Limitations of 3-phenylpropionylglycine in early screening for medium-chain acyl-coenzyme A dehydrogenase deficiency

J Pediatr. 1993 Jan;122(1):100-3. doi: 10.1016/s0022-3476(05)83499-7.

Abstract

Screening for medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency by urinary 3-phenylpropionylglycine may not be reliable in early infancy because young infants are not colonized with adult-type colonic flora. In this study we delineated the microbes that produce 3-phenylpropionic acid, the precursor of 3-phenylpropionylglycine. We found that the use of some antibiotics may alter gut flora, thereby confounding this method of screening for MCAD deficiency.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acyl-CoA Dehydrogenase
  • Adult
  • Anti-Bacterial Agents / administration & dosage
  • Anti-Bacterial Agents / pharmacology
  • Bacteria, Anaerobic / drug effects
  • Bacteria, Anaerobic / metabolism*
  • Caproates / metabolism
  • Child
  • Clostridium / metabolism
  • Fatty Acid Desaturases / deficiency*
  • Glycine / analogs & derivatives*
  • Glycine / biosynthesis
  • Glycine / urine
  • Humans
  • Infant
  • Phenylalanine / metabolism
  • Phenylpropionates / metabolism

Substances

  • Anti-Bacterial Agents
  • Caproates
  • Phenylpropionates
  • hexanoic acid
  • 3-phenylpropionylglycine
  • N-caproylglycine
  • Phenylalanine
  • 3-phenylpropionic acid
  • Fatty Acid Desaturases
  • Acyl-CoA Dehydrogenase
  • Glycine