背景:伴有慢性肠衰竭的短肠综合征(SBS/CIF)是无法维持蛋白质能量,流体,电解质,或微量营养素平衡由于短肠。尽管SBS/CIF很少见,它的临床管理很复杂,具有挑战性,贵,而且耗时。
目的:本研究旨在分析单中心在接受家庭肠外营养(HPN)治疗的成年患者中使用SBS/CIF的经验。
方法:在1994年1月至2023年8月的HPN计划中纳入了对所有13例SBS/CIF连续成年患者的回顾性单中心分析。
结果:在1992年至2023年之间,有13例患者被纳入HPN计划。主要病理基础为急性肠系膜缺血。开始HPN的中位年龄为44岁。大多数人都接受了广泛的肠切除术和后肠重建的多次手术。13名患者中有5名在HPN期间死亡,中位持续时间为42个月。与HPN相关的死亡原因是导管脓毒症,心内膜炎伴心力衰竭,或者肝衰竭.1例患者因病理原因死亡:盆腔脓肿及放疗相关出血。八个病人还活着,HPN的中位时间为173个月。在HPN支持期间,最常见的并发症是静脉导管感染和静脉区域血栓形成.存活的8名患者均无肝功能衰竭。最近有两名患者开始使用teduglutide,耐受性良好,需要减少HPN支持。所有八名患者的生活质量都令人满意(肠胃外支持需求范围为每周五到两个营养袋)。结论:家庭肠外营养仍是SBS/CIF治疗的金标准,尽管teduglutide可以减少HPN需求和并发症,并提供更好的生活质量。尽管患者人数很少,这项研究显示的结果并不逊色于大体积中心。圣安东尼奥大学医院SBS/CIF专业人员的承诺和兴趣的存在,葡萄牙,是实现这些结果的根本关键。为HPN提供支持的多学科医疗保健小组对于确保这些患者的生存和生活质量至关重要。
BACKGROUND: Short bowel syndrome with chronic intestinal failure (SBS/CIF) is the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balance due to a short bowel. Although SBS/CIF is rare, its clinical management is complex, challenging, expensive, and time-consuming.
OBJECTIVE: This study aimed to analyze a single center\'s experience with SBS/CIF in adult patients treated with home parenteral nutrition (HPN).
METHODS: A retrospective single-center analysis of all 13 consecutive adult patients with SBS/CIF was included in an HPN program between January 1994 and August 2023.
RESULTS: Between 1992 and 2023, 13 patients were included in an HPN program. The primary underlying pathology was acute mesenteric ischemia. The median age of starting HPN was 44 years. Most were subjected to several surgeries of extensive intestinal resection with posterior intestinal reconstruction. Five of the 13 patients died while on HPN with a median duration of 42 months. The causes of death related to HPN were catheter sepsis, endocarditis with cardiac failure, or hepatic failure. One patient died due to underlying pathology: pelvic abscesses and bleeding related to radiotherapy. Eight patients remain alive, with a median time of HPN of 173 months. During the HPN support, the most frequent complications were venous catheter infection and venous territory thrombosis. None of the eight patients alive have hepatic failure. Two patients recently started teduglutide with good tolerance and need a reduction in HPN support. All eight patients have a satisfactory quality of life (parenteral support needs range between five and two nutrition bags per week). Conclusion: Home parenteral nutrition remains the gold standard of SBS/CIF treatment, although teduglutide may reduce HPN needs and complications and provide a better quality of life. Despite the small number of patients, the results shown in this study are not inferior to those in large-volume centers. The existence of the commitment and interest of professionals involved in SBS/CIF at Centro Hospitalar Universitário de Santo António, Portugal, was a fundamental key to achieving those results. A multidisciplinary healthcare group for HPN support can be essential to ensuring these patients\' survival and quality of life.