Increased excretion of propan-1,3-diol and 3-hydroxypropionic acid apparently caused by abnormal bacterial metabolism in the gut.

Abstract

Three patients who died in infancy showed an unusual urinary organic acid pattern with excessive excretion of 3-hydroxypropionic acid but none of the other metabolites normally associated with propionyl-CoA carboxylase deficiency. Propan-1,3-diol was present in the urine in all three cases. In the two patients examined propionyl-CoA carboxylase activity was not deficient in cultured skin fibroblasts. A fourth patient, also severely ill, showed similar urinary abnormalities. Feeding a medium-chain triglyceride-rich diet to this patient increased the ratio of 3-hydroxypropionic acid to propan-1,3-diol and resulted also in the appearance of malonic acid in the urine. These abnormal metabolites disappeared on the administration of neomycin and presumably were produced by gut bacteria.

• Authors: R J Pollitt, B Fowler, I B Sardharwalla, M A Edwards, R G Gray
• Journal: Clinica chimica acta; international journal of clinical chemistry (Clin Chim Acta) Vol. 169 Issue 2-3 Pg. 151-7 (Nov 16 1987) ISSN: 0009-8981 [Print] Netherlands
• PMID: 3427776 (Publication Type: Case Reports, Journal Article)

Chemical References

• Lactates
• Propylene Glycols
• Lactic Acid
• 1,3-propanediol
• hydracrylic acid

Topics

• Aging (metabolism)
• Bacterial Infections (microbiology, urine)
• Digestive System (microbiology)
• Female
• Gas Chromatography-Mass Spectrometry
• Humans
• Infant
• Infant, Newborn
• Lactates (urine)
• Lactic Acid (analogs & derivatives)
• Male
• Propylene Glycols (urine)