Protein-losing enteropathy in patients with Fontan circulation: is it triggered by infection?
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2003
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2003
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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.
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Available onlinehttp://ccforum.com/content/7/2/185
Open Access Research Proteinlosing enteropathy in patients with Fontan circulation: is it triggered by infection? 1 23 24 Dominik Lenz, Jörg Hambsch, Peter Schneider, HansJürgen Häusler, Ursula Sauer, 5 6 John Hessand Attila Tárnok
1 Research Assistant, Research Laboratory, Department of Paediatric Cardiology, Cardiac Centre Leipzig, University Hospital, Leipzig, Germany 2 Assistant Medical Director, Department of Paediatric Cardiology, Cardiac Centre Leipzig, University Hospital, Leipzig, Germany 3 Director, Department of Paediatric Cardiology, Cardiac Centre Leipzig, University Hospital, Leipzig, Germany 4 Assistant Medical Director, Department of Paediatric Cardiology, German Cardiac Centre, Munich, Germany 5 Director, Department of Paediatric Cardiology, German Cardiac Centre, Munich, Germany 6 Director, Research Laboratory, Department of Paediatric Cardiology, Cardiac Centre Leipzig, University Hospital, Leipzig, Germany
Correspondence: Attila Tárnok, tarnok@medizin.unileipzig.de
Received: 5 September 2002
Revisions requested: 18 December 2002
Revisions received: 20 January 2003
Accepted: 17 February 2003
Published: 3 March 2003
Critical Care2003,7:185190 (DOI 10.1186/cc2166) This article is online at http://ccforum.com/content/7/2/185 © 2003 Lenzet al., licensee BioMed Central Ltd (Print ISSN 13648535; Online ISSN 1466609X). This is an Open Access article: verbatim copying and redistribution of this article are permitted in all media for any purpose, provided this notice is preserved along with the article's original URL.
Abstract IntroductionProteinlosing 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. PatientsWe report a 4.5yearold 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 conclusionThe 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.
KeywordsFontan type surgery, immunodeficiency, infection, inflammation
Introduction
The Fontan procedure was developed as a means for separat ing the systemic and pulmonary circulation in patients with tri cuspid atresia, and was then applied to other patients with a functionally single ventricle [1]. Venous hypertension is a general feature of this circulation. A late complication devel ops in some patients, consisting of a substantial decrease of serum protein andγglobulin, an increase in stoolαanti 1
trypsin level, and a substantial loss of circulating lymphocytes [2–5]. These features are typical of proteinlosing enteropathy (PLE), and are associated with significant mortality [4,6–8].
In the present report, we describe our observation of enteric infection and inflammatory response at the onset of PLE in a child with Fontan circulation. We have also reviewed the find ings in seven other patients with longstanding PLE.
EBV = Epstein–Barr virus; ELISA = enzymelinked immunosorbent assay; IL = interleukin; PLE = proteinlosing enteropathy.
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