Prof MandanaRafeey

یکشنبه ۱۶ اسفند ۱۳۹۴ 396 3 Minutes
Prof MandanaRafeey

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     Prof MandanaRafeey                                         
Tabriz University of Medical Sciences
                                    

      

European Society for Paediatric Gastroenterology, Hepatology and Nutrition

Monothematic Conference on Cystic Fibrosis related Liver disease

January 28th-29th, 2016,Imagine Institute, Paris, France

 

Cystic fibrosis (CF) is the most frequent autosomal recessive disorder in Caucasians. With the improvement in the management of lung disease and nutrition and the increased life expectancy of CF patients, CF-related liver disease (CFLD) has become clinically more important and one of the leading causes of death among patients with CF. The detailed pathophysiology and prognostic factors for the long-term evolution of CFLD have remained unclear.Three developments are expected to have major influences on the prevention and treatment of CFLD and on the understanding of its pathophysiology.    the increased life expectancy of CF patients has led to CFLD patients advancing well into adult life. The clinical course of CFLD in adult CF patients, however, has not been characterized, nor the prognostic factors for long-term complications;    the emergence of novel treatments, either CF specific (correctors or potentiators) or aspecific (bile acid analogues, antifibrotic agents), necessitate the identification of defined criteria of (stages of) CFLD and of biomarkers for the severity of CFLD disease;    the availability of novel experimental models allow for more detailed pathophysiologic and mechanistic studies to identify the underlying mechanisms of CFLD and possible targets of interventions. Recently the possible roles of the intestinal microbiome, of nuclear hormone activation and of extra-epithelial expression of CFTR in the development of CFLD have been hypothesized.


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Objective of this meeting

The goal of the meeting is to provide an overview of these three emerging fields with respect to CFLD:

the clinical course of CFLD into adulthood;    the definition of relevant parameters for CFLD development and severity to assess the effects of novel treatments;

the understanding of CFLD development based on studies in novel experimental models

 

Three developments are expected to have major influences on the prevention and treatment of CFLD and on the understanding of its pathophysiology :

.Biomarkers and non invasive tools to measure liver fibrosis

Treatments including CFTR correctors or potentiators, and bile acid analogues or antifibrotic agents ;

Experimental models allowing for pathophysiologic and mechanistic studies and identify possible targets of interventions. Recently the possible roles of the intestinal microbiome, of nuclear hormone activation and of extra-epithelial expression of CFTR in the development of CFLD have been hypothesized .

This monothematic conference on CFLD is a unique chance to provide an overview of these emerging fields :

the clinical course of CFLD into adulthood ;

the definition of relevant parameters for CFLD development and severity to assess the effects of novel treatments ;

the understanding of CFLD development based on studies in novel experimental models.

 

 

 

 

 

 




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