PROTEIN-LOSING ENTEROPATHY AFTER THE MODIFIED FONTAN OPERATION - ORALPREDNISONE TREATMENT WITH BIOPSY AND LABORATORY PROVED IMPROVEMENT

Protein-losing enteropathy occurs in up to 10% of patients following t he modified Fontan procedure, Treatment is still controversial. We des cribe a female adolescent who developed protein-losing enteropathy 4 y ears after a modified Fontan procedure. Treatment with oral prednisone attenuated the protein loss with subsequent normalization of her seru m total protein and albumin levels. Discontinuation of prednisone ther apy was associated with relapse, which was again treated successfully with low-dose oral prednisone, Small bowel biopsy-proved diagnosis wit h improvement, relapse, and improvement again are documented, as are o ther useful laboratory findings.