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Protein-losing enteropathy in patients with Fontan circulation: is it triggered by infection?
Dominik Lenz1, Jörg Hambsch, Peter Schneider
1Department of Paediatric Cardiology, Cardiac Centre Leipzig, University Hospital, Leipzig, Germany.
Insights
Protein-losing enteropathy (PLE) following Fontan circulation may be triggered by infections. This study suggests infections and inflammation could initiate PLE onset in patients with compromised immune systems.
Area of Science:
- Pediatric Cardiology
- Gastroenterology
- Immunology
Background:
- Protein-losing enteropathy (PLE) is a known complication of Fontan circulation.
- The exact cause and delayed onset of PLE post-Fontan surgery remain unclear.
Observation:
- A case of a 4.5-year-old girl with Fontan circulation who developed PLE nearly a year after surgery.
- The onset of PLE coincided with rotavirus enteritis and streptococcal tonsillitis.
- Review of seven other patients with PLE revealed infections at symptom onset in six, with no opportunistic infections noted.
Findings:
- Infections and inflammation appear to be associated with the onset of PLE in patients with Fontan circulation.
- While the immune system is compromised in PLE patients, opportunistic infections are rare.
Implications:
- Infections may play a role in triggering the onset of Protein-losing enteropathy after Fontan surgery.
- Further research is needed to elucidate the precise mechanisms by which infections trigger PLE.
- Understanding this link could lead to improved management strategies for Fontan patients at risk of PLE.
Introduction:
Protein-losing enteropathy (PLE) is a recognised complication of the Fontan circulation. Its pathogenesis is not fully understood, however, and it is unclear why its onset occurs months or even years after Fontan surgery.
Patients:
We report a 4.5-year-old girl with Fontan circulation who developed PLE almost 1 year after surgery. At the time of onset the patient had rotavirus enteritis and streptococcal tonsillitis. We have reviewed the records of seven other patients with longstanding PLE. In six of these patients we identified infections at the onset of symptoms. None of our patients had evidence of opportunistic infection.
Discussion And Conclusion:
The immune system of patients with PLE is compromised, but reports on recurrent opportunistic infections are rare. The present observations suggest that infection and inflammation may be associated with the onset of PLE. The mechanism of how infection may trigger PLE warrants further investigation.