Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review.

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.

Protein losing enteropathy in critically ill adult patients with burns: a preliminary report.

OBJECTIVE: Few data have been published regarding protein losing enteropathy in adult patients with burns. This study characterised the presence of protein-losing enteropathy in adults with burns and examined the relationship between the magnitude of burn size and the severity of protein loss. METHODS: Twenty adult patients with burns (BSA 31+/-25%, range 2-80%) were studied. Fluid resuscitation was based on the Parkland's formula. Protein loss into the gastrointestinal tract was measured using faecal alpha1-antitrypsin (FA-1-AT) concentrations. Serial measurements of serum protein and albumin concentrations were performed. RESULTS: Fourteen patients demonstrated elevations in FA-1-AT levels. The mean peak FA-1-AT level was 3.6+/-4.2 mg/g dry weight of stool. Two patients demonstrated elevated FA-1-AT excretion 1.5 months and 3 months after the burns. There was a good correlation between burn size and FA-1-AT excretion (R2=0.40). CONCLUSIONS: Protein losing enteropathy was demonstrable in patients with major burns. The magnitude of this phenomenon appears to be proportional to the burns size.