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Cystic Kidney Diseases Associated with Increased Cancer Risk: Tuberous Sclerosis Complex, Von Hippel-Lindau, and Birt-Hogg-Dubé

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Polycystic Kidney Disease

Abstract

Tuberous sclerosis complex, von Hippel-Lindau disease, and Birt-Hogg-Dubé disease are tumor predisposition syndromes associated with renal cystic disease. While better known for their benign and malignant tumor associations, these disease-associated proteins all have a strong mechanistic link to the mTORC1 pathway and primary cilia signaling. The clinical, basic, and translational science aspects are explored, and a common mechanism is posited.

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Authors and Affiliations

  1. Chief, Division of Nephrology, Department of Pediatrics, Director, Tuberous Sclerosis Center of Excellence at Le Bonheur Children’s Hospital, Medical Director, Nephrology, St. Jude Children’s Research Hospital, University of Tennessee Health Science Center, Children’s Foundation Research Center, Memphis, TN, USA

    John J. Bissler MD, FAAP, FASN

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  1. John J. Bissler MD, FAAP, FASN
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Correspondence to John J. Bissler MD, FAAP, FASN .

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Editors and Affiliations

  1. John Gammill Professor in Polycystic Kidney Disease, Chief, Section of Nephrology and Hypertension, Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA

    Benjamin D. Cowley, Jr.

  2. Federal Express Chair of Excellence, Chief, Division of Nephrology, Department of Pediatrics, Director, Tuberous Sclerosis Center of Excellence at Le Bonheur Children’s Hospital, Medical Director, Nephrology St. Jude Children’s Research Hospital, University of Tennessee Health Science Center, Children’s Foundation Research Center, Memphis, Tennessee, USA

    John J. Bissler

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Bissler, J.J. (2018). Cystic Kidney Diseases Associated with Increased Cancer Risk: Tuberous Sclerosis Complex, Von Hippel-Lindau, and Birt-Hogg-Dubé. In: Cowley, Jr., B., Bissler, J. (eds) Polycystic Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-7784-0_3

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