Developmental Anomalies and Cystic Kidney Diseases

  • Stephen M. Bonsib
Part of the Atlas of Anatomic Pathology book series (AAP)


Cystic and developmental diseases of the kidney encompass a vast number of complex entities that may be renal-limited or part of a multiorgan malformation sequence. They also may be sporadic, hereditary and syndromic, or acquired, and may be associated with a neoplastic diathesis. In the past few decades, there has been enormous progress in unraveling the pathogenesis of many of these entities, and their genetic and molecular basis. There is a heterogeneous group of diseases resulting from mutation of one or more key master genes crucial to the proper development of the kidneys and lower urinary tract. Collectively, these are referred to as congenital anomalies of the kidneys and urinary tract (CAKUT). CAKUT lesions are common. They occur in approximately 10 % of the population and account for up to 50 % of renal failure in children.


Tuberous Sclerosis Complex Autosomal Dominant Polycystic Kidney Disease Horseshoe Kidney Renal Dysplasia Autosomal Recessive Polycystic Kidney Disease 
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General: Books and Book Chapters

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Classification of Cystic Kidney Diseases and Congenital Malformation of the Kidney and Urinary Tract

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Abnormalities in Form, Position, and Number

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Acquired Cystic Kidney Disease

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Localized Polycystic Kidney Disease

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Renal Dysplasias

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Glomerulocystic Kidney

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Tubulointerstitial Syndromes With or Without Cysts

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Phycomatoses and Renal Diseases

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Miscellaneous Abnormalities

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Stephen M. Bonsib
    • 1
  1. 1.NephropathLittle RockUSA

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