Transient improvement of congenital lactic acidosis in a male infant with pyruvate decarboxylase deficiency treated with dichloroacetate

The Journal of Pediatrics

Volumn 123, Issue 3, 1993, Pages 427-430

  Tóth, Peter P ^a   El Shanti, Hatem ^a   Eivins, Sandra ^a   Rhead, William J ^a   Klein, Jonathan M ^a  

^a UNIVERSITY OF IOWA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

DICHLOROACETIC ACID; PYRUVATE DECARBOXYLASE;

ARTICLE; CASE REPORT; COMA; ENZYME DEFICIENCY; HUMAN; INTRAVENOUS DRUG ADMINISTRATION; LACTIC ACIDOSIS; MALE; NEWBORN; ORAL DRUG ADMINISTRATION; PRIORITY JOURNAL;

ACIDOSIS, LACTIC; CASE REPORT; DICHLOROACETATE; HUMAN; INFANT, NEWBORN; MALE; PYRUVATE DECARBOXYLASE; PYRUVATE METABOLISM, INBORN ERRORS;

EID: 0027170619     PISSN: 00223476     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0022-3476(05)81751-2     Document Type: Article

References (13)

1. Regulation of mammalian pyruvate dehydrogenase complex by a phosphorylation-dephosphorylation cycle
2. α-Keto acid dehydrogenase complexes. X. Regulation of the activity of the pyruvate dehydrogenase complex from beef kidney mitochondria by phosphorylation and dephosphorylation
3. Absence of pyruvate decarboxylase activity in man: a cause of congenital lactic acidosis in man
4. Partial pyruvate decarboxylase deficiency with profound lactic acidosis and hyperammonemia: responses to dichloroacetate and benzoate
5. Lacticacidemia due to pyruvate dehydrogenase deficiency, with evidence of protein polymorphism in the α-subunit of the enzyme
6. 1 component of the complex
7. Mechanism of activation of pyruvate dehydrogenase by dichloroacetate and other halogenated carboxylic acids
8. Treatment of chronic congenital lactic acidosis by oral administration of dichloroacetate
9. Treatment of lactic acidosis with dichloroacetate
10. Pyruvate dehydrogenase complex activity in normal and deficient fibroblasts
11. Systemic deficiency of the first component of the pyruvate dehydrogenase complex
12. Lactic acidemia, neurologic deterioration and carbohydrate dependence in a girl with dihydrolipoyl dehydrogenase deficiency