DIHYDROLIPOYL DEHYDROGENASE-DEFICIENCY - A THERAPEUTIC TRIAL WITH BRANCHED-CHAIN AMINO-ACID RESTRICTION

被引:22
作者
SAKAGUCHI, Y
YOSHINO, M
ARAMAKI, S
YOSHIDA, I
YAMASHITA, F
KUHARA, T
MATSUMOTO, I
HAYASHI, T
机构
[1] KURUME UNIV,SCH MED,DEPT PEDIAT,67 ASAHI MACHI,KURUME,FUKUOKA 830,JAPAN
[2] KURUME UNIV,SCH MED,MED MASS SPECTROMETRY RES INST,KURUME,FUKUOKA 830,JAPAN
[3] NATL CTR NERVOUS MENTAL & MUSCULAR DISORDERS,KODAIRA,TOKYO 187,JAPAN
关键词
D O I
10.1007/BF00439399
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
引用
收藏
页码:271 / 274
页数:4
相关论文
共 14 条
[1]  
BLASS J P, 1970, Journal of Clinical Investigation, V49, P423, DOI 10.1172/JCI106251
[2]   INHERITED DEFECT AFFECTING TRICARBOXYLIC ACID CYCLE IN A PATIENT WITH CONGENITAL LACTIC ACIDOSIS [J].
BLASS, JP ;
SCHULMAN, JD ;
YOUNG, DS ;
HOM, E .
JOURNAL OF CLINICAL INVESTIGATION, 1972, 51 (07) :1845-&
[3]  
HAYASHI T, 1982, ANAL BIOCHEM, V122, P173, DOI 10.1016/0003-2697(82)90267-6
[4]   LACTIC DEHYDROGENASE OF MUSCLE [J].
KORNBERG, A .
METHODS IN ENZYMOLOGY, 1955, 1 :441-443
[5]  
KUHARA T, 1983, CLIN CHIM ACTA, V133, P133
[6]   LIPOAMIDE DEHYDROGENASE-DEFICIENCY WITH PRIMARY LACTIC-ACIDOSIS - FAVORABLE RESPONSE TO TREATMENT WITH ORAL LIPOIC ACID [J].
MATALON, R ;
STUMPF, DA ;
MICHALS, K ;
HART, RD ;
PARKS, JK ;
GOODMAN, SI .
JOURNAL OF PEDIATRICS, 1984, 104 (01) :65-69
[7]   CONGENITAL LACTIC-ACIDOSIS, ALPHA-KETOGLUTARIC ACIDURIA AND VARIANT FORM OF MAPLE SYRUP URINE DISEASE DUE TO A SINGLE ENZYME DEFECT - DIHYDROLIPOYL DEHYDROGENASE-DEFICIENCY [J].
MUNNICH, A ;
SAUDUBRAY, JM ;
TAYLOR, J ;
CHARPENTIER, C ;
MARSAC, C ;
ROCCHICCIOLI, F ;
AMEDEEMANESME, O ;
COUDE, FX ;
FREZAL, J ;
ROBINSON, BH .
ACTA PAEDIATRICA SCANDINAVICA, 1982, 71 (01) :167-171
[8]  
Reed L.J., 1966, METHOD ENZYMOL, V9, P247
[9]   DEFICIENCY OF DIHYDROLIPOYL DEHYDROGENASE (A COMPONENT OF PYRUVATE AND ALPHA-KETOGLUTARATE DEHYDROGENASE COMPLEXES) - CAUSE OF CONGENITAL CHRONIC LACTIC-ACIDOSIS IN INFANCY [J].
ROBINSON, BH ;
TAYLOR, J ;
SHERWOOD, WG .
PEDIATRIC RESEARCH, 1977, 11 (12) :1198-1202
[10]   LACTIC ACIDEMIA, NEUROLOGIC DETERIORATION AND CARBOHYDRATE DEPENDENCE IN A GIRL WITH DIHYDROLIPOYL DEHYDROGENASE-DEFICIENCY [J].
ROBINSON, BH ;
TAYLOR, J ;
KAHLER, SG ;
KIRKMAN, HN .
EUROPEAN JOURNAL OF PEDIATRICS, 1981, 136 (01) :35-39