Emergency Treatments View Full Text


Ontology type: schema:Chapter     


Chapter Info

DATE

1995

AUTHORS

H. Ogier de Baulny , J.-M. Saudubray

ABSTRACT

The emergency treatment of children suspected of having an inborn error of metabolism implies three simultaneous actions: collecting all samples necessary for the diagnosis, deciding on adequate treatment, and organizing clinical and biochemical supervision. As already stated in the first chapter (Saudubray et al.), management depends on the physiopathology involved. This chapter focuses on the emergency treatment of those disorders which lead to an acute intoxication secondary to accumulation of toxic compounds proximal to the metabolic block. Mainly, they comprise branched-chain organic acidurias (BCOA), urea cycle defects (UCD), and some β-oxidation defects. Age of onset (neonatal versus late-onset) and intercurrent decompensations are other aspects which influence management. More... »

PAGES

47-55

Book

TITLE

Inborn Metabolic Diseases

ISBN

978-3-662-03149-0
978-3-662-03147-6

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/978-3-662-03147-6_3

DOI

http://dx.doi.org/10.1007/978-3-662-03147-6_3

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1019666616


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