Proli Francesco, Faragalli Andrea, Talbotec Cécile, Bucci Andrea, Zemrani Boutaina, Chardot Christophe, Abi Nader Elie, Goulet Olivier, Lambe Cécile
Division of Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Intestinal Diseases, Intestinal Rehabilitation Center, Certified Center for Home Parenteral Nutrition, Hôpital Necker Enfants Malades, Université de Paris, Faculté de Médecine Paris-Descartes, France; Department of Pediatrics, Department of Woman, Child Health and Public Health, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy.
Centre of Epidemiology, Biostatistics and Medical Information Technology, Università Pol. Delle Marche, Ancona, Italy.
Clin Nutr. 2021 Aug;40(8):4941-4947. doi: 10.1016/j.clnu.2021.07.017. Epub 2021 Jul 18.
BACKGROUND & AIMS: Long-term parenteral nutrition (PN) is the mainstay of the therapeutic strategy in intestinal failure (IF) due to neonatal short bowel syndrome (SBS). Our aim was to identify prognostic factors for PN weaning and to assess if measuring plasma citrulline concentrations over time could account for the intestinal adaptation in progress.
This retrospective study included children with neonatal SBS with surgical measurement of the residual bowel length and repeated plasma citrulline assessments during a 4-year follow-up. The degree of IF was assessed by the PN dependency index (PN caloric intake/Resting energy expenditure). The analysis was carried out according to SBS anatomical groups: end-jejunostomy (type 1), jejuno-colic (type 2) and jejuno-ileal anastomosis (type 3).
Fifty-five patients (8 type 1, 27 type 2, 20 type 3) were included. None of the patients with SBS type 1, 11 (41%) with type 2 and 11 (55%) with type 3 were weaned off during the follow-up period. Plasma citrulline levels significantly increased with time in patients who were finally weaned off PN; conversely, the levels did not consistently increase in patients who were still on PN at the end of the study period. There was an inverse relationship between plasma citrulline levels and the PN dependency index. The increasing citrulline levels had a positive effect on the probability of weaning, 2.7 times higher for each point increase in citrulline. No significant effect of age and residual bowel length at baseline was found.
The increased plasma citrulline level over time in addition to the SBS anatomical type is a reliable marker for subsequent PN weaning. The prediction of PN weaning assessed solely by the residual bowel length or a single measurement of citrulline is insufficient and should also take into account the anatomical type of SBS and repeated measurements of plasma citrulline levels.
长期肠外营养(PN)是新生儿短肠综合征(SBS)所致肠衰竭(IF)治疗策略的主要支柱。我们的目的是确定PN撤机的预后因素,并评估随时间测量血浆瓜氨酸浓度是否能反映正在进行的肠道适应情况。
这项回顾性研究纳入了患有新生儿SBS的儿童,在4年随访期间进行了手术测量残余肠长度并重复进行血浆瓜氨酸评估。通过PN依赖指数(PN热量摄入/静息能量消耗)评估IF的程度。根据SBS解剖学分组进行分析:空肠末端造口术(1型)、空肠结肠吻合术(2型)和空肠回肠吻合术(3型)。
纳入55例患者(1型8例,2型27例,3型20例)。随访期间,1型SBS患者无一例撤机,2型中有11例(41%)撤机,3型中有11例(55%)撤机。最终撤机的患者血浆瓜氨酸水平随时间显著升高;相反,在研究期末仍接受PN治疗的患者中,血浆瓜氨酸水平并未持续升高。血浆瓜氨酸水平与PN依赖指数呈负相关。瓜氨酸水平升高对撤机概率有积极影响,瓜氨酸每升高一个点,撤机概率高2.7倍。未发现基线时年龄和残余肠长度有显著影响。
除SBS解剖类型外,血浆瓜氨酸水平随时间升高是后续PN撤机的可靠标志物。仅通过残余肠长度或单次瓜氨酸测量评估PN撤机是不够的,还应考虑SBS的解剖类型以及血浆瓜氨酸水平的重复测量。