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

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

Description

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.

Sujets

• Immunodeficiency
• Infection
• Inflammation

Informations

• Publié par: biomed
• Publié le: 01 janvier 2003
• Nombre de lectures: 7
• Langue: English

Extrait

Available onlinehttp://ccforum.com/content/7/2/185

Open Access Research Proteinlosing enteropathy in patients with Fontan circulation: is it triggered by infection? 1 23 24 Dominik Lenz, Jörg Hambsch, Peter Schneider, HansJürgen 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, [e-mail]leipzig.de

Received: 5 September 2002

Revisions requested: 18 December 2002

Revisions received: 20 January 2003

Accepted: 17 February 2003

Published: 3 March 2003

Critical Care2003,7:185190 (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 13648535; Online ISSN 1466609X). 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 IntroductionProtein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. PatientsWe 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 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 andγglobulin, an increase in stoolαanti 1

trypsin level, and a substantial loss of circulating lymphocytes [2–5]. These features are typical of proteinlosing 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 = enzymelinked immunosorbent assay; IL = interleukin; PLE = proteinlosing enteropathy.

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