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多囊性发育不良肾 - 根据具体情况治疗每个病例。

Multicystic dysplastic kidney - treat each case on its merits.

机构信息

Medical School, University of Western Australia, 35 Stirling Highway, Crawley, Western Australia, 6009; Department of Paediatric Surgery, Perth Children's Hospital, Hospital Avenue, Nedlands, Western Australia, 6009.

Medical School, University of Western Australia, 35 Stirling Highway, Crawley, Western Australia, 6009; Department of Paediatric Surgery, Perth Children's Hospital, Hospital Avenue, Nedlands, Western Australia, 6009.

出版信息

J Pediatr Surg. 2020 Nov;55(11):2497-2503. doi: 10.1016/j.jpedsurg.2019.12.008. Epub 2020 Jan 10.

DOI:10.1016/j.jpedsurg.2019.12.008
PMID:31987520
Abstract

OBJECTIVES

To assess outcomes of unilateral multicystic dysplastic kidney (MCDK) managed at an Australasian centre over a 15 year period. To assess if MCDK involution could be predicted based on change noted between first two postnatal ultrasound scans 6 months apart.

SUBJECTS AND METHODS

A retrospective study was performed.

RESULTS

One-hundred-and-six cases of unilateral MCDK were studied. Eighty-four of these presented antenatally. Twenty-two MCDK cases presented postnatally. Urological anomalies associated with MCDK included vesicoureteric reflux (VUR), ureterocele and contralateral pelviureteric junction obstruction (PUJO). Children undergoing surgical intervention for these anomalies were offered concurrent MCDK nephrectomy. Morbidity associated with MCDK under surveillance included febrile culture-positive urinary tract infection in 20 cases (20.7%), hypertension in four (3.7%) and Wilms' tumor in one (0.9%). Thirty-six cases (34%) underwent complete involution, 32 (30.2%) were in the process of involuting and 38 cases (35.8%) underwent nephrectomy because of failure of involution or associated morbidity. If the MCDK reduced in cranio-caudal interpolar length by 20% or more between the first postnatal USS and the next one 6 months later, then it was very likely to involute spontaneously. If the MCDK did not reduce in cranio-caudal interpolar length by 20% between the first postnatal scan and the next one 6 months later, then it was highly likely to fail to involute, and in our study, correlated with the outcome of nephrectomy.

CONCLUSION

Although MCDK is a benign condition, it should be carefully investigated and followed-up, as involution may not occur in over a third. In some cases, morbidity may occur. Each case of MCDK should be managed on its own merits.

LEVEL OF EVIDENCE

Level II - Prognosis study, Retrospective.

摘要

目的

评估在澳大利亚中心对单侧多囊性发育不良肾(MCDK)进行管理的 15 年期间的结果。评估 MCDK 萎缩是否可以基于相隔 6 个月的前两次产后超声检查中观察到的变化来预测。

对象和方法

进行了一项回顾性研究。

结果

研究了 106 例单侧 MCDK 病例。其中 84 例在产前出现。22 例 MCDK 病例在产后出现。与 MCDK 相关的泌尿系统异常包括输尿管反流(VUR)、输尿管囊肿和对侧肾盂输尿管连接部梗阻(PUJO)。对这些异常接受手术干预的儿童被提供同时进行 MCDK 肾切除术。在监测下与 MCDK 相关的发病率包括 20 例(20.7%)有发热阳性的尿路感染、4 例(3.7%)高血压和 1 例(0.9%)Wilms 瘤。36 例(34%)完全萎缩,32 例(30.2%)正在萎缩过程中,38 例(35.8%)因萎缩失败或相关发病率而行肾切除术。如果 MCDK 在第一次产后 USS 与 6 个月后的下一次 USS 之间在头尾径长度上减少 20%或更多,则很可能会自发萎缩。如果 MCDK 在第一次产后扫描与下一次扫描之间在头尾径长度上没有减少 20%,则极有可能无法萎缩,并且在我们的研究中,这与肾切除术的结果相关。

结论

尽管 MCDK 是一种良性疾病,但应仔细进行调查和随访,因为超过三分之一的病例可能不会发生萎缩。在某些情况下,可能会发生发病率。应根据每个 MCDK 病例的具体情况进行处理。

证据水平

二级 - 预后研究,回顾性。

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