A defect in the transport of long-chain fatty acids associated with acute liver failure

被引:41
作者
Al Odaib, A
Shneider, BL
Bennett, MJ
Pober, BR
Reyes-Mugica, M
Friedman, AL
Suchy, FJ
Rinaldo, P
机构
[1] Yale Univ, Sch Med, Dept Genet, New Haven, CT 06510 USA
[2] Yale Univ, Sch Med, Dept Pediat, New Haven, CT 06510 USA
[3] Yale Univ, Sch Med, Dept Pathol, New Haven, CT 06510 USA
[4] Yale Univ, Sch Med, Dept Surg, New Haven, CT 06510 USA
[5] Univ Texas, SW Med Ctr, Dept Pathol, Dallas, TX USA
[6] Univ Texas, SW Med Ctr, Dept Pediat, Dallas, TX USA
关键词
D O I
10.1056/NEJM199812103392405
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
[No abstract available]
引用
收藏
页码:1752 / 1757
页数:6
相关论文
共 30 条
[1]  
ABUMRAD NA, 1993, J BIOL CHEM, V268, P17665
[2]   FULMINANT-HEPATITIS ASSOCIATED WITH CENTRILOBULAR HEPATIC-NECROSIS IN YOUNG-CHILDREN [J].
ALONSO, EM ;
SOKOL, RJ ;
HART, J ;
TYSON, RW ;
NARKEWICZ, MR ;
WHITTINGTON, PF .
JOURNAL OF PEDIATRICS, 1995, 127 (06) :888-894
[3]   Mitochondrial short-chain L-3-hydroxyacyl-coenzyme a dehydrogenase deficiency: A new defect of fatty acid oxidation [J].
Bennett, MJ ;
Weinberger, MJ ;
Kobori, JA ;
Rinaldo, P ;
Burlina, AB .
PEDIATRIC RESEARCH, 1996, 39 (01) :185-188
[4]   METABOLIC DISEASE AND SUDDEN, UNEXPECTED DEATH IN INFANCY [J].
BENNETT, MJ ;
POWELL, S .
HUMAN PATHOLOGY, 1994, 25 (08) :742-746
[5]  
Berk PD, 1996, P SOC EXP BIOL MED, V212, P1
[6]   Retrospective biochemical screening of fatty acid oxidation disorders in postmortem livers of 418 cases of sudden death in the first year of life [J].
Boles, RG ;
Buck, EA ;
Blitzer, MG ;
Platt, MS ;
Cowan, TM ;
Martin, SK ;
Yoon, HR ;
Madsen, JA ;
Reyes-Mugica, M ;
Rinaldo, P .
JOURNAL OF PEDIATRICS, 1998, 132 (06) :924-933
[7]   BIOCHEMICAL-DIAGNOSIS OF FATTY-ACID OXIDATION DISORDERS BY METABOLITE ANALYSIS OF POSTMORTEM LIVER [J].
BOLES, RG ;
MARTIN, SK ;
BLITZER, MG ;
RINALDO, P .
HUMAN PATHOLOGY, 1994, 25 (08) :735-741
[8]   2-DEOXY-D-GLUCOSE UPTAKE AND FATTY-ACID CONTENT IN FIBROBLAST-CULTURES FROM CHILDREN WITH SYNDROMIC PAUCITY OF INTERLOBULAR BILE-DUCTS (ALAGILLE SYNDROME) [J].
COUTURIER, M ;
LEMONNIER, F .
JOURNAL OF INHERITED METABOLIC DISEASE, 1991, 14 (02) :215-227
[9]   Mammalian mitochondrial beta-oxidation [J].
Eaton, S ;
Bartlett, K ;
Pourfarzam, M .
BIOCHEMICAL JOURNAL, 1996, 320 :345-357
[10]  
Fitscher BA, 1996, P SOC EXP BIOL MED, V212, P15