RESTORE "amendment": from Argentina to Latin America, new steps of the First prospective Intestinal Failure Registry due to short bowel syndrome.

Background: Type III Intestinal Failure (IF) due to short bowel syndrome remains a rare condition. Few registries worldwide are able to collect prospective information on diagnoses, management, complications and outcomes in the subject. Aim: to report the progression, updated results and future steps of the RESTORE amendment registry. Methods: A prospective multicenter observational registry was created in 2017 for adult patients with type III-IF due to SBS in Argentina (RESTORE). In May 2020, it was amended in order to include pediatric patients and to expand to Latin America in 2 steps (first including centers from Chile, Colombia, México, Peru and Uruguay; the second step will include the rest of the countries in the region). Demographics, clinical data, nutritional assessment, home parenteral nutrition (HPN) provision and management, medical and surgical rehabilitation techniques, related complications, overall survival, and freedom from PN survival were analyzed, from 6/2017 to 12/2022, accomplishing the first step. Data were collected in RedCap; statistics were run on SPSS v24.0. Results: Of the initially 37 identified centers, 25 obtained approval and enrolled patients; monitored data on 108 patients are analyzed. Table 1 shows data at enrollment, patients are grouped according to age. Etiologies of the IF were: surgical complications 35%, intestinal ischemia 24%, trauma 9%, mechanical obstruction 8%, volvulus 5%, Crohn’s 4% and others 15%, for adults; atresia 34%, gastroschisis 21%, NEC 21%, volvulus 12%, and others 12%, for children. At enrolment all patients were on PN and 83 (77%) were receiving HPN; at the end of the analysis 96 (89%) were on HPN. IF/PN related complications are shown in table 2 The mean time of follow up was 33.7±20.5 months for adults and 8.1±5.6months for children. During the whole period, 32 surgeries were performed on 28 patients (adults: 27 AGIRS, children: 2 STEP and 3 AGIRS). Sixteen patients received enterohormones (1 GLP-1, 15 GLP-2 [4 children]), no severe adverse events were observed. By the end of the period, 19 of 108 patients (17.5%) (all adults) achieved enteral autonomy; 4 of them were on GLP2. In the remaining 89 patients, weekly PN volume was reduced by 24% and PN calories by 39 % in adults, and 17% and 28 % respectively in children. One adult received a multivisceral transplant. Five patients were lost to follow up. Twelve patients died (10 adults), causes were: sepsis 6, diabetic ketoacidosis 1, post-surgical complications 1, while was not registered in 4.. Overall actuarial survival was 82% (83% for adults and 91% for children). Conclusion: The RESTORE “amendment” highlights the value of having a registry; it provides unprecedented and novel data on epidemiology, main causes, current medical and surgical approach, as well as outcome and complications related to IF/PN at dedicated centers in the region, providing a system to standardize care.