Short bowel syndrome related intestinal failure outcomes in Latin America: Insights from the RESTORE Registry

BackgroundShort bowel syndrome is considered a low prevalence disease. The scant information available about intestinal failure in Latin America was the driving force to expand this registry.MethodsA prospective, multicenter observational registry was created for patients with chronic intestinal failure short bowel at specialized centers in Latin America. Demographics, clinical characteristics, nutrition assessment, parenteral nutrition management, intestinal rehabilitation, related complications, clinical outcome, and survival were analyzed.ResultsFrom May 2020 to July 2023, 167 patients (115 adults, 52 children) from 20 centers were enrolled. For the adults, the mean age was 37.2 +/- 18 years, 48% were female, and the mean follow-up was 22.6 +/- 18.3 months. The main etiology was surgical resections (postsurgical complications: 37%; ischemia: 25%); the mean intestinal length was 73 +/- 55 cm. The complications were as follows: infections: 0.4/1000 catheter-days; thrombosis: 0.24/1000 catheter-days; liver disease: 2.6%. The outcomes were as follows: 28% were rehabilitated, 15% died, 9.6% were lost to follow-up, 0.9% underwent transplant, and 45.6% continued follow-up. For the children, the mean age 48 +/- 52 months, 48% were female, 52% were premature. The mean follow-up was 17.2 +/- 5.6 months; the mean remaining intestinal length was 38 +/- 45 cm. The leading etiologies were atresia (25%), NEC (23%), and gastroschisis (21%). The complication were as follows: infections: 2/1000 catheter-days; thrombosis: 2.22/1000 catheter-day; 25% developed liver disease. The outcomes were as follows: 7.7% died, 3.8% were rehabilitated, and 88.5% continued follow-up.ConclusionThe RESTORE amendment served as a registry and educational tool for the participating teams. The aspiration is to objectively show current aspects of intestinal failure in the region and carry them to international standards. Including all Latin American countries and etiologies of chronic intestinal failure besides short gut would serve to complete this registry.