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胎盘间充质发育异常与宫内生长受限及胎儿死亡的高发生率相关:11例新病例报告及文献综述

Placental mesenchymal dysplasia is associated with high rates of intrauterine growth restriction and fetal demise: A report of 11 new cases and a review of the literature.

作者信息

Pham Truc, Steele Julie, Stayboldt Carla, Chan Linda, Benirschke Kurt

机构信息

Department of Pathology, University of California, San Diego, CA 92103-8720, USA.

出版信息

Am J Clin Pathol. 2006 Jul;126(1):67-78. doi: 10.1309/RV45-HRD5-3YQ2-YFTP.

Abstract

Placental mesenchymal dysplasia (PMD) is a rare condition of placentomegaly and abnormal chorionic villi often clinically mistakenly as partial hydatidiform mole. However, it is clinicopathologically distinct with high incidence of intrauterine growth restriction (IUGR) and fetal death. This study presents 11 new PMD cases and provides a meta-analysis of the associated IUGR and fetal death rates. The cases were identified between 1971 and 2005, mostly from consultation files. To our knowledge, 71 PMD cases have previously been reported; 15 of these were associated with Beckwith-Wiedemann syndrome (BWS). With the addition of our new results, among all cases without BWS, 50% had IUGR and 43% had intrauterine fetal demise (IUFD) or neonatal death. Females represented 82% of cases. Thus, PMD is associated with high IUGR and IUFD/neonatal death rates and disproportionally affects females. The cause and pathogenesis are yet unknown. The current understanding and hypotheses involving PMD are discussed.

摘要

胎盘间充质发育异常(PMD)是一种罕见的胎盘肿大和绒毛膜绒毛异常的病症,临床上常被误诊为部分性葡萄胎。然而,它在临床病理上有明显特征,宫内生长受限(IUGR)和胎儿死亡的发生率很高。本研究报告了11例新的PMD病例,并对相关的IUGR和胎儿死亡率进行了荟萃分析。这些病例是在1971年至2005年间确定的,大多来自会诊档案。据我们所知,此前已报告71例PMD病例;其中15例与贝克威思-维德曼综合征(BWS)有关。加上我们的新结果,在所有无BWS的病例中,50%有IUGR,43%有宫内胎儿死亡(IUFD)或新生儿死亡。女性占病例的82%。因此,PMD与高IUGR和IUFD/新生儿死亡率相关,且对女性的影响不成比例。其病因和发病机制尚不清楚。本文讨论了目前对PMD的认识和假说。

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