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一例伴有肾发育不全的戴克-戴维多夫-马森综合征:一种罕见的关联。

A Case of Dyke-Davidoff-Masson Syndrome with Hypoplasia of the Kidney: An Unusual Association.

作者信息

Patel Prem Shankar, Krishna Amresh, Kumar Om

机构信息

Department of Nephrology, Indira Gandhi Institute of Medical Science, Patna, Bihar, India.

Department of Microbiology, All India Institute of Medical Sciences, Patna, Bihar, India.

出版信息

Int J Appl Basic Med Res. 2023 Oct-Dec;13(4):259-262. doi: 10.4103/ijabmr.ijabmr_189_23. Epub 2023 Dec 8.

DOI:10.4103/ijabmr.ijabmr_189_23
PMID:38229724
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10789462/
Abstract

Dyke-Davidoff-Masson syndrome (DDMS) is a rare neuro-osteal syndrome of childhood and a constellation of cerebral hemiatrophy, facial asymmetry, seizures, osseous changes, and hemiplegia. It commonly presents with seizures and hemiplegia. The involvement of the kidney in DDMS is not known in the available literature, except in a case report that described ectopic kidney in DDMS. We present the case of a 15-year-old boy who presented with recurrent seizures, right facial palsy, left hemiparesis, and advanced renal failure. The neuroimaging revealed diffuse right cerebral atrophy, dilatation of the ipsilateral lateral ventricle, and ipsilateral thickening of the calvaria. The nephrological evaluation suggested the diagnosis of chronic kidney disease stage VD, probably secondary to congenital hypoplasia of the kidney.

摘要

戴克-戴维多夫-马森综合征(DDMS)是一种罕见的儿童神经骨综合征,表现为一系列症状,包括大脑半球萎缩、面部不对称、癫痫发作、骨质改变和偏瘫。它通常表现为癫痫发作和偏瘫。在现有文献中,除了一篇描述DDMS患者异位肾的病例报告外,肾脏在DDMS中的受累情况尚不清楚。我们报告一例15岁男孩,他出现反复发作的癫痫、右侧面神经麻痹、左侧偏瘫和晚期肾衰竭。神经影像学检查显示右侧大脑弥漫性萎缩、同侧侧脑室扩张和同侧颅骨增厚。肾脏科评估提示诊断为慢性肾脏病ⅤD期,可能继发于先天性肾发育不全。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70dd/10789462/82aba7bae804/IJABMR-13-259-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70dd/10789462/c53e8491a0b7/IJABMR-13-259-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70dd/10789462/82aba7bae804/IJABMR-13-259-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70dd/10789462/c53e8491a0b7/IJABMR-13-259-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70dd/10789462/82aba7bae804/IJABMR-13-259-g002.jpg

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本文引用的文献

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Dyke-Davidoff-Masson syndrome: A case report with a literature review.戴克-戴维多夫-马森综合征:一例病例报告并文献复习
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