Protein-losing enteropathy in patients with Fontan circulation: is it triggered by infection?
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Department of Paediatric Cardiology, Cardiac Centre Leipzig, University Hospital, Leipzig, Germany.
Dominik
D
Lenz
Jörg
J
Hambsch
Peter
P
Schneider
Hans-Jürgen
HJ
Häusler
Ursula
U
Sauer
John
J
Hess
Attila
A
Tárnok
D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D006801:Humans; D009894:Opportunistic Infections / Q000150:complications* / Q000276:immunology; D011183:Postoperative Complications / Q000209:etiology* / Q000276:immunology; D011504:Protein-Losing Enteropathies / Q000209:etiology* / Q000276:immunology; D012189:Retrospective Studies; D012400:Rotavirus Infections / Q000150:complications / Q000276:immunology; D013290:Streptococcal Infections / Q000150:complications / Q000276:immunology