Floris Ea Udink Ten Cate1, Tobias Hannes2, Ingo Germund1, Markus Khalil3, Michael Huntgeburth4, Christian Apitz5, Konrad Brockmeier1, Narayanswami Sreeram1. 1. Department of Paediatric Cardiology, Heart Centre Cologne, University Hospital of Cologne, Cologne, Germany. 2. Department of Paediatrics, Children's Hospital, University Hospital of Cologne, Cologne, Germany. 3. Paediatric Heart Centre, Justus-Liebig University, Giessen, Germany. 4. Department of Cardiology, Heart Centre Cologne, University Hospital of Cologne, Cologne, Germany. 5. Department of Paediatric Cardiology, University Children's Hospital Ulm, Ulm, Germany.
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. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
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. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
Authors: Sven Dittrich; Anja Weise; Robert Cesnjevar; Oliver Rompel; André Rüffer; Martin Schöber; Julia Moosmann; Martin Glöckler Journal: Thorac Cardiovasc Surg Date: 2020-12-31 Impact factor: 1.827
Authors: Ja-Kyoung Yoon; Gi Beom Kim; Mi Kyoung Song; Sang Yun Lee; Seong Ho Kim; So Ick Jang; Woong Han Kim; Chang-Ha Lee; Kyung Jin Ahn; Eun Jung Bae Journal: Korean Circ J Date: 2022-03-16 Impact factor: 3.101