title: Oral budesonide as a therapy for protein-losing enteropathy in children after the Fontan operation.
abstract: OBJECTIVE
  Protein-losing enteropathy is a rare complication of the Fontan palliation surgery. Budesonide is an effective treatment option for protein-losing enteropathy. We reviewed our retrospective experience in four patients who were treated with oral budesonide.
  
  
  METHODS
  Four patients with refractory protein-losing enteropathy after the Fontan operation were started on oral budesonide 9 mg/daily. After achieving normal serum albumin the dose was tapered to 3 mg. Response to oral budesonide, side effects, and serum albumin levels before the treatment and at first, fourth, and ninth months of the budesonide course were recorded. Efficacy was measured based on serum albumin levels and clinical symptoms.
  
  
  RESULTS
  Mean pretherapy albumin was 2.25 g/dL (range 1.7 to 2.5 g/dL) and nine months after therapy it was 4.15 g/dL (range 3.9 to 4.4 g/dL) (p < 0.05). All patients had at least a transient improvement in serum albumin levels and clinical findings. Systemic side effects included cushingoid features and oral moniliasis. All patients had improvement in side effects after tapering budesonide to 3 mg. The treatment was terminated in one case as soon as serum albumin level exceeded 3 g/dL. One death occurred from respiratory arrest six months after budesonide discontinuation.
  
  
  CONCLUSIONS
  Budesonide can be used to treat protein-losing enteropathy in selected patients with cardiac diseases.
authors.affiliation: Department of Paediatric Cardiology, Baskent University, Ankara, Turkey.
authors.forename: Hazım Alper
authors.initials: HA
authors.lastname: Gursu
authors.forename: Ilkay
authors.initials: I
authors.lastname: Erdogan
authors.forename: Birgul
authors.initials: B
authors.lastname: Varan
authors.forename: Ayla
authors.initials: A
authors.lastname: Oktay
authors.forename: Figen
authors.initials: F
authors.lastname: Ozcay
authors.forename: Murat
authors.initials: M
authors.lastname: Ozkan
authors.forename: Sait
authors.initials: S
authors.lastname: Aslamaci
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D015415:Biomarkers / Q000097:blood; D019819:Budesonide / Q000008:administration & dosage* / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D005938:Glucocorticoids / Q000008:administration & dosage* / Q000009:adverse effects; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000188:drug therapy*; D011504:Protein-Losing Enteropathies / Q000175:diagnosis / Q000188:drug therapy*; D012189:Retrospective Studies; D012709:Serum Albumin / Q000032:analysis; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; D012709:Serum Albumin; D019819:Budesonide