Hyperammonemia with coma, tachypnea, and respiratory alkalosis developed in a 3-year-old boy with prune-belly syndrome during a urinary tract infection with Proteus mirabilis. Hyperammonemia is thought to have resulted from the production within the massively dilated urinary tract of excessive amounts of ammonia due to bacterial urease, and its subsequent reabsorption into the systemic circulation. The patient rapidly improved following parenteral antibiotic therapy and continuous catheter drainage of the urinary tract.

To our knowledge this is the first reported cluster of O111 infection and only the second caused by non-O157 Shiga toxin-producing E. coli in North America.

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