title: Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment.
abstract: Protein-losing enteropathy is a relatively uncommon complication of Fontan procedures for palliation of complex congenital cardiac disease. However, the relative infrequency of protein-losing enteropathy belies the tremendous medical, psychosocial and financial burdens it places upon afflicted patients, their families and the healthcare system that supports them. Unfortunately, because of the complexity and rarity of this disease process, the pathogenesis and pathophysiology of protein-losing enteropathy remain poorly understood, and attempts at treatment seldom yield long-term success. The most comprehensive analyses of protein-losing enteropathy in this patient population are now over a decade old, and re-evaluation of the prevalence and progress in treatment of this disease is needed. This report describes a single institution experience with the evaluation, management, and treatment of protein-losing enteropathy in patients with congenital cardiac disease in the current era, follows with a comprehensive review of protein-losing enteropathy, focused upon what is known and not known about the pathophysiology of protein-losing enteropathy in this patient population, and concludes with suggestions for prevention and treatment.
authors.affiliation: Division of Pediatric Cardiology, University of California-San Francisco, 505 Parnassus Avenue, San Francisco, CA 94143-4144, USA. jeffery.meadows@ucsf.edu
authors.forename: Jeffery
authors.initials: J
authors.lastname: Meadows
authors.forename: Kathy
authors.initials: K
authors.lastname: Jenkins
mesh_terms: D000046:Academic Medical Centers; D000293:Adolescent; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003131:Combined Modality Therapy; D004359:Drug Therapy, Combination; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000379:methods; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D011379:Prognosis; D011504:Protein-Losing Enteropathies / Q000209:etiology / Q000401:mortality / Q000517:prevention & control* / Q000628:therapy*; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult