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  • Severe illness caused by the products of bacterial metabolism in a child with a short gut.

Severe illness caused by the products of bacterial metabolism in a child with a short gut.

European journal of pediatrics (1985-05-01)
E Haan, G Brown, A Bankier, D Mitchell, S Hunt, J Blakey, G Barnes
ABSTRACT

An 8-year-old boy with a short gut had six episodes of metabolic acidosis and neurological dysfunction over a 1 month period. The neurological features consisted of a depressed conscious state, confusion, aggressive behaviour, slurred speech and ataxia. The organic acid profile of urine demonstrated increased amounts of lactic, 3-hydroxypropionic, 3-hydroxyisobutyric, 2-hydroxyisocaproic, phenyllactic, 4-hydroxyphenylacetic and 4-hydroxyphenyllactic acids. Of the lactic acid 99% was D-lactic acid. The anaerobic gut flora consisted almost entirely of Lactobacilli in unusually large numbers. A course of vancomycin prevented further episodes. A urinary organic acid profile may be diagnostic when a person with a short gut develops metabolic acidosis or an unusual encephalopathy and bacterial metabolites should be considered in other patients with unusual combinations of organic acids in the urine.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
2-Hydroxyisocaproic acid, 99%
Sigma-Aldrich
(S)-(−)-2-Hydroxyisocaproic acid, 98%