{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T08:09:15Z","timestamp":1777968555226,"version":"3.51.4"},"reference-count":27,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T00:00:00Z","timestamp":1764460800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Background: 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. Methods: 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. Results: 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\u201352.5). Patients diagnosed with SBS exhibited a lower gestational age and birthweight compared with the other groups (p &lt; 0.05). Patients diagnosed with SBS and CE exhibited a lower mean age at the onset of HPN (p &lt; 0.05). Furthermore, patients with CE exhibited the lowest weight-for-age Z-score at the initiation of HPN (p &lt; 0.05). Conclusions: 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.<\/jats:p>","DOI":"10.3390\/nu17233768","type":"journal-article","created":{"date-parts":[[2025,12,3]],"date-time":"2025-12-03T10:34:01Z","timestamp":1764758041000},"page":"3768","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Epidemiological Characteristics of Pediatric Patients with Intestinal Failure in Spain: Data from the REPAFI Registry"],"prefix":"10.3390","volume":"17","author":[{"given":"Marta","family":"Germ\u00e1n-D\u00edaz","sequence":"first","affiliation":[{"name":"Pediatric Gastroenterology, Hepatology and Nutrition Department, Hospital Universitario 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roc\u00edo","family":"Gonz\u00e1lez-Sacrist\u00e1n","sequence":"additional","affiliation":[{"name":"Intestinal Rehabilitation Unit, Hospital Universitario La Paz, 28046 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vanessa","family":"Cabello","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Nutrition Support Unit, Hospital Universitario Vall D\u2019Hebron, 08035 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4008-3747","authenticated-orcid":false,"given":"Javier","family":"Blasco-Alonso","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Nutrition Department, Hospital Regional Universitario de M\u00e1laga, 29010 M\u00e1laga, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alejandro","family":"Rodr\u00edguez","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology Department, Hospital Universitario Virgen del Roc\u00edo, 41013 Sevilla, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mariela","family":"de los Santos","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology, Hepatology and Nutrition Department, Hospital Universitario Sant Joan de Deu, 08950 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1660-6993","authenticated-orcid":false,"given":"Jos\u00e9 Vicente","family":"Arcos-Machancoses","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology, Hepatology and Nutrition, Hospital Cl\u00ednic Universitari, 46014 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6273-2600","authenticated-orcid":false,"given":"Mercedes","family":"Murray-Hurtado","sequence":"additional","affiliation":[{"name":"Pediatric Nutrition and Inborn Errors of Metabolism Section, Complejo Hospitalario Universitario de Canarias, 38320 San Crist\u00f3bal de La Laguna, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3017-1340","authenticated-orcid":false,"given":"Ruth","family":"Garc\u00eda-Romero","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Nutrition Department, Hospital Universitario Miguel Servet, 50008 Zaragoza, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4420-5051","authenticated-orcid":false,"given":"Rafael","family":"Galera-Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Nutrition Department, Hospital Universitario Torrec\u00e1rdenas, 04009 Almer\u00eda, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina","family":"Mart\u00edn-Arriscado","sequence":"additional","affiliation":[{"name":"Research Institute, Hospital Universitario 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susana","family":"Redecillas-Ferreiro","sequence":"additional","affiliation":[{"name":"Pediatric Gastroenterology and Nutrition Support Unit, Hospital Universitario Vall D\u2019Hebron, 08035 Barcelona, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1513-6579","authenticated-orcid":false,"given":"Jos\u00e9 Manuel","family":"Moreno-Villares","sequence":"additional","affiliation":[{"name":"Pediatrics Departement, Cl\u00ednica Universitaria de Navarra, 28027 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6743-9198","authenticated-orcid":false,"given":"Esther","family":"Ramos-Boluda","sequence":"additional","affiliation":[{"name":"Intestinal Rehabilitation Unit, Hospital Universitario La Paz, 28046 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,11,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"S16","DOI":"10.1053\/j.gastro.2005.12.002","article-title":"Causes and management of intestinal failure in children","volume":"130","author":"Goulet","year":"2006","journal-title":"Gastroenterology"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Diamanti, A., Capriati, T., Gandullia, P., Di Leo, G., Lezo, A., Lacitignola, L., Spagnuolo, M., Gatti, S., D\u2019antiga, L., and Verlato, G. (2017). Pediatric Chronic Intestinal Failure in Italy: Report from the 2016 Survey on Behalf of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP). Nutrients, 9.","DOI":"10.3390\/nu9111217"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1097\/MPG.0b013e318268a9e3","article-title":"Intestinal failure in children: The European view","volume":"56","author":"Goulet","year":"2013","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"20145","DOI":"10.1053\/j.gastro.2018.03.067","article-title":"Advances in Evaluation of Chronic Diarrhea in Infants","volume":"154","author":"Thiagarajah","year":"2018","journal-title":"Gastroenterology"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1016\/j.clnu.2012.05.004","article-title":"Outcome on home parenteral nutrition for benign intestinal failure: A review of the literature and benchmarking with the European prospective survey of ESPEN","volume":"31","author":"Pironi","year":"2012","journal-title":"Clin. Nutr."},{"key":"ref_6","first-page":"745","article-title":"Pediatric parenteral nutrition: Clinical practice guidelines from the Spanish Society of Parenteral and Enteral Nutrition (SENPE), the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP) and the Spanish Society of Hospital Pharmacy (SEFH)","volume":"34","author":"Terradillos","year":"2017","journal-title":"Nutr. Hosp."