一名体重 9.7 千克的小儿全肝移植受体出现 "小而全 "综合征。

IF 1.2 4区 医学 Q3 PEDIATRICS Pediatric Transplantation Pub Date : 2024-03-01 DOI:10.1111/petr.14716
Haytham Maria, Pamela L Valentino, Patrick J Healey, Yong K Kwon
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引用次数: 0

摘要

背景:小儿肝移植受者,尤其是体重小于 10 千克的小儿肝移植受者,出现体型过小综合征 (SFSS) 的情况非常罕见。本报告描述了一例15个月大的全肝移植受者发生SFSS的病例,并进行了系统的文献回顾,以确定此类病例的结果以及SFSS的潜在风险因素:一名15个月大的幼儿因胆道闭锁接受了死亡供体全肝移植手术。移植物重160克,移植物与受体重量比(GRWR)为1.6%。术后因大量腹水导致血流动力学不稳定和肝动脉血流受损而并发了 SFSS。使用奥曲肽进行药物干预后,患者最终康复:结论:对于体型较小的儿童受者,尤其是体重不足 10 千克的受者,其原生肝脏体重比(LBWR)明显较高。在为这些受者选择合适大小的移植物时,应考虑到这一较高的比率。文献综述表明,在体重小于 10 公斤的小儿受者中,肝脏重量比小于 2% 与较高的小尺寸综合症发病率有关。
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Small-for-size syndrome in a 9.7 kg pediatric recipient with a whole liver graft.

Background: Small-for-size syndrome (SFSS) in pediatric liver transplant recipients, particularly those weighing less than 10 kg, is rare. This report describes a case of a 15-month-old whole liver transplant recipient who suffered SFSS, and systematic literature review was performed to identify outcomes of such cases and potential risk factors for SFSS.

Case presentation: A 15-month-old toddler with a history of biliary atresia underwent a deceased donor whole liver transplant. The graft weighed 160 g, resulting in a graft-to-recipient weight ratio (GRWR) of 1.6%. The post-operative course was complicated by SFSS, characterized by massive ascites causing hemodynamic instability and compromised hepatic artery flow. Pharmacological intervention with octreotide was initiated, and the patient eventually recovered.

Conclusion: In small pediatric recipients, especially those weighing less than 10 kg, the native liver body weight ratio (LBWR) is significantly higher. When selecting an appropriately sized graft for these recipients, this higher ratio should be taken into consideration. The literature review suggests that a GRWR of less than 2% is associated with a higher incidence of small-for-size syndrome in small pediatric recipients weighing less than 10 kg.

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来源期刊
Pediatric Transplantation
Pediatric Transplantation 医学-小儿科
CiteScore
2.90
自引率
15.40%
发文量
216
审稿时长
3-8 weeks
期刊介绍: The aim of Pediatric Transplantation is to publish original articles of the highest quality on clinical experience and basic research in transplantation of tissues and solid organs in infants, children and adolescents. The journal seeks to disseminate the latest information widely to all individuals involved in kidney, liver, heart, lung, intestine and stem cell (bone-marrow) transplantation. In addition, the journal publishes focused reviews on topics relevant to pediatric transplantation as well as timely editorial comment on controversial issues.
期刊最新文献
Donor Characteristics and Outcomes of Pediatric Heart Transplantation in South Korea. Outcomes of Pediatric Liver Transplantation in Glycogen Storage Disease Type 1b-A Single-Center Experience. Cardiovascular Risk in Pediatric Renal Transplant Recipients. Liver Transplantation for Nijmegen Breakage Syndrome With Hepatic Malignancy and Hepatopulmonary Syndrome After Bone Marrow Transplantation: A Case Report. Pediatric Organ Donation: 16-Year Experience of PICU/ICU of a Third Level Hospital in Portugal, 2006-2021.
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