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Original article
Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review
  1. Floris EA Udink ten Cate1,
  2. Tobias Hannes2,
  3. Ingo Germund1,
  4. Markus Khalil3,
  5. Michael Huntgeburth4,
  6. Christian Apitz5,
  7. Konrad Brockmeier1,
  8. Narayanswami Sreeram1
  1. 1Department of Paediatric Cardiology, Heart Centre Cologne, University Hospital of Cologne, Cologne, Germany
  2. 2Department of Paediatrics, Children's Hospital, University Hospital of Cologne, Cologne, Germany
  3. 3Paediatric Heart Centre, Justus-Liebig University, Giessen, Germany
  4. 4Department of Cardiology, Heart Centre Cologne, University Hospital of Cologne, Cologne, Germany
  5. 5Department of Paediatric Cardiology, University Children's Hospital Ulm, Ulm, Germany
  1. Correspondence to Dr Floris EA Udink ten Cate, Department of Paediatric Cardiology, Heart Centre Cologne, University Hospital of Cologne, Kerpener Str. 62, Cologne 50937, Germany; floris.udink-ten-cate{at}uk-koeln.de

Abstract

Objective A standardised diagnostic definition of protein-losing enteropathy (PLE) in Fontan patients serves both patient care and research. The present study determined whether a diagnostic definition of PLE was routinely used in published clinical Fontan studies, and to identify potentially relevant diagnostic criteria for composing a uniform PLE definition.

Methods A systematic review was conducted in adherence to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) recommendations. Published clinical Fontan studies that were written in English and included at least four patients with PLE were selected. PLE definitions were quantitatively analysed using a lateral thinking tool in which definitions were fractionated into constituent pieces of information (building blocks or diagnostic criteria).

Results We identified 364 papers. In the final analysis, data from 62 published articles were extracted. A diagnostic definition of PLE was used in only 27/62 (43.5%) of selected studies, and definitions were very heterogeneous. We identified eight major diagnostic criteria. Hypoalbuminaemia (n=23 studies, 85.2%), clinical presentation (n=18, 66.7%), documentation of enteric protein loss (n=16, 59.3%) and exclusion of other causes of hypoproteinaemia (n=17, 63.0%), were the most frequently used diagnostic criteria. Most studies used three diagnostic variables (n=13/27, 48.1%). Cut-off values for laboratory parameters (serum albumin, protein or faecal α-1-antitrypsin) were frequently incorporated in the PLE definition (n=16, 59.3%).

Conclusions Establishment of a universally accepted PLE definition for routine use in clinical research and daily practice is required. The diagnostic criteria may help constitute a diagnostic PLE definition.

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