Use of oral budesonide in the management of protein-losing enteropathy after the Fontan operation.
BACKGROUND
Intestinal inflammation is a component of the pathophysiology of protein-losing enteropathy after the Fontan operation. Oral controlled-release budesonide is 90% metabolized at first pass through the liver, has high enteric anti-inflammatory activity and relatively low systemic effects, and may be an ideal agent for use in treating this disease.


METHODS
Budesonide was administered to 9 patients (4 male) with protein-losing enteropathy after the Fontan operation. The median interval between the Fontan operation and diagnosis of protein-losing enteropathy was 4 years (range, 0.1 to 13.3). Prior interventional therapy included pulmonary artery stent (1), fenestration (3), pacemaker placement (3) and Fontan revision (2). Prior medical therapy included oral prednisone (5), heparin (4), sildenafil (2), infliximab (1), and octreotide (1), all without persistent success. The starting daily dose of budesonide was 9 mg for patients 4 years old or older, and 6 mg for patients less than 4 years of age.


RESULTS
Mean serum albumin level 3 months before starting budesonide was 1.9 g/dL (range, 1 to 2.4 g/dL). Serum albumin level improved in all patients within 6 months of starting budesonide (mean 2.9 g/dL; range, 2.2 to 3.8 g/dL). Albumin levels of 3 g/dL or more were achieved in 8 of 9 patients within a median of 4.3 months (range, 2 to 25). Side effects included Cushingoid features and osteoporosis (3), infection requiring antibiotic treatment (5), and acne exacerbation (1). Weaning from high initial dose to a lower dose was possible with sustained effect; however, discontinuation of budesonide resulted in recurrence of hypoalbuminemia.


CONCLUSIONS
Oral budesonide is an effective therapy for treating protein-losing enteropathy after the Fontan operation. To maintain response, low-dose therapy must be continued.
Thacker
Deepika
D
Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
Patel
Akash
A
Dodds
Kathryn
K
Goldberg
David J
DJ
Semeao
Edisio
E
Rychik
Jack
J
D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage* / Q000009:adverse effects; D019819:Budesonide / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D008297:Male; D011504:Protein-Losing Enteropathies / Q000097:blood / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D012709:Serum Albumin / Q000032:analysis; D055815:Young Adult
D000893:Anti-Inflammatory Agents; D012709:Serum Albumin; D019819:Budesonide