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Fontan-associated protein-losing enteropathy and plastic bronchitis
Kurt R Schumacher1, Kathleen A Stringer2, Janet E Donohue1
1University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
The Journal of Pediatrics
|February 10, 2015
Summary
This study highlights patient-specific factors linked to Fontan-associated protein-losing enteropathy (PLE) and plastic bronchitis, revealing diverse, unpatterned treatment strategies for these rare conditions.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Gastroenterology
Background:
- Fontan-associated protein-losing enteropathy (PLE) and plastic bronchitis are rare, serious complications following the Fontan procedure.
- Current understanding of patient-specific factors and treatment patterns for these conditions is limited.
- Social media platforms offer a novel approach for recruiting participants in rare disease research.
Purpose of the Study:
- To characterize the medical history, disease progression, and treatment of current-era patients with Fontan-associated protein-losing enteropathy (PLE) and plastic bronchitis.
- To identify patient-specific factors associated with the development of PLE and plastic bronchitis post-Fontan procedure.
Main Methods:
- A novel online survey was distributed via a Facebook portal to recruit participants, leveraging social media for study recruitment.
- The survey collected data on demographics, medical history, and treatment information from patients with and without PLE or plastic bronchitis.
- Case control analyses compared patients with PLE and plastic bronchitis to uncomplicated control patients who underwent the Fontan procedure.
Main Results:
- The survey included 671 subjects: 76 with PLE, 46 with plastic bronchitis, and 7 with both.
- Patients with PLE showed higher incidences of hypoplastic left ventricle, chylothorax, and additional cardiothoracic surgery compared to controls.
- Patients with plastic bronchitis had increased rates of chylothorax and seasonal allergies compared to controls.
Conclusions:
- Patient-specific factors are significantly associated with the development of Fontan-associated protein-losing enteropathy and plastic bronchitis.
- Treatment strategies for these conditions are highly diverse and lack clear, consistent patterns.
- Further research is needed to establish consensus-based treatment approaches and design future therapeutic studies for these rare complications.
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