Pediatric Intestinal Rehabilitation and Transplantation Registry: Initial Report from a European Collaborative Registry
20 April 2017
04 July 2017
24 August 2017 (eFirst)
Introduction Short bowel syndrome (SBS) is the main cause of intestinal failure (IF) in the pediatric population. To promote the standardization of care of these patients, the registry of Pediatric Intestinal Rehabilitation and Transplantation (PIRAT) has been established. The aim of this study is to describe patients with IF using PIRAT database.
Materials and Methods Data from two tertiary care European referral Centers registered in PIRAT (https://www.studeon.eu/pirat) were analyzed (1994–2015). Neonatal SBS-related IF was defined as need for parenteral nutrition (PN) to sustain life and growth for more than 75 days, after extensive bowel resection during neonatal period. Data included patient demographics, disease at birth, residual small intestine, and intestinal autonomy (PN on/off).
Results In this study, 114 children with SBS-related IF were identified (male 60%). Median gestational age was 35.3 weeks (interquartile range [IQR]: 33.0–38.0); median birth weight was 2,440 g (IQR: 1,700–2,990). The main causes of SBS were intestinal atresia in 31 (27%), midgut volvulus in 29 (25%), necrotizing enterocolitis in 23 (20%), and gastroschisis in 12 (11%). Nine (7.9%) patients died on PN (six sepsis, two IF-associated liver disease, and one multiorgan failure). Median residual small bowel length was 46 cm (IQR: 13.0–92.5). Ileocecal valve was resected in 48 patients (42%). Intestinal autonomy was achieved in 68% patients.
Conclusion We present the web-based registry PIRAT and the first results of patients with IF registered from two European Centers. PIRAT could give the opportunity to create a dedicated international network (IF-net) to standardize, improve, and spread the therapeutic paths for the rare and heterogeneous condition of SBS-related IF.
Keywordsintestinal failure - short bowel syndrome - parenteral nutrition - registry - intestinal transplantation
* Both the authors contributed equally to the article.
- 1 Höllwarth ME. Surgical strategies in short bowel syndrome. Pediatr Surg Int 2017; 33 (04) 413-419
- 2 D'Antiga L, Goulet O. Intestinal failure in children: the European view. J Pediatr Gastroenterol Nutr 2013; 56 (02) 118-126
- 3 Pironi L, Goulet O, Buchman A. , et al; Home Artificial Nutrition and Chronic Intestinal Failure Working Group of ESPEN. Outcome on home parenteral nutrition for benign intestinal failure: a review of the literature and benchmarking with the European prospective survey of ESPEN. Clin Nutr 2012; 31 (06) 831-845
- 4 Neelis EG, Roskott AM, Dijkstra G. , et al. Presentation of a nationwide multicenter registry of intestinal failure and intestinal transplantation. Clin Nutr 2016; 35 (01) 225-229
- 5 Smith JM, Skeans MA, Horslen SP. , et al. OPTN/SRTR 2015 Annual Data Report: intestine. Am J Transplant 2017; 17 (Suppl. 01) 252-285
- 6 Diamanti A, Conforti A, Panetta F. , et al. Long-term outcome of home parenteral nutrition in patients with ultra-short bowel syndrome. J Pediatr Gastroenterol Nutr 2014; 58 (04) 438-442
- 7 Fusaro F, Hermans D, Wanty C, Veyckemans F, Pirenne J, Reding R. Post-serial transverse enteroplasty bowel redilatation treated by longitudinal intestinal lengthening and tailoring procedure. J Pediatr Surg 2012; 47 (10) e19-e22
- 8 Wood SJ, Khalil B, Fusaro F, Folaranmi SE, Sparks SA, Morabito A. Early structured surgical management plan for neonates with short bowel syndrome may improve outcomes. World J Surg 2013; 37 (07) 1714-1717
- 9 Fusaro F, Gamba PG, Orzali A, De Coppi P, Scarpa MG, Zanon GF. Vascular access in patients affected by short bowel syndrome. J Vasc Access 2000; 1 (01) 33-35
- 10 Fusaro F, Hermans D, Reding R. Autologous gastrointestinal reconstructive surgery: complement or alternative to intestinal transplantation. Curr Opin Organ Transplant 2013; 18 (03) 286-290
- 11 Sauvat F, Fusaro F, Lacaille F. , et al. Is intestinal transplantation the future of children with definitive intestinal insufficiency?. Eur J Pediatr Surg 2008; 18 (06) 368-371
- 12 Goulet O, Fusaro F, Lacaille F, Sauvat F. Permanent intestinal failure. Indian Pediatr 2008; 45 (09) 753-763