Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Amsterdam University Medical Center, University of Amsterdam, 1105 AZ Amsterdam, The Netherlands.
Tytgat Institute for Liver and Intestinal Research, Amsterdam Gastroenterology Endocrinology Metabolism, Academic Medical Center, University of Amsterdam, 1105 BK Amsterdam, The Netherlands.
Nutrients. 2024 Sep 3;16(17):2966. doi: 10.3390/nu16172966.
Pediatric chronic intestinal failure (PIF) is a rare and heterogeneous condition characterized by the inability of the patient's intestine to adequately absorb the required fluids and/or nutrients for growth and homeostasis. As a result, patients will become dependent on home parenteral nutrition (HPN). A MEDLINE search was performed in May 2024 with keywords "intestinal failure", "parenteral nutrition" and "pediatric". Different underlying conditions which may result in PIF include short bowel syndrome, intestinal neuromuscular motility disorders and congenital enteropathies. Most common complications associated with HPN are catheter-related bloodstream infections, catheter-related thrombosis, intestinal failure-associated liver disease, small intestinal bacterial overgrowth, metabolic bone disease and renal impairment. Treatment for children with PIF has markedly improved with a great reduction in morbidity and mortality. Centralization of care in specialist centers and international collaboration between centers is paramount to further improve care for this vulnerable patient group. A recently promising medical therapy has become available for children with short bowel syndrome which includes glucagon-like peptide 2, a naturally occurring hormone which is known to delay gastric emptying and induce epithelial proliferation. Despite advances in curative and supportive treatment, further research is necessary to improve nutritional, pharmacological and surgical care and prevention of complications associated with parenteral nutrition use.
儿科慢性肠衰竭(PIF)是一种罕见且异质性的疾病,其特征是患者的肠道无法充分吸收生长和体内平衡所需的液体和/或营养物质。因此,患者将依赖于家庭肠外营养(HPN)。2024 年 5 月,使用“肠衰竭”、“肠外营养”和“儿科”等关键词进行了 MEDLINE 搜索。可能导致 PIF 的不同潜在疾病包括短肠综合征、肠神经肌肉运动障碍和先天性肠病。与 HPN 相关的最常见并发症包括导管相关血流感染、导管相关血栓形成、肠衰竭相关肝病、小肠细菌过度生长、代谢性骨病和肾功能损害。儿童 PIF 的治疗方法已有显著改善,发病率和死亡率明显降低。在专门中心集中治疗并在中心之间开展国际合作,对于进一步改善这一脆弱患者群体的护理至关重要。一种最近有前途的医学治疗方法已可用于短肠综合征患儿,包括胰高血糖素样肽 2,这是一种天然存在的激素,已知可延迟胃排空并诱导上皮增殖。尽管在治愈和支持治疗方面取得了进展,但仍有必要开展进一步的研究,以改善与肠外营养使用相关的营养、药理学和手术护理以及预防并发症。