This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.Currently there is no specific treatment for children with hepatic disorders of the liver that lead to significant fibrosis or cirrhosis other than eventual liver transplantation. In recent years colchicine has shown promise as an antifibrogenic agent in adults with cirrhosis. The rationale for the use of colchicine in cirrhosis is its demonstrated inhibition of microtubule assembly within cells by binding to the protein tubulin, interference with collagen secretion (that may be microtubule-dependent), and stimulation of the collagenase activity. Colchicine may block the progression and amplification of the inflammatory response as has been observed in disorders such as gout and familial Mediterranean fever. Colchicine will be administered to pediatric patients with hepatic fibrosis or cirrhosis for whom active inflammation is not the predominant histologic feature of the liver disorder. The subjects will be followed at 6-12 month intervals during the 15 year duration of colchicine therapy in order to determine if there is a clinical benefit in the severity of liver injury or non-invasive measures of hepatic fibrosis and portal hypertension.
Showing the most recent 10 out of 837 publications