title: Liver involvement in children with ciliopathies.
abstract: Abnormalities in primary cilia lead to diseases called ciliopathies. Multiple organ involvement is the norm since primary cilia are present in most cells. When cholangiocyte cilia are abnormal, ductal plate malformation ensues leading to such conditions as congenital hepatic fibrosis, Caroli disease or syndrome, or other fibrocystic disease.
authors: Swiss Center for Liver Disease in Children, Department of Pediatrics, University Hospitals of Geneva, 5, rue Willy-Donze, 1205 Geneva, Switzerland. Electronic address: Nathalie.rock@hcuge.ch.
authors: N
authors: N
authors: Rock
authors: Swiss Center for Liver Disease in Children, Department of Pediatrics, University Hospitals of Geneva, 5, rue Willy-Donze, 1205 Geneva, Switzerland.
authors: V
authors: V
authors: McLin
mesh_terms: D002648:Child; D002925:Ciliary Motility Disorders / Q000150:complications*; D030342:Genetic Diseases, Inborn; D006801:Humans; D008103:Liver Cirrhosis; D008107:Liver Diseases / Q000175:diagnosis / Q000209:etiology*