肾囊性疾病:分类与发病机制*

Congenital anomalies Pub Date : 1993-03-01 DOI:10.1111/j.1741-4520.1993.tb00507.x

J. Bernstein

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引用次数: 2

摘要

在遗传性和发展性肾囊性疾病的主要类型中(表1)，多囊性肾病(PKD)因其发病率和医学重要性而突出。PKD的两种主要类型是常染色体显性(AD-PKD)和常染色体隐性(AR-PKD)。囊性肾病通常与典型的PKD难以区分，也存在一些明确的综合征，其中包括结节性硬化症和von Hippel-Lindau病。

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Renal Cystic Disease : Classification and Pathogenesis *

Among the principal types of hereditary and developmental renal cystic disease (Table I), polycystic kidney disease (PKD) stands out because of its frequency and medical importance. The two principal categories of PKD are autosomal dominant (AD-PKD) and autosomal recessive (AR-PKD). Cystic kidney disease, often indistinguishable from classic PKD, is also inherent in several well-defined syndromes, among them tuberous sclerosis and von Hippel-Lindau disease.

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Congenital anomalies

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