Corticosteroid-responsive enteropathy of infancy.

Nineteen American infants aged less than three months developed persistent diarrhoea, acidosis, hypoalbuminaemia, and malnutrition, without evidence of enteric pathogens. Symptoms began 11-59 days before admission to the University of North Carolina Children's Hospital, and infants were fed semielemental formula. Despite further treatment with amino acid-based formula by continuous nasogastric infusion, diarrhoea persisted. Endoscopic biopsies showed inflammation in the stomach, duodenum, and/or colon. A trial of intravenous corticosteroids was initiated in 14 infants. Corticosteroids were associated with rapid resolution of diarrhoea (duration after corticosteroids = 3.8 +/- 1.7 days [mean +/- SD]). In contrast, five infants with identical history were not treated with corticosteroids. In three infants, diarrhoea lasted for 92-147 days versus 31 +/- 3 total days in the treated group. In the other two infants, diarrhoea worsened after discharge, but were treated later with corticosteroids, with rapid resolution. Corticosteroids were uneventfully weaned over a four-month period. The results suggest that a trial of corticosteroids in infants with unresponsive persistent diarrhoea of unknown origin is beneficial and deserves prospective evaluation.