1例6岁1型神经纤维瘤病肾动脉狭窄患者行肾自体移植的早期治疗。

IF 0.9 Q3 MEDICINE, GENERAL & INTERNAL
Samantha J Wala, Morgan Beebe, Patrick Warren, Mahmoud Kallash, Jaimie Nathan, Sara Rasmussen
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引用次数: 0

摘要

背景:1型神经纤维瘤病所致肾动脉狭窄是小儿继发性肾血管性高血压的重要来源。目前还没有关于儿童肾动脉狭窄的治疗指南,并且由于患者规模小,支架和旁路移植术的应用受到限制。自体肾移植治疗肾动脉狭窄的小儿科患者可能是一种可行的替代治疗方法,省去了肾切除术的需要。病例介绍:在这篇文章中,我们报告了一例6岁,15.8公斤的尼泊尔患者,患有1型神经纤维瘤病,伴有顽固性高血压和右肾主动脉近端高度狭窄。患者接受球囊血管成形术,但未能扩张狭窄。他后来发展为高血压急症,需要住进儿科重症监护室。考虑到狭窄段的长度及其弯曲,该患者不适合重复血管成形术。多种降压药无法控制血压,患者最终发展为高血压急症。因此,在多学科评估后进行右肾自体移植。右肾动脉口内膜明显肥大,约占其周长的50%。该肾脏的获取方式与活体肾供体肾切除术相同。原位动员肾脏,并给予肝素。用手术吻合器分离肾动脉和肾静脉。从病人身上取出肾脏,移到手术台上。在后台上,用冷器官保存液冲洗器官,并检查血管。切除右肾动脉病变部位至无明显内膜增厚的部位。判断有足够长度的健康动脉剩余允许安全的再植入术。肾动脉和肾静脉分别移植到腹主动脉和下腔静脉。患者对手术耐受良好,术后2年仅需要一种降压药。结论:由于自体肾移植在技术上的困难,对于小儿科患者,肾切除术可能优于自体肾移植。我们证明了一名体重15.8公斤、6岁的儿童患者在肾自体移植后血压控制有显著的临床改善。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Early experience with renal autotransplant for renal artery stenosis in a 6-year-old patient with neurofibromatosis type 1: a case report.

Background: Renal artery stenosis due to neurofibromatosis type 1 is a known important source of secondary renovascular hypertension in pediatric patients. There are no guidelines on the management of renal artery stenosis in children, and the utility of stents and bypass grafting is limited given small patient size. Renal autotransplant to treat renal artery stenosis in a small pediatric patient may be a viable alternative for treatment and spare the need for nephrectomy.

Case presentation: In this article, we present a case of renal autotransplant in a 6-year-old, 15.8 kg Nepali patient with neurofibromatosis type 1 with refractory hypertension and high-grade stenosis of the proximal right main renal artery. The patient underwent balloon angioplasty, which failed to dilate the stenosis. He later developed hypertensive urgency and required admission to the pediatric intensive care unit. The patient was not a candidate for repeat angioplasty given the length of the stenotic segment and its tortuosity. Blood pressure was unable to be controlled on multiple antihypertensive agents and the patient eventually developed hypertensive urgency. Therefore, a renal autotransplant of the right kidney was performed after multidisciplinary evaluation. The right renal artery ostium had significant hypertrophied intima involving 50% of its circumference. The kidney was procured in the same fashion as a living kidney donor nephrectomy. The kidney was mobilized in situ, and heparin was administered. The renal artery and renal vein were divided with surgical staplers. The kidney was removed from the patient and moved to the back table. On the back table, the organ was flushed with cold organ preservation solution and vessels inspected. The diseased portion of the right renal artery was resected to the location of no gross intimal thickening. It was judged that there was adequate length of the healthy artery remaining to allow safe reimplantation. The renal artery and vein were reimplanted to the abdominal aorta and inferior vena cava, respectively. The patient tolerated the surgery well, and 2 years postoperatively, he only requires one antihypertensive medication.

Conclusion: Nephrectomy may be favored over renal autotransplant in small pediatric patients due to technical difficulties associated with autotransplant. We demonstrate significant clinical improvement in blood pressure control in a 15.8 kg, 6-year-old pediatric patient after renal autotransplant.

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来源期刊
Journal of Medical Case Reports
Journal of Medical Case Reports Medicine-Medicine (all)
CiteScore
1.50
自引率
0.00%
发文量
436
期刊介绍: JMCR is an open access, peer-reviewed online journal that will consider any original case report that expands the field of general medical knowledge. Reports should show one of the following: 1. Unreported or unusual side effects or adverse interactions involving medications 2. Unexpected or unusual presentations of a disease 3. New associations or variations in disease processes 4. Presentations, diagnoses and/or management of new and emerging diseases 5. An unexpected association between diseases or symptoms 6. An unexpected event in the course of observing or treating a patient 7. Findings that shed new light on the possible pathogenesis of a disease or an adverse effect
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