1Epatologia Pediatrica e Trapianto di Fegato, ISMETT, University of Pittsburgh Medical Center di Palermo
In the last few years, there has been considerable progress in the understanding of the role of primary cilia in various human diseases. They sense the extracellular environment and transduce signals into appropriate cellular response thus directing embryonic development and organ function. defects have been associated with a growing number of pediatric conditions including a number of complex syndromes and a few of organ-specific conditions. Hepatic ciliopathies are congenital Hepatic Fibrosis, caroli Syndrome and disease.