Abstract
Protein-losing enteropathy occurs in up to 10% of patients following the modified Fontan procedure. Treatment is still controversial. We describe a female adolescent who developed protein-losing enteropathy 4 years after a modified Fontan procedure. Treatment with oral prednisone attenuated the protein loss with subsequent normalization of her serum total protein and albumin levels. Discontinuation of prednisone therapy was associated with relapse, which was again treated successfully with low-dose oral prednisone. Small bowel biopsy-proved diagnosis with improvement, relapse, and improvement again are documented, as are other useful laboratory findings.
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References
Driscoll DJ, Offord KP, Feldt RH, et al (1992) Five- to fifteen-year follow-up after Fontan operation.Circulation 85:469–496
Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia.Thorax 26:240–248
Moodie D, Ritter D, Tajik AJ, O'Fallon W (1984) Long-term follow-up in the unoperated univentricular heart.Am J Cardiol 53: 1124–1128
Rothman A, Snyder J (1991) Protein-losing enteropathy following the Fontan operation: resolution with prednisone therapy.Am Heart J 121:618–619
Rychik J, Piccoli DA, Barber G (1991) Usefulness of corticosteroid therapy for proten-losing enteropathy after the Fontan procedure.Am J Cardiol 68:819–821
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Zellers, T.M., Brown, K. Protein-Losing enteropathy after the modified fontan operation: Oral prednisone treatment with biopsy and laboratory proved improvement. Pediatr Cardiol 17, 115–117 (1996). https://doi.org/10.1007/BF02505095
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DOI: https://doi.org/10.1007/BF02505095