of blood glucose levels. Using these tests a screening procedure for the three commonest types of liver glycogen disease has been developed.
引用
收藏
页码:311 / +
页数:1
相关论文
共 19 条
[1]  
DICKENS F, 1968, CARBOHYDRATE META ED, V2, P151
[2]  
FERNANDE.J, 1965, PEDIATRICS, V35, P470
[3]  
FERNANDES J, 1968, PEDIATRICS, V41, P935
[4]   LACTATE METABOLISM - STUDIES OF A CHILD WITH A SERIOUS CONGENITAL DEVIATION [J].
HARTMANN, AF ;
PURKERSON, ML ;
WESLEY, ME ;
WOHLTMANN, HJ .
JOURNAL OF PEDIATRICS, 1962, 61 (02) :165-+
[5]  
HERS H. G., 1964, ADVAN METAB DISORDERS, V1, P1
[6]  
HERS H G, 1959, Rev Int Hepatol, V9, P35
[7]  
HERS HG, 1968, CARBOHYD METABOL, V2, P151
[8]  
HOWELL RR, 1962, PEDIATRICS, V29, P553
[9]   GLUCAGON TOLERANCE TEST IN GLYCOGEN STORAGE DISEASE [J].
HUG, G .
JOURNAL OF PEDIATRICS, 1962, 60 (04) :545-+