MEDIUM-CHAIN ACYL-COA DEHYDROGENASE-DEFICIENCY - METABOLIC EFFECTS AND THERAPEUTIC EFFICACY OF LONG-TERM L-CARNITINE SUPPLEMENTATION

被引:28
作者
TREEM, WR
STANLEY, CA
GOODMAN, SI
机构
[1] UNIV PENN,SCH MED,DEPT PEDIAT,PHILADELPHIA,PA 19104
[2] UNIV COLORADO,SCH MED,DEPT PEDIAT,DENVER,CO 80262
关键词
D O I
10.1007/BF01800712
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
引用
收藏
页码:112 / 119
页数:8
相关论文
共 20 条
  • [1] MEDIUM-CHAIN ACYL-COA DEHYDROGENASE-DEFICIENCY IN 2 SIBLINGS WITH A REYE-LIKE SYNDROME
    BOUGNERES, PF
    ROCCHICCIOLI, F
    KOLVRAA, S
    HADCHOUEL, M
    LALAUKERALY, J
    CHAUSSAIN, JL
    WADMAN, SK
    GREGERSEN, N
    [J]. JOURNAL OF PEDIATRICS, 1985, 106 (06) : 918 - 921
  • [2] URINARY-EXCRETION OF L-CARNITINE AND ACYLCARNITINES BY PATIENTS WITH DISORDERS OF ORGANIC-ACID METABOLISM - EVIDENCE FOR SECONDARY INSUFFICIENCY OF L-CARNITINE
    CHALMERS, RA
    ROE, CR
    STACEY, TE
    HOPPEL, CL
    [J]. PEDIATRIC RESEARCH, 1984, 18 (12) : 1325 - 1328
  • [3] COATES PM, 1985, PEDIATR RES, V19, P6711
  • [4] PRIMARY SYSTEMIC CARNITINE DEFICIENCY .2. RENAL HANDLING OF CARNITINE
    ENGEL, AG
    REBOUCHE, CJ
    WILSON, DM
    GLASGOW, AM
    ROMSHE, CA
    CRUSE, RP
    [J]. NEUROLOGY, 1981, 31 (07) : 819 - 825
  • [5] FLUOROMETRIC ASSAY OF ACYL-COA DEHYDROGENASES IN NORMAL AND MUTANT HUMAN-FIBROBLASTS
    FRERMAN, FE
    GOODMAN, SI
    [J]. BIOCHEMICAL MEDICINE, 1985, 33 (01): : 38 - 44
  • [6] MCGARRY JD, 1976, J LIPID RES, V17, P277
  • [7] IDENTIFICATION OF LONG-CHAIN DICARBOXYLIC-ACIDS IN THE SERUM OF 2 PATIENTS WITH REYES-SYNDROME
    NG, KJ
    ANDRESEN, BD
    HILTY, MD
    BIANCHINE, JR
    [J]. JOURNAL OF CHROMATOGRAPHY, 1983, 276 (01): : 1 - 10
  • [8] NOVAK M, 1965, J LIPID RES, V6, P431
  • [9] METABOLIC RESPONSE TO CARNITINE IN METHYLMALONIC ACIDURIA - AN EFFECTIVE STRATEGY FOR ELIMINATION OF PROPIONYL GROUPS
    ROE, CR
    HOPPEL, CL
    STACEY, TE
    CHALMERS, RA
    TRACEY, BM
    MILLINGTON, DS
    [J]. ARCHIVES OF DISEASE IN CHILDHOOD, 1983, 58 (11) : 916 - 920
  • [10] DIAGNOSTIC AND THERAPEUTIC IMPLICATIONS OF MEDIUM-CHAIN ACYLCARNITINES IN THE MEDIUM-CHAIN ACYL-COA DEHYDROGENASE-DEFICIENCY
    ROE, CR
    MILLINGTON, DS
    MALTBY, DA
    BOHAN, TP
    KAHLER, SG
    CHALMERS, RA
    [J]. PEDIATRIC RESEARCH, 1985, 19 (05) : 459 - 466