TRANSIENT IMPROVEMENT OF CONGENITAL LACTIC-ACIDOSIS IN A MALE INFANT WITH PYRUVATE DECARBOXYLASE DEFICIENCY TREATED WITH DICHLOROACETATE

Authors
TOTH PP ELSHANTI H EIVINS S RHEAD WJ KLEIN JM
Citation
Pp. Toth et al., TRANSIENT IMPROVEMENT OF CONGENITAL LACTIC-ACIDOSIS IN A MALE INFANT WITH PYRUVATE DECARBOXYLASE DEFICIENCY TREATED WITH DICHLOROACETATE, The Journal of pediatrics, 123(3), 1993, pp. 427-430
Citations number
13
Categorie Soggetti
Pediatrics
Journal title
The Journal of pediatrics → ACNP
ISSN journal
00223476
Volume
123
Issue
3
Year of publication
1993
Pages
427 - 430
Database
ISI
SICI code
0022-3476(1993)123:3<427:TIOCLI>2.0.ZU;2-P
Abstract
A comatose male newborn infant with congenital lactic acidosis caused by pyruvate decarboxylase deficiency was treated with dichloroacetate (DCA), which stimulated an 88% drop in serum lactate concentration and reversed his coma. The response to DCA was temporary and the lactic a cidosis worsened until his death, but DCA may confer more lasting bene fit in less severely affected infants.