| 摘要: |
| ［摘要］ 先天性巨结肠，是一种神经嵴细胞源性疾病和多基因遗传病，其病因至今虽然尚未完全清楚，但对该病病因和发病机制的研究已取得较大的进展。本文从遗传学、Cajal间质细胞、缝隙连接蛋白43和环境因素等方面阐述HD的发病原因。 |
| 关键词: 先天性巨结肠 病因学 无神经节细胞症 |
| DOI：10.3969/j.issn.1674-3806.2009.05.015 |
| 分类号:R 656.9 |
| 基金项目: |
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| The development on the etiology of hirschsprung′s disease |
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JIANG Li, WANG Hong
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Pediatric Surgery, the First Affiliated Hospital of Guangxi Medical University, Nanning 530021, China
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| Abstract: |
| ［Abstract］ Hirschsprung′s disease (HD), a congenital disease, is characterized by the absence of ganglion cells in the ganglion plexuses of the caudal most gut. It stands as a model for complex multifactorial or multigenic disorders. In the last two decades, there has been a greater understanding of the etiopathogenisis and pathogenesy of hirschsprung′s disease. The goals of this review are to summarize progress in the study of etiological factor, such as genic mutation, the disorders of the interstitial cells of Cajal and connexin 43 (Cx43), the effect of environment. |
| Key words: Hirschsprung′s disease Etiology Aganglionosis |
