Successful diuretics treatment of protein-losing enteropathy in Noonan syndrome.

Abstract

There are few reports on successful high-dose spironolactone treatment of refractory protein-losing enteropathy (PLE) caused by Fontan procedure. We report successful diuretics treatment with spironolactone and furosemide at standard dose, of refractory PLE in a patient with Noonan syndrome and repaired congenital heart disease. This is the first successful application of diuretics treatment in a patient with refractory PLE without Fontan procedure. This case illustrates that diuretics treatment can be the first-line treatment of PLE regardless of the causative physiology, and can be effective in refractory PLE with Noonan syndrome.

• Authors: Tatsuki Mizuochi, Kenji Suda, Yoshitaka Seki, Tadahiro Yanagi, Hironaga Yoshimoto, Yoshiyuki Kudo, Motofumi Iemura, Ken Tanikawa, Toyojiro Matsuishi
• Journal: Pediatrics international : official journal of the Japan Pediatric Society (Pediatr Int) Vol. 57 Issue 2 Pg. e39-41 (Apr 2015) ISSN: 1442-200X [Electronic] Australia
• PMID: 25868959 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2015 Japan Pediatric Society.

Chemical References

• Diuretics
• Spironolactone
• Furosemide

Topics

• Child
• Diuretics (therapeutic use)
• Female
• Fontan Procedure (adverse effects)
• Furosemide (therapeutic use)
• Heart Defects, Congenital (surgery)
• Humans
• Noonan Syndrome (complications)
• Protein-Losing Enteropathies (drug therapy, etiology)
• Spironolactone (therapeutic use)