PT  - JOURNAL ARTICLE
AU  - Udink ten Cate, Floris EA
AU  - Hannes, Tobias
AU  - Germund, Ingo
AU  - Khalil, Markus
AU  - Huntgeburth, Michael
AU  - Apitz, Christian
AU  - Brockmeier, Konrad
AU  - Sreeram, Narayanswami
TI  - Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review
AID  - 10.1136/heartjnl-2015-308823
DP  - 2016 Jul 15
TA  - Heart
PG  - 1115--1119
VI  - 102
IP  - 14
4099  - http://heart.bmj.com/content/102/14/1115.short
4100  - http://heart.bmj.com/content/102/14/1115.full
SO  - Heart2016 Jul 15; 102
AB  - 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.