Cystic Kidney Diseases From the Adult Nephrologist’s Point of View

Cystic kidney diseases affect patients of all age groups with the onset spanning from prenatal disease to late adulthood. Autosomal-dominant polycystic kidney disease (ADPKD) is by far the most common renal cystic disease. However, there are various cystic kidney diseases, the onset of which occurs...

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Main Authors: Roman-Ulrich Müller, Thomas Benzing
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-03-01
Series:Frontiers in Pediatrics
Subjects:
Online Access:http://journal.frontiersin.org/article/10.3389/fped.2018.00065/full
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author Roman-Ulrich Müller
Thomas Benzing
author_facet Roman-Ulrich Müller
Thomas Benzing
author_sort Roman-Ulrich Müller
collection DOAJ
description Cystic kidney diseases affect patients of all age groups with the onset spanning from prenatal disease to late adulthood. Autosomal-dominant polycystic kidney disease (ADPKD) is by far the most common renal cystic disease. However, there are various cystic kidney diseases, the onset of which occurs at different times in life and depends on the type of the disease and the causative genes involved. When genetic kidney diseases are discussed in the adult setting this view is usually limited on autosomal-dominant kidney disease, the most frequent genetic disorder causing adult onset ESRD. Other diseases—such as autosomal-recessive polycystic kidney disease—are often being viewed as a disorder only important in pediatric nephrology. However, more recent data has revealed that, despite clear age peaks of onset for each disorder, all of them can also show highly variable phenotypes with classical adult onset genetic diseases being of importance in pediatrics and vice versa. Furthermore, the affected children need to be seen by adult nephrologists in the long term after transition, requiring knowledge on the underlying pediatric disease, potential extrarenal manifestations, and genetic counseling. Consequently, the view on these diseases should be widened on both ends. Close interaction between pediatric and adult nephrology is key to appropriate care of patients suffering from genetic kidney disease to profit from each other’s experience.
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spelling doaj.art-24a914dcf62846dc85947bcd0ee50b372022-12-21T17:45:05ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602018-03-01610.3389/fped.2018.00065337027Cystic Kidney Diseases From the Adult Nephrologist’s Point of ViewRoman-Ulrich MüllerThomas BenzingCystic kidney diseases affect patients of all age groups with the onset spanning from prenatal disease to late adulthood. Autosomal-dominant polycystic kidney disease (ADPKD) is by far the most common renal cystic disease. However, there are various cystic kidney diseases, the onset of which occurs at different times in life and depends on the type of the disease and the causative genes involved. When genetic kidney diseases are discussed in the adult setting this view is usually limited on autosomal-dominant kidney disease, the most frequent genetic disorder causing adult onset ESRD. Other diseases—such as autosomal-recessive polycystic kidney disease—are often being viewed as a disorder only important in pediatric nephrology. However, more recent data has revealed that, despite clear age peaks of onset for each disorder, all of them can also show highly variable phenotypes with classical adult onset genetic diseases being of importance in pediatrics and vice versa. Furthermore, the affected children need to be seen by adult nephrologists in the long term after transition, requiring knowledge on the underlying pediatric disease, potential extrarenal manifestations, and genetic counseling. Consequently, the view on these diseases should be widened on both ends. Close interaction between pediatric and adult nephrology is key to appropriate care of patients suffering from genetic kidney disease to profit from each other’s experience.http://journal.frontiersin.org/article/10.3389/fped.2018.00065/fullpolycystic kidney diseasesautosomal dominant polycystic kidney diseaseautosomal-recessive polycystic kidney diseasetuberous sclerosis complexvon Hippel–Lindau diseasenephronophthisis
spellingShingle Roman-Ulrich Müller
Thomas Benzing
Cystic Kidney Diseases From the Adult Nephrologist’s Point of View
Frontiers in Pediatrics
polycystic kidney diseases
autosomal dominant polycystic kidney disease
autosomal-recessive polycystic kidney disease
tuberous sclerosis complex
von Hippel–Lindau disease
nephronophthisis
title Cystic Kidney Diseases From the Adult Nephrologist’s Point of View
title_full Cystic Kidney Diseases From the Adult Nephrologist’s Point of View
title_fullStr Cystic Kidney Diseases From the Adult Nephrologist’s Point of View
title_full_unstemmed Cystic Kidney Diseases From the Adult Nephrologist’s Point of View
title_short Cystic Kidney Diseases From the Adult Nephrologist’s Point of View
title_sort cystic kidney diseases from the adult nephrologist s point of view
topic polycystic kidney diseases
autosomal dominant polycystic kidney disease
autosomal-recessive polycystic kidney disease
tuberous sclerosis complex
von Hippel–Lindau disease
nephronophthisis
url http://journal.frontiersin.org/article/10.3389/fped.2018.00065/full
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