Hepatology Gastroenterology and Nutrition Unit, "Bambino Gesù" Children Hospital, 00165 Rome, Italy.
Pediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, 41121 Modena, Italy.
Nutrients. 2021 Feb 27;13(3):786. doi: 10.3390/nu13030786.
Intestinal failure (IF) is defined as reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption. In most cases, IF results from intrinsic diseases of the gastrointestinal tract (digestive IF) (DIF); few cases arise from digestive vascular components, gut annexed (liver and pancreas) and extra-digestive organs or from systemic diseases (non-digestive IF) (NDIF). The present review revised etiology and treatments of DIF and NDIF, with special focus on the pathophysiological mechanisms, whereby NDIF develops.
We performed a comprehensive search of published literature from January 2010 to the present by selecting the following search strings: "intestinal failure" OR "home parenteral nutrition" OR "short bowel syndrome" OR "chronic pseudo-obstruction" OR "chronic intestinal pseudo-obstruction" OR "autoimmune enteropathy" OR "long-term parenteral nutrition".
We collected overall 1656 patients with well-documented etiology of IF: 1419 with DIF (86%) and 237 with NDIF (14%), 55% males and 45% females. Among DIF cases, 66% had SBS and among NDIF cases 90% had malabsorption/maldigestion.
The improved availability of diagnostic and therapeutic tools has increased prevalence and life expectancy of rare and severe diseases responsible for IF. The present review greatly expands the spectrum of knowledge on the pathophysiological mechanisms through which the diseases not strictly affecting the intestine can cause IF. In view of the rarity of the majority of pediatric IF diseases, the development of IF Registries is strongly required; in fact, through information flow within the network, the Registries could improve IF knowledge and management.
肠衰竭（IF）被定义为消化和吸收所需的最低肠道功能减少。在大多数情况下，IF 是由胃肠道（消化 IF）（DIF）的内在疾病引起的；少数情况是由消化血管成分、附属肠道（肝和胰腺）和非消化器官或系统性疾病（非消化 IF）（NDIF）引起的。本综述修订了 DIF 和 NDIF 的病因和治疗方法，特别关注 NDIF 发展的病理生理机制。
我们通过选择以下搜索字符串，对 2010 年 1 月至目前发表的文献进行了全面搜索：“肠衰竭”或“家庭肠外营养”或“短肠综合征”或“慢性假性肠梗阻”或“慢性肠假性肠梗阻”或“自身免疫性肠病”或“长期肠外营养”。
我们共收集了 1656 例病因明确的 IF 患者：1419 例为 DIF（86%），237 例为 NDIF（14%），男性占 55%，女性占 45%。在 DIF 病例中，66%为 SBS，而在 NDIF 病例中，90%为吸收不良/消化不良。
诊断和治疗工具的改进提高了罕见和严重疾病导致 IF 的患病率和预期寿命。本综述极大地扩展了知识范围，了解了并非严格影响肠道的疾病如何导致 IF 的病理生理机制。鉴于大多数儿科 IF 疾病的罕见性，强烈需要开发 IF 登记处；事实上，通过网络内的信息流，登记处可以提高 IF 的知识和管理水平。
