Studies conducted at Charles University on cystic fibrosis recently published
March 2nd, 2009
2009 MAR 2 -- "Cystic fibrosis-related liver disease affects approximately one third of all patients with cystic fibrosis," scientists in Prague, Czech Republic report.
"Initial signs of other liver diseases including the genetically determined disorders of the liver co-inherited with cystic fibrosis may be obscured by or ascribed to cystic fibrosis-related liver disease. We report a patient shown to suffer simultaneously from cystic fibrosis and hepatic Wilson disease," wrote R. Kotalova and colleagues, Charles University.
The researchers concluded: "Our case documents that in patients with cystic fibrosis presenting with liver disease, when unusual clinical and/or laboratory abnormalities appear and fail to respond to standard therapy, a second disease, including rare inherited metabolic disorders such as the hepatic form of Wilson disease or alpha(1)-antitrypsin deficiency, should be suspected."
Kotalova and colleagues published their study in the Journal of Cystic Fibrosis (Wilson disease as a cause of liver injury in cystic fibrosis. Journal of Cystic Fibrosis, 2009;8(1):63-65).
For more information, contact R. Kotalova, Charles University Prague, School of Medicine 2, Dept. of Pediatrics, V Uvalu 84, Prague 15006 5, Czech Republic.
Publisher contact information for the Journal of Cystic Fibrosis is: Elsevier Science BV, PO Box 211, 1000 AE Amsterdam, Netherlands.
