A multicentre retrospective study of 66 Italian children with food protein-induced enterocolitis syndrome: different management for different phenotypes.

BACKGROUND: Food Protein-Induced Enterocolitis Syndrome (FPIES) is a non-IgE-mediated paediatric disorder triggered by the ingestion of specific food proteins. Many features of this syndrome are not yet well defined.\r\n\r\nOBJECTIVE: The aim of our study was to describe demographic features, causative agents, clinical features, treatments and outcomes of children suffering from acute FPIES at three Italian of Pediatric Allergology Centers.\r\n\r\nMETHODS: A retrospective study was performed over a 7-year period (2004-2010). Hospital medical record databases and hospital outpatient electronic charts were screened for the diagnosis of FPIES. Information on the first and subsequent FPIES' episodes was collected.\r\n\r\nRESULTS: We diagnosed 66 children with FPIES. The number of diagnoses significantly increased between 2008 and 2010 (P < 0.001). We collected a total of 165 FPIES episodes (median per child 2, range 1-10). Cow's milk was the most common trigger food (65%), followed by fish, egg, rice, soy, corn, poultry and goat's milk. Fifty-six (85%) children reacted to a single food. Mean documented time from ingestion to symptom onset was 2.4 h (SD 0.7 h). Vomiting was the most common symptom (98%). Among patients diagnosed with OFC, 78% reacted after eating a whole serving size of the trigger food per age. Skin prick tests (SPT) for trigger foods were negative in 97% of cases. Thirty-two/66 children (48%) achieved tolerance at a mean age of 29 months (SD 17 months). Age of achieved tolerance for cow's milk was significantly lower compared to that of other foods (24 ± 8 vs. 53 ± 17 months, P < 0.0006).\r\n\r\nCONCLUSION AND CLINICAL RELEVANCE: This article provides new insights on FPIES in Italy by describing its largest series, and shows how a significant increase in the FPIES diagnosis has been observed in the last few years. We also discussed selected management aspects of this syndrome where different phenotypes can be found.