PERITONEAL-DIALYSIS IN MAPLE-SYRUP-URINE DISEASE - STUDIES ON BRANCHED-CHAIN AMINO AND KETO ACIDS

被引：23
|
作者：
WENDEL, U [1 ]
BECKER, K [1 ]
PRZYREMBEL, H [1 ]
BULLA, M [1 ]
MANEGOLD, C [1 ]
MENCHHOINOWSKI, A [1 ]
LANGENBECK, U [1 ]
机构：
[1] UNIV COLOGNE,CHILDRENS HOSP,D-5000 COLOGNE 41,FED REP GER
来源：
EUROPEAN JOURNAL OF PEDIATRICS | 1980年 / 134卷 / 01期
关键词：
D O I：
10.1007/BF00442404
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
引用
收藏
页码：57 / 63
页数：7
相关论文
共 50 条
  • [1] BRANCHED-CHAIN ALPHA-KETO ACIDS FOR THE DIAGNOSIS OF MAPLE-SYRUP-URINE DISEASE
    PARSONS, H
    FUNG, E
    SNYDER, FF
    NEW ENGLAND JOURNAL OF MEDICINE, 1987, 316 (15): : 951 - 951
  • [2] Metabolism of branched-chain amino acids in maple syrup urine disease
    P. Schadewaldt
    U. Wendel
    European Journal of Pediatrics, 1997, 156 : S62 - S66
  • [3] Metabolism of branched-chain amino acids in maple syrup urine disease
    Schadewaldt, P
    Wendel, U
    EUROPEAN JOURNAL OF PEDIATRICS, 1997, 156 (Suppl 1) : S62 - S66
  • [4] Pathophysiology of maple syrup urine disease: Focus on the neurotoxic role of the accumulated branched-chain amino acids and branched-chain ?-keto acids
    Amaral, Alexandre Umpierrez
    Wajner, Moacir
    NEUROCHEMISTRY INTERNATIONAL, 2022, 157
  • [5] INTERMITTENT BRANCHED-CHAIN KETONURIA - VARIANT OF MAPLE-SYRUP-URINE DISEASE
    DANCIS, J
    HUTZLER, J
    ROKKONES, T
    NEW ENGLAND JOURNAL OF MEDICINE, 1967, 276 (02): : 84 - &
  • [6] CORRELATIONS BETWEEN BRANCHED-CHAIN AMINO-ACIDS AND BRANCHED-CHAIN ALPHA-KETO ACIDS IN BLOOD IN MAPLE SYRUP URINE DISEASE
    LANGENBECK, U
    WENDEL, U
    MENCHHOINOWSKI, A
    KUSCHEL, D
    BECKER, K
    PRZYREMBEL, H
    BREMER, HJ
    CLINICA CHIMICA ACTA, 1978, 88 (02) : 283 - 291
  • [7] EXCHANGE-TRANSFUSION IN ACUTE EPISODES OF MAPLE SYRUP URINE DISEASE - STUDIES ON BRANCHED-CHAIN AMINO AND KETO ACIDS
    WENDEL, U
    LANGENBECK, U
    LOMBECK, I
    BREMER, HJ
    EUROPEAN JOURNAL OF PEDIATRICS, 1982, 138 (04) : 293 - 296
  • [8] MAPLE SYRUP URINE DISEASE - BRANCHED-CHAIN KETO ACID DECARBOXYLATION IN FIBROBLASTS AS MEASURED WITH AMINO-ACIDS AND KETO ACIDS
    DANCIS, J
    HUTZLER, J
    COX, RP
    AMERICAN JOURNAL OF HUMAN GENETICS, 1977, 29 (03) : 272 - 279
  • [9] MAPLE SYRUP URINE DISEASE - BRANCHED-CHAIN KETO-ACIDURIA
    DANCIS, J
    LEVITZ, M
    WESTALL, RG
    PEDIATRICS, 1960, 25 (01) : 72 - 79
  • [10] DEFINING BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE-DEFICIENCY IN MAPLE-SYRUP-URINE DISEASE
    FISHER, CW
    LAU, KS
    GRIFFIN, TA
    CHUANG, JL
    HU, CWC
    COX, RP
    CHUANG, DT
    FASEB JOURNAL, 1988, 2 (05): : A1547 - A1547
12345