Unidad de Soporte Nutricional, Rehabilitación y Trasplante Intestinal, Hospital Universitario Fundación Favaloro (HUFF), Buenos Aires, Argentina.
Unidad de Soporte Nutricional, Sanatorio Delta, Rosario, Argentina.
JPEN J Parenter Enteral Nutr. 2022 Sep;46(7):1623-1631. doi: 10.1002/jpen.2387. Epub 2022 May 30.
Short bowel syndrome (SBS) is considered a low prevalence disease. In Argentina, no registries are available on chronic intestinal failure (CIF) and SBS. This project was designed as the first national registry to report adult patients with this disease.
A prospective multicenter observational registry was created including adult patients with CIF/SBS from approved centers. Demographics, clinical characteristics, nutrition assessment, home parenteral nutrition (HPN) management, surgeries performed, medical treatment, overall survival, and freedom from HPN survival were analyzed.
Of the 61 enrolled patients, 56 with available follow-up data were analyzed. At enrollment, the mean intestinal length was 59.5 ± 47.3 cm; the anatomy was type 1 (n = 41), type 2 (n = 10), and type 3 (n = 5). At the end of the interim analysis, anatomy changed to type 1 in 31, type 2 in 17, and type 3 in 8 patients. The overall mean time on HPN before enrollment was 33.5 ± 56.2 months. Autologous gastrointestinal reconstruction surgery was performed before enrollment on 21 patients, and afterward on 11. Nine patients (16.1%) were weaned off HPN with standard medical nutrition treatment; 12 patients received enterohormones, and 2 of them suspended HPN; one patient was considered a transplant candidate. In 23.7 ± 14.5 months, 11 of 56 patients discontinued HPN; Kaplan-Meier freedom from HPN survival was 28.9%. The number of cases collected represented 19.6 new adult CIF/SBS patients per year.
The RESTORE project allowed us to know the incidence, the current medical and surgical approach for this pathology, as well as its outcome and complications at dedicated centers.
短肠综合征（SBS）被认为是一种低发病率疾病。在阿根廷，尚无慢性肠衰竭（CIF）和 SBS 的登记。本项目旨在创建第一个报告此类疾病的成年患者的全国登记处。
创建了一个前瞻性多中心观察性登记处，纳入来自认可中心的成年 CIF/SBS 患者。分析人口统计学、临床特征、营养评估、家庭肠外营养（HPN）管理、手术、药物治疗、总生存率和无 HPN 生存率。
在纳入的 61 名患者中，对 56 名具有可获得随访数据的患者进行了分析。在入组时，平均肠长为 59.5±47.3cm；解剖类型为 1 型（n=41）、2 型（n=10）和 3 型（n=5）。在中期分析结束时，31 例解剖类型变为 1 型，17 例变为 2 型，8 例变为 3 型。入组前总体平均 HPN 时间为 33.5±56.2 个月。21 例患者在入组前接受了自体胃肠重建手术，此后又有 11 例患者接受了该手术。9 例（16.1%）患者在接受标准医学营养治疗后成功脱离 HPN；12 例患者接受肠激素治疗，其中 2 例患者停用 HPN；1 例患者被认为是移植候选者。在 23.7±14.5 个月时，56 例患者中有 11 例停止了 HPN；Kaplan-Meier 无 HPN 生存率为 28.9%。所收集的病例数代表每年新增 19.6 例成年 CIF/SBS 患者。
RESTORE 项目使我们能够了解该疾病在专门中心的发病情况、当前的医疗和手术方法以及其结局和并发症。
