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2/2017
vol. 92 abstract:
Editorial
Liuer and kidney disorders in ciliopathies
Patryk Lipiński
,
Irena Jankowska
,
Ryszard Grenda
PEDIATRIA POLSKA 92 (2017) 121-128
Online publish date: 2018/03/07
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Ciliopathies constitute a group of disorders characterized by cilia abnormalities and an extremely heterogeneous clinical presentation. The liver and kidneys are the most commonly affected organs and the term hepatorenal ńbrocystic disorders is used to describe ciliopathies with combined liver and kidney involvement. Liver disorders in ciliopathies can be grouped into three categories: congenital hepatic ńbrosis, Caroli's disease and polycystic liver disease. Kidney disorders related to primary cilia abnormalities include autosomal dominant and recessive polycystic kidney diseases and nephronophthisis.
keywords:
Ciliopathies, Primary cilia, Congenital hepatic ńbrosis, Caroli"s disease, Polycystic liver disease, Autosomal recessive polycystic kidney disease |
POLSKI
