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Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment
Jeffery Meadows1, Kathy Jenkins
1Division of Pediatric Cardiology, University of California-San Francisco, 505 Parnassus Avenue, San Francisco, CA 94143-4144, USA. jeffery.meadows@ucsf.edu
Insights
Protein-losing enteropathy is a rare but burdensome complication after Fontan procedures for congenital heart disease. This study reviews current management strategies and knowledge gaps for this challenging condition.
Area of Science:
- Cardiology
- Gastroenterology
- Pediatric Medicine
Background:
- Protein-losing enteropathy (PLE) is an uncommon complication following Fontan procedures for complex congenital cardiac disease.
- PLE imposes significant medical, psychosocial, and financial burdens on patients, families, and healthcare systems.
- Current understanding of PLE pathogenesis and pathophysiology in this population is limited, with few long-term successful treatments.
Purpose of the Study:
- To describe a single institution's experience in evaluating, managing, and treating PLE in patients with congenital heart disease.
- To provide a comprehensive review of PLE, focusing on current knowledge and uncertainties regarding its pathophysiology.
- To offer suggestions for the prevention and treatment of PLE in this specific patient group.
Main Methods:
- Retrospective analysis of patient data from a single institution.
- Review of existing literature on protein-losing enteropathy in congenital cardiac disease.
- Clinical case review and treatment outcome assessment.
Main Results:
- Detailed description of the evaluation and management approaches used at the institution.
- Identification of key knowledge gaps in the pathophysiology and treatment of PLE.
- Analysis of treatment outcomes, highlighting challenges in achieving long-term success.
Conclusions:
- Protein-losing enteropathy remains a significant challenge post-Fontan procedure, necessitating further research.
- A multidisciplinary approach is crucial for managing patients with PLE.
- Future research should focus on elucidating pathophysiology to develop more effective prevention and treatment strategies.
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.
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