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多囊肾病:I. 病因与发病机制

Polycystic kidney disease: I. Etiology and pathogenesis.

作者信息

Grantham J J

机构信息

Department of Medicine, University of Kansas School of Medicine, Kansas City.

出版信息

Hosp Pract (Off Ed). 1992 Mar 15;27(3):51-9. doi: 10.1080/21548331.1992.11705379.

DOI:10.1080/21548331.1992.11705379
PMID:1541655
Abstract

Geneticists and cell biologists are simultaneously studying the disease's underlying mechanisms. Although a great many questions remain about cyst formation and its role in kidney failure, three fundamental constructs on its etiology and pathogenesis have been generated: renal tubular epithelial proliferation, fluid accumulation, and remodeling of the extracellular matrix.

摘要

遗传学家和细胞生物学家正在同时研究该疾病的潜在机制。尽管关于囊肿形成及其在肾衰竭中的作用仍有许多问题,但关于其病因和发病机制已形成了三个基本概念:肾小管上皮细胞增殖、液体蓄积和细胞外基质重塑。

相似文献

1
Polycystic kidney disease: I. Etiology and pathogenesis.多囊肾病:I. 病因与发病机制
Hosp Pract (Off Ed). 1992 Mar 15;27(3):51-9. doi: 10.1080/21548331.1992.11705379.
2
A mechanistic approach to inherited polycystic kidney disease.一种针对遗传性多囊肾病的机制性研究方法。
Pediatr Nephrol. 2005 May;20(5):558-66. doi: 10.1007/s00467-004-1665-z. Epub 2005 Feb 18.
3
The pathogenesis of polycystic kidney disease.多囊肾病的发病机制。
Histol Histopathol. 1995 Jan;10(1):213-21.
4
Genetics and pathogenesis of polycystic kidney disease.多囊肾病的遗传学与发病机制
J Am Soc Nephrol. 2002 Sep;13(9):2384-98. doi: 10.1097/01.asn.0000028643.17901.42.
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Polycystic kidney disease: primary extracellular matrix abnormality or defective cellular differentiation?多囊肾病:原发性细胞外基质异常还是细胞分化缺陷?
Kidney Int. 1993 Jan;43(1):101-8. doi: 10.1038/ki.1993.17.
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Autosomal dominant polycystic kidney disease: molecular genetics and pathophysiology.常染色体显性多囊肾病:分子遗传学与病理生理学
J Lab Clin Med. 2003 Feb;141(2):91-101. doi: 10.1067/mlc.2003.13.
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Pathophysiology of renal tubular cyst formation in murine models of polycystic kidney disease.
Contrib Nephrol. 1992;97:23-34. doi: 10.1159/000421642.
8
Polycystic kidney disease--a truly pediatric problem.多囊肾病——一个典型的儿科问题。
Pediatr Nephrol. 1994 Dec;8(6):762-7. doi: 10.1007/BF00869116.
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Recent advances in understanding the pathogenesis of polycystic kidney disease: therapeutic implications.多囊肾病发病机制认识的最新进展:治疗意义
Drugs. 2004;64(12):1285-94. doi: 10.2165/00003495-200464120-00002.
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Cystic disease of the kidney.肾囊性疾病
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引用本文的文献

1
Deficiency in Six2 during prenatal development is associated with reduced nephron number, chronic renal failure, and hypertension in Br/+ adult mice.产前发育期间Six2的缺乏与Br/+成年小鼠的肾单位数量减少、慢性肾衰竭和高血压有关。
Am J Physiol Renal Physiol. 2009 May;296(5):F1166-78. doi: 10.1152/ajprenal.90550.2008. Epub 2009 Feb 4.
2
Polycystin expression during embryonic development of human kidney in adult tissues and ADPKD tissue.
Histochem J. 1997 Nov-Dec;29(11-12):847-56. doi: 10.1023/a:1026489723733.
3
Differential rescue of the renal and hepatic disease in an autosomal recessive polycystic kidney disease mouse mutant. A new model to study the liver lesion.常染色体隐性多囊肾病小鼠突变体中肾脏和肝脏疾病的差异挽救。一种研究肝脏病变的新模型。
Am J Pathol. 1997 Jun;150(6):2231-41.
4
Genetic mapping of the polycystic kidney gene, pcy, on mouse chromosome 9.多囊肾基因pcy在小鼠9号染色体上的遗传定位。
Biochem Genet. 1995 Dec;33(11-12):401-12. doi: 10.1007/BF00554598.