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

Dominik Lenz,Hans-Jürgen Häusler,Peter Schneider,Jörg Hambsch,John Hess,Attila Tárnok,Ursula Sauer

doi:10.1186/cc2166

Abstract

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. 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. 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.

Highlights

Protein-losing enteropathy (PLE) is a recognised complication of the Fontan circulation
We describe our observation of enteric infection and inflammatory response at the onset of protein-losing enteropathy (PLE) in a child with Fontan circulation
The patient was admitted to hospital with rotavirus and streptococcal tonsillitis

Summary

Introduction

Protein-losing enteropathy (PLE) is a recognised complication of the Fontan circulation. 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. A late complication develops in some patients, consisting of a substantial decrease of serum protein and γ-globulin, an increase in stool α1-antitrypsin level, and a substantial loss of circulating lymphocytes [2,3,4,5]. These features are typical of protein-losing enteropathy (PLE), and are associated with significant mortality [4,6,7,8]. We have reviewed the findings in seven other patients with longstanding PLE

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