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"666","DOI":"10.1056\/NEJMra1602650","article-title":"Pediatric Intestinal Failure","volume":"377","author":"Duggan","year":"2017","journal-title":"N. Eng. J. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/j.jpeds.2016.10.027","article-title":"Outcomes from a 12-week, open-label, multicenter clinical trial of teduglutide in pediatric short bowel syndrome","volume":"181","author":"Carter","year":"2017","journal-title":"J. Pediatr."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.clnu.2006.07.004","article-title":"Prevalence of home artificial nutrition in Italy in 2005: A survey by the Italian Society for Parenteral and Enteral Nutrition (SINPE)","volume":"26","author":"Pironi","year":"2007","journal-title":"Clin. Nutr."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1016\/j.clnu.2011.02.003","article-title":"Trends in paediatric home parenteral nutrition and implications for service development","volume":"30","author":"Beath","year":"2011","journal-title":"Clin. Nutr."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1016\/j.clnu.2015.01.010","article-title":"Presentation of a nationwide multicenter registry of intestinal failure and intestinal transplantation","volume":"35","author":"Neelis","year":"2016","journal-title":"Clin. Nutr."},{"key":"ref_12","first-page":"409","article-title":"Chronic intestinal failure in children","volume":"109","author":"Krawinkel","year":"2012","journal-title":"Dtsch. Arztebl. Int."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"5278","DOI":"10.1016\/j.clnu.2021.08.002","article-title":"Pediatric Home Parenteral Nutrition in France: A six years national survey","volume":"40","author":"Goulet","year":"2021","journal-title":"Clin. Nutr."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"e85","DOI":"10.1097\/MPG.0000000000002635","article-title":"Portuguese Prevalence of Pediatric Chronic Intestinal Failure","volume":"70","author":"Antunes","year":"2020","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1128","DOI":"10.1016\/j.clnu.2014.11.009","article-title":"The continued rise of paediatric home parenteral nutrition use: Implications for service and the improvement of longitudinal data collection","volume":"34","author":"Barclay","year":"2015","journal-title":"Clin. Nutr."},{"key":"ref_16","first-page":"1304","article-title":"Nutrici\u00f3n parenteral domiciliaria en Espa\u00f1a, 2019: Informe del Grupo de Nutrici\u00f3n Artificial Domiciliaria y Ambulatoria NADYA [Home and Ambulatory Artificial Nutrition (NADYA) Group report: Home parenteral nutrition in Spain, 2019]","volume":"38","author":"Candela","year":"2021","journal-title":"Nutr. Hosp."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1016\/j.jbi.2008.08.010","article-title":"Researcho electronic data capture (REDCap)\u2014A metadata-driven methodology and workflow process for providing translational research informatics support","volume":"42","author":"Harris","year":"2009","journal-title":"J. Biomed. Inform."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1761","DOI":"10.1038\/ajg.2017.416","article-title":"Research to the N-Power: The Strengths of Networked Clinical Collaboration in Spain","volume":"112","author":"McNicholl","year":"2017","journal-title":"Am. J. Gastroenterol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1097\/MPG.0b013e31802c6971","article-title":"Long-term outcome of children receiving home parenteral nutrition: A 20-year single-center experience in 302 Patients","volume":"44","author":"Colomb","year":"2007","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1054\/clnu.2000.0380","article-title":"Clinical, social and rehabilitation status of long-term home parenteral nutrition patients: Results of a European multicenter survey","volume":"20","author":"Vahedi","year":"2001","journal-title":"Clin. Nutr."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1002\/ncp.10180","article-title":"Early History of Home Parenteral Nutrition: From Hospital to Home","volume":"33","author":"Hurt","year":"2018","journal-title":"Nutr. Clin. Pract."},{"key":"ref_22","first-page":"359","article-title":"Outcomes of a general hospital-based home parenteral nutrition (HPN) program; report of our experience from a 26-year period","volume":"30","author":"Higuera","year":"2014","journal-title":"Nutr. Hosp."},{"key":"ref_23","first-page":"170","article-title":"Nutrici\u00f3n parenteral domiciliaria: Experiencia inicial","volume":"44","year":"1996","journal-title":"An. Esp. Pediatr."},{"key":"ref_24","unstructured":"(2025, October 10). Gu\u00eda de Nutrici\u00f3n Parenteral Domiciliaria en el Sistema Nacional de Salud. Ministerio de Sanidad y pol\u00edtica Social 2009. Available online: https:\/\/www.sanidad.gob.es\/profesionales\/prestacionesSanitarias\/publicaciones\/docs\/guiaNPD.pdf."},{"key":"ref_25","first-page":"686","article-title":"Los registros de pacientes pedi\u00e1tricos con nutrici\u00f3n artificial en el domicilio. La experiencia espa\u00f1ola [The registers of pediatric patients with home artificial nutrition. The Spanish experience]","volume":"41","year":"2024","journal-title":"Nutr. Hosp."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1016\/j.dld.2010.04.012","article-title":"Long-term home parenteral nutrition in children with chronic intestinal failure: A 15-year experience at a single Italian centre","volume":"43","author":"Gandullia","year":"2011","journal-title":"Dig. Liver Dis."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1016\/j.jpedsurg.2013.02.070","article-title":"The impact of multi-disciplinary intestinal rehabilitation programs on the outcome of pediatric patients with intestinal failure: A systematic review and meta-analysis","volume":"48","author":"Stanger","year":"2013","journal-title":"J. Pediatr. Surg."}],"container-title":["Nutrients"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6643\/17\/23\/3768\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,3]],"date-time":"2025-12-03T10:43:07Z","timestamp":1764758587000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6643\/17\/23\/3768"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,30]]},"references-count":27,"journal-issue":{"issue":"23","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["nu17233768"],"URL":"https:\/\/doi.org\/10.3390\/nu17233768","relation":{},"ISSN":["2072-6643"],"issn-type":[{"value":"2072-6643","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11,30]]}}}