Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15 years Experience

Molecular Genetics and Metabolism

Volumn 116, Issue 4, 2015, Pages 260-268

  Roe, Charles R ^a,c   Brunengraber, Henri ^b  

^a UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

^b CASE WESTERN RESERVE UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c BAYLOR UNIVERSITY MEDICAL CENTER   (United States)

Author keywords

Anaplerosis; Fat oxidation disorders; Ketogenic diet therapy; Triheptanoin

Indexed keywords

CARNITINE; TRIHEPTANOIN; ACYLCARNITINE; CITRIC ACID; FATTY ACID; LACTIC ACID; MALIC ACID; MALIC ACID DERIVATIVE; SUCCINIC ACID; TRIACYLGLYCEROL;

ADOLESCENT; ADULT; ARTICLE; BLOOD CHEMISTRY; DIET THERAPY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DRUG DOSE INCREASE; DRUG SAFETY; FEMALE; HUMAN; HYPOTHESIS; INFANT; LONG CHAIN MITOCHONDRIAL BETA OXIDATION DISORDER; MAJOR CLINICAL STUDY; NEWBORN SCREENING; PRIORITY JOURNAL; QUANTITATIVE ANALYSIS; TREATMENT OUTCOME; ANALOGS AND DERIVATIVES; BLOOD; CHILD; LIPID METABOLISM, INBORN ERRORS; MALE; METABOLISM; MIDDLE AGED; MITOCHONDRIAL DISEASES; MORTALITY; NEWBORN; ORAL DRUG ADMINISTRATION; OXIDATION REDUCTION REACTION; PRESCHOOL CHILD; SURVIVAL ANALYSIS; URINE;

ADMINISTRATION, ORAL; ADOLESCENT; ADULT; CARNITINE; CHILD; CHILD, PRESCHOOL; CITRIC ACID; FATTY ACIDS; FEMALE; HUMANS; INFANT; INFANT, NEWBORN; LACTIC ACID; LIPID METABOLISM, INBORN ERRORS; MALATES; MALE; MIDDLE AGED; MITOCHONDRIAL DISEASES; OXIDATION-REDUCTION; SUCCINIC ACID; SURVIVAL ANALYSIS; TREATMENT OUTCOME; TRIGLYCERIDES;

EID: 84961575911     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2015.10.005     Document Type: Article

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