INVESTIGATION OF METABOLIC PATTERN IN MAPLE SYRUP URINE DISEASE BY MEANS OF GLASS CAPILLARY GAS-CHROMATOGRAPHY AND MASS-SPECTROMETRY

被引：47

作者：

JAKOBS, C

SOLEM, E

EK, J

HALVORSEN, K

JELLUM, E

机构：

[1] RIKS HOSP OSLO,INST CLIN BIOCHEM,OSLO,NORWAY

[2] RIKS HOSP OSLO,DEPT PEDIAT,OSLO,NORWAY

[3] AKERSHUS CENT HOSP,NORDBYHAGEN,NORWAY

来源：

JOURNAL OF CHROMATOGRAPHY | 1977年 / 143卷 / 01期

关键词：

D O I：

10.1016/S0378-4347(00)81324-9

中图分类号：

O65 [分析化学];

学科分类号：

070302 ; 081704 ;

摘要：

引用

收藏

页码：31 / 38

页数：8

相关论文

共 16 条

[1] AUERBACH VH, 1973, INBORN ERRORS METABO, P337
[2] DANCIS J, 1960, PEDIATRICS, V25, P72
[3] ENZYME-ACTIVITY IN CLASSICAL AND VARIANT FORMS OF MAPLE SYRUP URINE DISEASE
DANCIS, J
COX, RP
SNYDERMAN, SE
HUTZLER, J
[J]. JOURNAL OF PEDIATRICS, 1972, 81 (02) : 312 - +
[4] FURTHER OBSERVATIONS ON BIOCHEMICAL LESION IN MAPLE SYRUP URINE DISEASE
DREYFUS, PM
PRENSKY, AL
[J]. NATURE, 1967, 214 (5085) : 276 - &
[5] GAS-CHROMATOGRAPHIC DIAGNOSIS OF INTERMITTENT MAPLE SYRUP URINE DISEASE (BRANCH-CHAIN KETOACIDURIA)
GOMPERTZ, D
DRAFFAN, GH
[J]. CLINICA CHIMICA ACTA, 1972, 40 (01) : 5 - &
[6] GREER MELVIN, 1967, BIO CHEM MED, V1, P87, DOI 10.1016/0006-2944(67)90026-9
[7] CONFIGURATION OF ALLOISOLEUCINE PRESENT IN MAPLE SYRUP URINE DISEASE PLASMA
HALPERN, B
POLLOCK, GE
[J]. BIOCHEMICAL MEDICINE, 1970, 4 (3-4): : 352 - &
[8] BRANCHED-CHAIN ALPHA-KETO ACIDS ISOLATED AS OXIME DERIVATIVES - RELATIONSHIP TO CORRESPONDING HYDROXY-ACIDS AND AMINO-ACIDS IN MAPLE SYRUP URINE DISEASE
LANCASTER, G
MAMER, OA
SCRIVER, CR
[J]. METABOLISM-CLINICAL AND EXPERIMENTAL, 1974, 23 (03): : 257 - 265
[9] INCREASED URINARY-EXCRETION OF 3-HYDROXYISOVALERIC ACID IN PATIENTS WITH KETOACIDOSIS
LANDAAS, S
[J]. CLINICA CHIMICA ACTA, 1974, 54 (01) : 39 - 46
[10] ACCUMULATION OF 3-HYDROXYISOBUTYRIC ACID, 2-METHYL-3-HYDROXYBUTYRIC ACID AND 3-HYDROXYISOVALERIC ACID IN KETOACIDOSIS
LANDAAS, S
[J]. CLINICA CHIMICA ACTA, 1975, 64 (02) : 143 - 154