title: 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: Department of Pediatrics and Child Health, Kurume University School of Medicine, Kurume, Japan.
authors: Tatsuki
authors: T
authors: Mizuochi
authors: Kenji
authors: K
authors: Suda
authors: Yoshitaka
authors: Y
authors: Seki
authors: Tadahiro
authors: T
authors: Yanagi
authors: Hironaga
authors: H
authors: Yoshimoto
authors: Yoshiyuki
authors: Y
authors: Kudo
authors: Motofumi
authors: M
authors: Iemura
authors: Ken
authors: K
authors: Tanikawa
authors: Toyojiro
authors: T
authors: Matsuishi
mesh_terms: D002648:Child; D004232:Diuretics / Q000627:therapeutic use*; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects; D005665:Furosemide / Q000627:therapeutic use*; D006330:Heart Defects, Congenital / Q000601:surgery; D006801:Humans; D009634:Noonan Syndrome / Q000150:complications*; D011504:Protein-Losing Enteropathies / Q000188:drug therapy* / Q000209:etiology; D013148:Spironolactone / Q000627:therapeutic use*
chemical_list: D004232:Diuretics; D013148:Spironolactone; D005665:Furosemide
keywords: Noonan syndrome; diuretic; protein-losing enteropathy; venous pressure