Forchielli Maria Luisa, Richardson Denise, Folkman Judah, Gura Kathleen, Lo Clifford W
Department of Pediatrics, S. Orsola-Malpighi, Bologna University, Bologna, Italy.
Nutrition. 2008 Jan;24(1):103-7. doi: 10.1016/j.nut.2007.10.001. Epub 2007 Nov 8.
We discuss 26 y of home parenteral nutrition (HPN) in an otherwise healthy patient with severe short bowel syndrome demonstrating a decrease in life-threatening complications after various management changes.
The patient is a 41-y-old male with a midgut volvulus from malrotation who developed short bowel syndrome and has been HPN dependent since the age of 15 y. His surgical and nutritional data were collected retrospectively and prospectively and included nutritional history, anthropometric parameters, laboratory results, activity levels and types, and treatments for complications. His entire HPN course has been prospectively followed.
Since becoming HPN dependent, the patient's energy intake range has been 20-45 kcal.kg(-1).d(-1), with 0.8-1.6 g of protein.kg(-1).d(-1). He receives HPN with electrolytes, multivitamins, and trace elements nightly and his intravenous fat emulsion ranges from one to seven times per week. Adjustments to magnesium, iron, zinc, selenium, vitamin E, and carnitine are often required. During his first years of HPN, he had six episodes of catheter-related sepsis and two central venous catheter occlusions. The current central venous catheter has been in place for >13 y without infection or replacement. He developed mild osteopenia but has maintained an active lifestyle without fractures. In the second and third decades of HPN, episodes of hepatic dysfunction occurred, with improvement or resolution using various interventions including oral fish oil.
This case illustrates the successful management of a life-long HPN-dependent patient in whom PN complications have been minimized, including a very recent occurrence of parenteral nutrition-associated cholestasis.
我们讨论了一名原本健康的严重短肠综合征患者接受26年家庭肠外营养(HPN)的情况,该患者在经过各种管理调整后,危及生命的并发症有所减少。
该患者为41岁男性,因肠旋转不良导致中肠扭转,继而发展为短肠综合征,自15岁起就依赖HPN。我们回顾性和前瞻性地收集了他的手术和营养数据,包括营养史、人体测量参数、实验室检查结果、活动水平及类型,以及并发症的治疗情况。对他整个HPN治疗过程进行了前瞻性跟踪。
自依赖HPN以来,患者的能量摄入量范围为20 - 45 kcal·kg⁻¹·d⁻¹,蛋白质摄入量为0.8 - 1.6 g·kg⁻¹·d⁻¹。他每晚接受含电解质、多种维生素和微量元素的HPN,静脉脂肪乳剂每周使用1至7次。经常需要调整镁、铁、锌、硒、维生素E和肉碱的用量。在HPN治疗的最初几年,他发生了6次导管相关败血症和2次中心静脉导管堵塞。目前的中心静脉导管已使用超过13年,未发生感染或更换。他出现了轻度骨质减少,但保持了积极的生活方式,未发生骨折。在HPN治疗的第二个和第三个十年中,出现了肝功能障碍,通过包括口服鱼油在内的各种干预措施,病情得到改善或缓解。
本病例说明了对一名终身依赖HPN的患者的成功管理,其中肠外营养并发症已降至最低,包括最近发生的肠外营养相关胆汁淤积。