HYPERALANINEMIA, HYPERPYRUVICEMIA AND LACTIC-ACIDOSIS DUE TO PYRUVATE-CARBOXYLASE DEFICIENCY OF LIVER - TREATMENT WITH THIAMINE AND LIPOIC ACID

被引:37
作者
MAESAKA, H
KOMIYA, K
MISUGI, K
TADA, K
机构
[1] KANAGAWA CHILDRENS MED CTR,DEPT PEDIAT,NEUROL SECT,YOKOHAMA,JAPAN
[2] KANAGAWA CHILDRENS MED CTR,DEPT PEDIAT,ENDOCRINOL & METAB SECT,MINAMI KU,YOKOHAMA,JAPAN
[3] KANAGAWA CHILDRENS MED CTR,DEPT LABS,PATHOL SECT,YOKOHAMA,JAPAN
[4] OSAKA CITY UNIV,SCH MED,DEPT PEDIAT,OSAKA,JAPAN
关键词
D O I
10.1007/BF00466274
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
引用
收藏
页码:159 / 168
页数:10
相关论文
共 17 条
[1]   A METHOD FOR ESTIMATION OF KREBS CYCLE AND RELATED INTERMEDIATES IN ANIMAL TISSUES BY GAS CHROMATOGRAPHY [J].
BARNETT, D ;
COHEN, RD ;
TASSOPOU.CN ;
TURTLE, JR ;
DIMITRIA.A ;
FRASER, TR .
ANALYTICAL BIOCHEMISTRY, 1968, 26 (01) :68-+
[2]  
BLASS J P, 1970, Journal of Clinical Investigation, V49, P423, DOI 10.1172/JCI106251
[3]  
BRUNETTE MG, 1972, PEDIATRICS, V50, P702
[4]   LEIGHS SUBACUTE NECROTIZING ENCEPHALOPATHY - CLINICAL AND BIOCHEMICAL STUDY WITH SPECIAL REFERENCE TO THERAPY WITH LIPOATE [J].
CLAYTON, BE ;
DOBBS, RH ;
PATRICK, AD .
ARCHIVES OF DISEASE IN CHILDHOOD, 1967, 42 (225) :467-+
[5]   EXPERIENCE WITH PHOSPHORYL TRANSFERASE INHIBITION IN SUBACUTE NECROTIZING ENCEPHALOMYELOPATHY [J].
COOPER, JR ;
PINCUS, JH ;
ITOKAWA, Y ;
PIROS, K .
NEW ENGLAND JOURNAL OF MEDICINE, 1970, 283 (15) :793-&
[6]  
DELVIN E, 1972, PEDIATR RES, V6, P392
[7]   BIOCHEMICAL STUDIES AND THERAPY IN SUBACUTE NECROTIZING ENCEPBALOMYELOPATHY (LEIGHS SYNDROME) [J].
GROVER, WD ;
PATEL, MS ;
AUERBACH, VH .
JOURNAL OF PEDIATRICS, 1972, 81 (01) :39-+
[8]   LEIGHS ENCEPHALOMYELOPATHY - AN INBORN ERROR OF GLUCONEOGENESIS [J].
HOMMES, FA ;
POLMAN, HA ;
REERINK, JD .
ARCHIVES OF DISEASE IN CHILDHOOD, 1968, 43 (230) :423-&
[9]   EFFECT OF THIAMINE TREATMENT ON ACTIVITY OF PYRUVATE DEHYDROGENASE - RELATION TO TREATMENT OF LEIGHS ENCEPHALOMYELOPATHY [J].
HOMMES, FA ;
BERGER, R ;
LUITDEHA.G .
PEDIATRIC RESEARCH, 1973, 7 (07) :616-619