Kinder- und Jugendmedizin 2011; 11(01): 25-31
DOI: 10.1055/s-0038-1630468
Aus der Praxis für die Praxis
Schattauer GmbH

Zystische Nierenerkrankungen im Kindesalter

Renal cystic diseases in childhood
B. Lange-Sperandio
1   Dr. v. Haunersches Kinderspital, Ludwig-Maximilians-Universität München;
I. Krüger-Stollfuß
1   Dr. v. Haunersches Kinderspital, Ludwig-Maximilians-Universität München;
J. Hoefele
1   Dr. v. Haunersches Kinderspital, Ludwig-Maximilians-Universität München;
2   Zentrum für Humangenetik und Laboratoriumsmedizin Dr. Klein und Dr. Rost, Martinsried
L. T. Weber
1   Dr. v. Haunersches Kinderspital, Ludwig-Maximilians-Universität München;
› Author Affiliations
Further Information

Publication History

received: 15 July 2010

Accepted after major revision 20 July 2010

Publication Date:
25 January 2018 (online)


Zysten in der kindlichen Niere sind bei einer Vielzahl von Erkrankungen nachweisbar. Sie treten entweder angeboren oder erworben auf, können Teil einer Multiorganerkrankung oder nur auf die Niere beschränkt sein. Ursächlich sind u. a. Mutationen in mehreren Genen, die zu einer Veränderung der Proteinstruktur primärer Zilien und damit zur Entstehung zystischer Nierenerkrankungen führen. Der Familienanamnese und molekulargenetischen Untersuchung kommt neben der Bildgebung eine entscheidende Rolle zu. Sonografisch kann die Diagnose teilweise bereits pränatal gestellt werden. Dabei ist es wichtig, auf eine Beteiligung extrarenaler Organe zu achten. Im Folgenden wird ein Überblick über die Klassifikation, Diagnostik und Therapie zystischer Nierenerkrankungen im Kindesalter gegeben.


Renal cysts in children can be found in a variety of diseases, which can be congenital or acquired. Renal cysts may be part of a multiorgan disorder or restricted to the kidneys only. Mutations in several genes, which are linked to dysfunctional primary cilia, have been identified to cause renal cystic diseases. Imaging as well as family history and genetic testing play an important role. Renal ultrasound helps to detect and characterize many of the cystic diseases even before birth. The search for extrarenal organ involvement is very important. The aim of this article is to give an overview on classification, diagnostic tools and therapy of renal cystic diseases in childhood.

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