Epidemiological Characteristics of Pediatric Patients with Intestinal Failure in Spain: Data from the REPAFI Registry

The paucity of data on the epidemiology of chronic intestinal failure (CIF) in pediatric patients is a matter of particular concern. The objective of this article is to provide a comprehensive description of the epidemiology of CIF in Spain, encompassing its incidence, geographical distribution, underlying causes, and demographic and clinical characteristics. These findings are based on data collected from the multicentre REPAFI registry.

This is a national, multicentre, ambispective cohort study including patients who initiated home parenteral nutrition (HPN) between January 2015 and January 2025. The data collected encompassed various demographic details, underlying diagnoses, the type of HPN utilized, and the nutritional status of the subjects at the commencement of HPN treatment.

The study included 163 patients (55.2% male) from 10 hospitals. The principal cause of CIF was short bowel syndrome (SBS) in 77.3% of cases, followed by severe motility disorders (12.9%), congenital enteropathies (CE) (5.5%), and other causes (4.3%). Among patients diagnosed with SBS, necrotizing enterocolitis was identified as the most prevalent underlying cause (32.5%). The most prevalent anatomical configuration was identified as type 2 (jejuno-colic anastomosis). A significant proportion, amounting to 62.7%, exhibited a lack of an ileocecal valve (ICV), while 23% demonstrated a residual bowel length (RBL) of less than 15 centimetres. The median RBL was 35 cm (IQR: 15.7-52.5). Patients diagnosed with SBS exhibited a lower gestational age and birthweight compared with the other groups (p < 0.05). Patients diagnosed with SBS and CE exhibited a lower mean age at the onset of HPN (p < 0.05). Furthermore, patients with CE exhibited the lowest weight-for-age Z-score at the initiation of HPN (p < 0.05).

The present study provides the first epidemiological data on the state of pediatric CIF in Spain. The most prevalent cause of CIF was SBS, with a younger age at the initiation of HPN in comparison to other published studies. Patients with CE exhibited the most severe degree of malnutrition at the initiation of HPN.