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中孕期筛查胎儿多囊性发育不良肾的产前诊断。

Prenatal diagnosis of multicystic dysplastic kidney disease in the second trimester screening.

机构信息

Department of Obstetrics and Gynecology, Bürgerhospital Frankfurt, Frankfurt am Main, Germany.

出版信息

Prenat Diagn. 2013 Aug;33(8):726-31. doi: 10.1002/pd.4112. Epub 2013 Jun 3.

DOI:10.1002/pd.4112
PMID:23584791
Abstract

OBJECTIVE

Multicystic dysplastic kidney (MCDK) is the most common antenatally diagnosed cystic renal pathology. It refers to the presence of multiple kidney cysts surrounded by dysplastic parenchymal tissue. The typical MCDK ultrasound finding shows multiple nonconnecting thin-walled cysts within an irregularly outlined kidney. We want to present our own cases as an addition to the relatively scarce literature about MCDK.

METHOD

We retrospectively review 54 cases (21 bilateral and 33 unilateral) with antenatally diagnosed MCDK between the years 1998 and 2012. We compare our data with the published literature and discuss different parameters.

RESULTS

Our findings show a male predominance of 1.8 : 1 and a left side/right side ratio of 0.73 : 1. Most of the cases were isolated findings, but 20.3% were associated with anomalies. One case was a Goldenhar syndrome. Of the bilateral MCDK cases, 14.3% had chromosomal defects. The mean gestational age at time of diagnosis was 20-21 weeks. Renal length was above the 90th normal percentile in 96.2% of the cases.

CONCLUSION

Isolated unilateral MCDK has good prognosis. Bad prognosis is linked to association with other anomalies as well as to bilateral MCDK.

摘要

目的

多囊性发育不良肾(MCDK)是最常见的产前诊断囊性肾病。它是指多个多囊肾周围存在发育不良的实质组织。典型的 MCDK 超声表现为在不规则轮廓的肾脏内有多个非连通的薄壁囊肿。我们希望通过介绍自己的病例,为相对较少的 MCDK 文献提供补充。

方法

我们回顾性分析了 1998 年至 2012 年间产前诊断为 MCDK 的 54 例(21 例双侧和 33 例单侧)。我们将我们的数据与已发表的文献进行比较,并讨论了不同的参数。

结果

我们的研究结果显示男性占 1.8:1,左侧/右侧比例为 0.73:1。大多数病例是孤立性发现,但 20.3%的病例与异常有关。一例为 Goldenhar 综合征。双侧 MCDK 病例中,14.3%存在染色体缺陷。诊断时的平均孕周为 20-21 周。96.2%的病例肾脏长度超过第 90 个百分位数。

结论

孤立性单侧 MCDK 预后良好。预后不良与其他异常以及双侧 MCDK 有关。

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Minimally invasive open nephrectomy on children with multicystic dysplastic kidney.对多囊性发育不良肾患儿行微创开放性肾切除术。
Exp Ther Med. 2016 Dec;12(6):3575-3578. doi: 10.3892/etm.2016.3816. Epub 2016 Oct 18.
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Positive predictive value and completeness of prenatally assigned International Classification of Disease-10 kidney anomaly diagnoses in the Danish National Patient Registry.
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Clin Epidemiol. 2016 Jan 18;8:9-14. doi: 10.2147/CLEP.S94394. eCollection 2016.
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Outcome of Multi-Cystic Dysplastic Kidneys in Children.儿童多囊性发育不良肾的转归
Iran J Pediatr. 2015 Oct;25(5):e2991. doi: 10.5812/ijp.2991. Epub 2015 Oct 6.