Octenylsuccinic aciduria in children fed protein-hydrolysate formulas containing modified cornstarch

R. I. Kelley

Research output: Contribution to journalArticle

Abstract

The excretion of 2-(2′-octenyl)succinic acid (OSA) and several metabolites of OSA was studied by gas chromatography/mass spectrometry in 17 infants and children fed one of three proprietary elemental or protein-hydrolysate formulas that use OSA-modified cornstarch as an emulsifying agent. Variable but often large amounts (up to 2500 mg/g creatinine) of the fatty acid-like OSA and its metabolites were found in the urine of these children, and levels of OSA in their blood ranged from 9.5 to 57.9 μmol/L. Apparently secondary abnormalities, such as increased urinary levels of glutaric acid and 2-ketoglutaric acid, were also found in more than half of the urine specimens. The molecular weight and mass fragmentation patterns of the nine compounds associated with the excretion of OSA are consistent with the proposal that OSA is metabolized in human infants and children by a combination of ω-, ω-1-, and β-oxidation steps, similar to the metabolism of another branched-chain fatty acid, valproic acid. The urinary organic acid pattern of children fed elemental formulas containing OSA-modified starch often was dominated by OSA and its metabolites, and in several children the OSA-related changes were mistaken for a primary metabolic disease. Physicians and laboratories evaluating children for suspected metabolic diseases should be aware of the possibility of abnormal organic acid studies associated with OSA-containing formulas.

Original languageEnglish (US)
Pages (from-to)564-569
Number of pages6
JournalPediatric Research
Volume30
Issue number6
StatePublished - 1991

Fingerprint

Protein Hydrolysates
Succinic Acid
Starch
Metabolic Diseases
Emulsifying Agents
Fatty Acids
Urine
Acids
Valproic Acid
Gas Chromatography-Mass Spectrometry
Creatinine
Molecular Weight

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Octenylsuccinic aciduria in children fed protein-hydrolysate formulas containing modified cornstarch. / Kelley, R. I.

In: Pediatric Research, Vol. 30, No. 6, 1991, p. 564-569.

Research output: Contribution to journalArticle

@article{d4f0f41331284e47a8ce940a76a9a4fc,
title = "Octenylsuccinic aciduria in children fed protein-hydrolysate formulas containing modified cornstarch",
abstract = "The excretion of 2-(2′-octenyl)succinic acid (OSA) and several metabolites of OSA was studied by gas chromatography/mass spectrometry in 17 infants and children fed one of three proprietary elemental or protein-hydrolysate formulas that use OSA-modified cornstarch as an emulsifying agent. Variable but often large amounts (up to 2500 mg/g creatinine) of the fatty acid-like OSA and its metabolites were found in the urine of these children, and levels of OSA in their blood ranged from 9.5 to 57.9 μmol/L. Apparently secondary abnormalities, such as increased urinary levels of glutaric acid and 2-ketoglutaric acid, were also found in more than half of the urine specimens. The molecular weight and mass fragmentation patterns of the nine compounds associated with the excretion of OSA are consistent with the proposal that OSA is metabolized in human infants and children by a combination of ω-, ω-1-, and β-oxidation steps, similar to the metabolism of another branched-chain fatty acid, valproic acid. The urinary organic acid pattern of children fed elemental formulas containing OSA-modified starch often was dominated by OSA and its metabolites, and in several children the OSA-related changes were mistaken for a primary metabolic disease. Physicians and laboratories evaluating children for suspected metabolic diseases should be aware of the possibility of abnormal organic acid studies associated with OSA-containing formulas.",
author = "Kelley, {R. I.}",
year = "1991",
language = "English (US)",
volume = "30",
pages = "564--569",
journal = "Pediatric Research",
issn = "0031-3998",
publisher = "Lippincott Williams and Wilkins",
number = "6",

}

TY - JOUR

T1 - Octenylsuccinic aciduria in children fed protein-hydrolysate formulas containing modified cornstarch

AU - Kelley, R. I.

PY - 1991

Y1 - 1991

N2 - The excretion of 2-(2′-octenyl)succinic acid (OSA) and several metabolites of OSA was studied by gas chromatography/mass spectrometry in 17 infants and children fed one of three proprietary elemental or protein-hydrolysate formulas that use OSA-modified cornstarch as an emulsifying agent. Variable but often large amounts (up to 2500 mg/g creatinine) of the fatty acid-like OSA and its metabolites were found in the urine of these children, and levels of OSA in their blood ranged from 9.5 to 57.9 μmol/L. Apparently secondary abnormalities, such as increased urinary levels of glutaric acid and 2-ketoglutaric acid, were also found in more than half of the urine specimens. The molecular weight and mass fragmentation patterns of the nine compounds associated with the excretion of OSA are consistent with the proposal that OSA is metabolized in human infants and children by a combination of ω-, ω-1-, and β-oxidation steps, similar to the metabolism of another branched-chain fatty acid, valproic acid. The urinary organic acid pattern of children fed elemental formulas containing OSA-modified starch often was dominated by OSA and its metabolites, and in several children the OSA-related changes were mistaken for a primary metabolic disease. Physicians and laboratories evaluating children for suspected metabolic diseases should be aware of the possibility of abnormal organic acid studies associated with OSA-containing formulas.

AB - The excretion of 2-(2′-octenyl)succinic acid (OSA) and several metabolites of OSA was studied by gas chromatography/mass spectrometry in 17 infants and children fed one of three proprietary elemental or protein-hydrolysate formulas that use OSA-modified cornstarch as an emulsifying agent. Variable but often large amounts (up to 2500 mg/g creatinine) of the fatty acid-like OSA and its metabolites were found in the urine of these children, and levels of OSA in their blood ranged from 9.5 to 57.9 μmol/L. Apparently secondary abnormalities, such as increased urinary levels of glutaric acid and 2-ketoglutaric acid, were also found in more than half of the urine specimens. The molecular weight and mass fragmentation patterns of the nine compounds associated with the excretion of OSA are consistent with the proposal that OSA is metabolized in human infants and children by a combination of ω-, ω-1-, and β-oxidation steps, similar to the metabolism of another branched-chain fatty acid, valproic acid. The urinary organic acid pattern of children fed elemental formulas containing OSA-modified starch often was dominated by OSA and its metabolites, and in several children the OSA-related changes were mistaken for a primary metabolic disease. Physicians and laboratories evaluating children for suspected metabolic diseases should be aware of the possibility of abnormal organic acid studies associated with OSA-containing formulas.

UR - http://www.scopus.com/inward/record.url?scp=0026332794&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0026332794&partnerID=8YFLogxK

M3 - Article

C2 - 1805153

AN - SCOPUS:0026332794

VL - 30

SP - 564

EP - 569

JO - Pediatric Research

JF - Pediatric Research

SN - 0031-3998

IS - 6

ER -