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多囊性发育不良肾:产前检测病例的自然病史。

Multicystic dysplastic kidney: natural history of prenatally detected cases.

作者信息

Dungan J S, Fernandez M T, Abbitt P L, Thiagarajah S, Howards S S, Hogge W A

机构信息

Department of Obstetrics and Gynecology, University of Virginia Health Sciences Center, Charlottesville 22908.

出版信息

Prenat Diagn. 1990 Mar;10(3):175-82. doi: 10.1002/pd.1970100307.

DOI:10.1002/pd.1970100307
PMID:2188249
Abstract

To delineate the natural history of fetal multicystic dysplastic kidneys (MDKs), all cases that were prenatally detected in the Prenatal Diagnosis Center of the University of Virginia from September 1985 to 31 August 1988 were reviewed. All patients were followed through the Center with serial ultrasound evaluations at approximately 4-week intervals, and each liveborn infant was evaluated and followed by one of the authors (S.S.H.). Of the 14 cases detected, ten were detected in the second trimester, the earliest at 16.5 weeks' gestation. Of the nine fetuses with non-lethal disease, there were two cases in which the lesion remained unchanged during observation. Both had an initial diagnosis in the third trimester. In those cases diagnosed in the second trimester (7), all showed an initial increase in the size and number of cysts, followed by involutional changes either in utero (2) or in the neonatal period (3). Two infants had immediate surgical removal of the MDK, one because of respiratory compromise, and the other because of an uncertain diagnosis on renal scan. Abnormalities of the contralateral kidney were found in 7 of 14 fetuses. Five were lethal conditions. Associated non-renal abnormalities were common in bilateral MDK (80 per cent), but rare in unilateral MDK (11 per cent).

摘要

为了描述胎儿多囊性发育不良肾(MDK)的自然病史,我们回顾了1985年9月至1988年8月31日在弗吉尼亚大学产前诊断中心产前检测出的所有病例。所有患者均通过该中心进行随访,每隔约4周进行一次超声检查,每位活产婴儿均由作者之一(S.S.H.)进行评估和随访。在检测出的14例病例中,10例在孕中期被检测出,最早为孕16.5周。在9例非致命性疾病的胎儿中,有2例在观察期间病变保持不变。这2例均在孕晚期初次诊断。在孕中期诊断出的病例(7例)中,所有病例最初均显示囊肿大小和数量增加,随后在子宫内(2例)或新生儿期(3例)出现退化性变化。2例婴儿立即接受了MDK手术切除,1例是因为呼吸功能不全,另1例是因为肾扫描诊断不明确。14例胎儿中有7例对侧肾存在异常。5例为致命情况。双侧MDK中常见相关非肾异常(80%),但单侧MDK中罕见(11%)。

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Curr Health Sci J. 2016 Jan-Mar;42(1):19-30. doi: 10.12865/CHSJ.42.01.03. Epub 2016 Mar 29.
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The PAX2 tanscription factor is expressed in cystic and hyperproliferative dysplastic epithelia in human kidney malformations.PAX2转录因子在人类肾脏畸形的囊性和过度增殖发育异常上皮中表达。
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