EFFECTS OF THIAMINE IN A PATIENT WITH A VARIANT FORM OF BRANCHED-CHAIN KETOACIDURIA

被引：17

作者：

DURAN, M

TIELENS, AGM

WADMAN, SK

STIGTER, JCM

KLEIJER, WJ

机构：

[1] ST FRANCISCUS GASTHUIS,ROTTERDAM,NETHERLANDS

[2] ERASMUS UNIV,DEPT CELL BIOL & GENET,ROTTERDAM,NETHERLANDS

来源：

ACTA PAEDIATRICA SCANDINAVICA | 1978年 / 67卷 / 03期

关键词：

D O I：

10.1111/j.1651-2227.1978.tb16336.x

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

引用

页码：367 / 372

页数：6

共 16 条

[1]

BIERVLIET JP, 1974, LANCET, V2, P1518

[2] INTERMITTENT BRANCHED-CHAIN KETONURIA - VARIANT OF MAPLE-SYRUP-URINE DISEASE [J].

DANCIS, J ;

HUTZLER, J ;

ROKKONES, T .

NEW ENGLAND JOURNAL OF MEDICINE, 1967, 276 (02) :84-&

[3] ENZYME-ACTIVITY IN CLASSICAL AND VARIANT FORMS OF MAPLE SYRUP URINE DISEASE [J].

DANCIS, J ;

COX, RP ;

SNYDERMAN, SE ;

HUTZLER, J .

JOURNAL OF PEDIATRICS, 1972, 81 (02) :312-+

[4] THIAMINE INCREASES SPECIFIC ACTIVITY OF HUMAN LIVER BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE [J].

DANNER, DJ ;

DAVIDSON, ED ;

ELSAS, LJ .

NATURE, 1975, 254 (5500) :529-530

[5]

ELSAS L, 1972, Clinical Research, V20, P43

[6] MAPLE SYRUP URINE DISEASE - COENZYME FUNCTION AND PRENATAL MONITORING [J].

ELSAS, LJ ;

PRIEST, JH ;

WHEELER, FB ;

DANNER, DJ ;

PASK, BA .

METABOLISM-CLINICAL AND EXPERIMENTAL, 1974, 23 (06) :569-579

[7]

FISCHER MH, 1971, PEDIATRICS, V48, P795

[8] MILD VARIANT OF MAPLE SYRUP URINE DISEASE [J].

KODAMA, S ;

SEKI, A ;

HANABUSA, M ;

MORISITA, Y ;

SAKURAI, T ;

MATSUO, T .

EUROPEAN JOURNAL OF PEDIATRICS, 1976, 124 (01) :31-36

[9] BRANCHED-CHAIN ALPHA-KETO ACIDS ISOLATED AS OXIME DERIVATIVES - RELATIONSHIP TO CORRESPONDING HYDROXY-ACIDS AND AMINO-ACIDS IN MAPLE SYRUP URINE DISEASE [J].

LANCASTER, G ;

MAMER, OA ;

SCRIVER, CR .

METABOLISM-CLINICAL AND EXPERIMENTAL, 1974, 23 (03) :257-265

[10] QUANTITATIVE GAS-CHROMATOGRAPHY AND SINGLE-ION DETECTION OF ALIPHATIC ALPHA-KETO ACIDS FROM URINE AS THEIR O-TRIMETHYLSILYLQUINOXALINOL DERIVATIVES [J].

LANGENBECK, U ;

HOINOWSKI, A ;

MANTEL, K ;

MOHRING, HU .

JOURNAL OF CHROMATOGRAPHY, 1977, 143 (01) :39-50

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