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1例合并左侧先天性膈疝的分裂脊索综合征：一种罕见的关联。

A Case of Split Notochord Syndrome with Left Congenital Diaphragmatic Hernia: A Rare Association.

作者信息

Pan Pradyumna

机构信息

Pediatric Surgery Unit, Ashish Hospital and Research Centre, Jabalpur, Madhya Pradesh, India.

出版信息

J Pediatr Neurosci. 2020 Jan-Mar;15(1):42-44. doi: 10.4103/JPN.JPN_85_19. Epub 2020 Mar 18.

DOI:10.4103/JPN.JPN_85_19
PMID:32435306
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7227745/
Abstract

Split notochord syndrome is a rare neural tube malformation involving the brain, spinal cord, and vertebral column. In nearly half of the cases, the malformation also involves the gastrointestinal tract in the form of a dorsal neurenteric fistula and imperforate anus. In the literature, less than 50 cases have been reported. To the best of my knowledge, this is the first reported case associated with a left congenital diaphragmatic hernia.

摘要

脊索分裂综合征是一种罕见的神经管畸形，累及脑、脊髓和脊柱。近半数病例中，该畸形还以背侧神经肠瘘和肛门闭锁的形式累及胃肠道。文献中报道的病例不足50例。据我所知，这是首例与左侧先天性膈疝相关的报道病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d8b1/7227745/72baa368f3ad/JPN-15-42-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d8b1/7227745/33dd2dc025cb/JPN-15-42-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d8b1/7227745/72baa368f3ad/JPN-15-42-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d8b1/7227745/33dd2dc025cb/JPN-15-42-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d8b1/7227745/72baa368f3ad/JPN-15-42-g002.jpg

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引用本文的文献

1
A rare case of split notochord syndrome with neurenteric cyst: clinical challenges, surgical approach, and literature review.一例罕见的伴有神经肠囊肿的分裂脊索综合征病例：临床挑战、手术方法及文献综述
Childs Nerv Syst. 2024 Dec 3;41(1):31. doi: 10.1007/s00381-024-06664-6.

本文引用的文献

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A case of split notochord syndrome: Presenting with respiratory failure in the neonatal period.一例脊索分裂综合征：以新生儿期呼吸衰竭为表现。
Intractable Rare Dis Res. 2016 May;5(2):121-3. doi: 10.5582/irdr.2016.01010.
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Pattern formation in the vertebrate neural tube: a sonic hedgehog morphogen-regulated transcriptional network.脊椎动物神经管中的模式形成：一种由音猬因子形态发生素调控的转录网络。
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Wnt signaling inhibitors regulate the transcriptional response to morphogenetic Shh-Gli signaling in the neural tube.
Wnt信号通路抑制剂调节神经管中对形态发生性Shh-Gli信号的转录反应。
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Split notochord syndrome with dorsal enteric fistula and sacral agenesis.
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Neurenteric cysts with meningomyelocele or meningocele. Split notochord syndrome.
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Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations.脊髓纵裂畸形：第一部分：双脊髓畸形胚胎发生的统一理论。
Neurosurgery. 1992 Sep;31(3):451-80. doi: 10.1227/00006123-199209000-00010.