Department of Paediatric Gastroenterology and Nutrition, RHSC, Edinburgh, UK.
Arch Dis Child. 2009 Dec;94(12):938-43. doi: 10.1136/adc.2008.141978. Epub 2009 Aug 17.
OBJECTIVES, SETTING AND PATIENTS: With complete case referral for prolonged parenteral nutrition (PN) beyond term equivalent, serving a stable population of 1.25 million people, we describe the long-term outcome and survival of patients referred to an intestinal failure (IF) nutrition support team over the first 8 years of existence at a regional paediatric centre, and extrapolate to potential numbers of national home parenteral nutrition (HPN) cases and intestinal transplantation data.
Retrospective analysis detailing patient demographics, interventions, use of HPN, occurrence of intestinal failure-associated liver disease (IFALD), and outcomes of enteral adaptation, survival, and referral for and receipt of organ transplantation.
23 patients were referred over 8 years, 20 being PN dependent within the neonatal period. Diagnoses included short bowel syndrome (SBS) (18), neuromuscular abnormalities (4) and congenital enterocyte disorder (1). 12 696 days of PN were delivered with 314 confirmed episodes of sepsis at a median of 12 episodes per patient. 144 central venous catheters (CVCs) were required at a median of four per patient. IFALD occurred in 17 (73%) patients, with 10 (44%) referred for transplant assessment. Thirteen (56%) children received HPN. Overall mortality was 44%. A significant predictor for survival in the SBS group was residual bowel >40 cm (82% vs 28%, p = 0.049).
Survival for IF at 56% was lower than reported from non-UK supra-regional centres, and nationally collected data, possibly reflecting pre-selected referral populations. Data from regional centres with complete ascertainment may be important both when counselling parents and when planning regional and national HPN and IF specialist services.
目的、背景和患者：通过对超出足月的长期肠外营养（PN）进行完整病例转诊，为一个稳定的 125 万人口的人群提供服务，我们描述了在一家区域儿科中心的 IF 营养支持小组存在的前 8 年中，转诊患者的长期结果和生存率，并推断出全国家庭肠外营养（HPN）病例和肠移植数据的潜在数量。
详细的回顾性分析患者的人口统计学、干预措施、HPN 的使用、发生的肠衰竭相关肝病（IFALD）以及肠内适应、生存、器官移植转诊和接受的情况。
8 年内共转诊 23 例患者，其中 20 例在新生儿期依赖 PN。诊断包括短肠综合征（SBS）（18 例）、神经肌肉异常（4 例）和先天性肠细胞疾病（1 例）。共提供了 12696 天的 PN，中位数为每位患者 12 例确诊的败血症发作。中位数每位患者需要 4 个中心静脉导管（CVC）。17 例（73%）患者发生 IFALD，其中 10 例（44%）接受移植评估。13 例（56%）儿童接受 HPN。总体死亡率为 44%。SBS 组生存的显著预测因素是残留肠>40cm（82%比 28%，p=0.049）。
IF 的生存率为 56%，低于英国非区域中心和全国性数据报告，可能反映了预先选择的转诊人群。具有完整确定信息的区域中心的数据对于父母咨询和规划区域和国家 HPN 和 IF 专科服务都很重要。
