URSODEOXYCHOLIC ACID EFFECTS ON CYSTIC FIBROSIS
LIVER DISEASE
IOANA MIHAIELA CIUCĂ1, LIVIU POP1, AURELIAN EMIL RANETTI2*, IULIANA M. POPESCU3, BOGDAN ALMAJAN-GUTA4, IOANA MARIA MALITA5, ION ANGHEL6
1Department of Paediatrics, Emergency Clinical County Hospital, “Victor Babes” University of Medicine and Pharmacy
Timisoara, Romania
2Department of Endocrinology, Emergency Central Military University Hospital, “Carol Davila” University of Medicine and
Pharmacy, Bucharest, Romania
3Department of Chemistry and Biochemistry, Faculty of Agriculture, Banat’s Agricultural Science University, Timisoara, Romania
4Department of Physical Education and Sports, West University, Timisoara, Romania
5Department of Pharmacology, “Victor Babes” University of Medicine and Pharmacy Timisoara, Romania
6ENT Department, “Coltea” Clinical Hospital, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania
*corresponding author: ranetti@gmail.com
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Abstract:
Cystic fibrosis (CF) is the most frequent monogenic autosomal recessive disease with lethal potential, manifested by an
important clinical polymorphism. Cystic fibrosis associated liver disease (CFLD) is the second non-pulmonary cause of death
in children with cystic fibrosis, being an important monitoring problem. The only existent treatment is ursodeoxicholic acid
(UDCA), which has controversial efficiency. The aim of this study was to evaluate the efficiency of the treatment with
UDCA on CFLD evolution. The study included 51 patients with CFLD, prospectively monitored for five years. They were
routinely followed-up by clinical assessment, liver biochemical tests and ultrasound examinations. All patients with CFLD
received UDCA in a dose of 15-20 mg/kg bw/day permanently. Liver tests results and ultrasound Williams score were used
for CFLD monitoring as primary endpoints. After UDCA administration, transaminases values decreased significantly in one
year of treatment. Important improvements in liver parenchyma were documented by the ultrasound score. UDCA showed a
valuable influence on CFLD outcome and should be recommended as soon as diagnosis is made.






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