when using infliximab, special awareness of potential risks is necessary in patients with chronic infections or in a state of immunodeficiency.

IntroductionThe Fontan procedure was developed as a means for separating the systemic and pulmonary circulation in patients with tricuspid 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 develops in some patients, consisting of a substantial decrease of serum protein and γ-globulin, an increase in stool α 1 -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].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 findings in seven other patients with longstanding PLE.185 EBV = Epstein-Barr virus; ELISA = enzyme-linked immunosorbent assay; IL = interleukin; PLE = protein-losing enteropathy. AbstractIntroduction 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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