SNFGE SNFGE
 
Thématique :
- Foie
Originalité :
Réexamen
Solidité :
Très solide
Doit faire évoluer notre pratique :
Immédiatement
 
 
Nom du veilleur :
Professeur Christine SILVAIN
Coup de coeur :
 
 
Hepatology
  2019/04  
 
  2019 Apr;69(4):1648-1656.  
  doi: 10.1002/hep.30148.  
 
  Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients.  
 
  Boëlle PY, Debray D, Guillot L, Clement A, Corvol H; French CF Modifier Gene Study Investigators.  
  https://www.ncbi.nlm.nih.gov/pubmed/30058245  
 
 

Abstract

Cystic fibrosis (CF)-related liver disease (CFLD) is a common symptom in patients with CF. However, its prevalence, risk factors, and evolution are unclear. We analyzed a large database of patients with CF to investigate the incidence of CFLD, its related risk factors, and the use and effect of ursodeoxycholic acid (UDCA) treatment. We retrospectively analyzed 3,328 CF patients with pancreatic insufficiency born after 1985 and recruited into the French CF Modifier Gene Study since 2004. We determined liver status, age at CFLD and severe CFLD onset, sex, CFTR genotype, history of meconium ileus, treatment with UDCA, and respiratory and nutritional status. The incidence of CFLD increased by approximately 1% every year, reaching 32.2% by age 25. The incidence of severe CFLD increased only after the age of 5, reaching 10% by age 30. Risk factors for CFLD and severe CFLD were male sex, CFTR F508del homozygosity, and history of meconium ileus. Increasingly precocious initiation of UDCA treatment did not change the incidence of severe CFLD. Finally, patients with severe CFLD had worse lung function and nutritional status than other CF patients. Conclusion: CFLD occurs not only during childhood but also later in the lifetime of patients with CF; male sex, CFTR F508del homozygosity, and history of meconium ileus are independent risk factors for CFLD development; earlier use of UDCA over the last 20 years has not changed the incidence of severe CFLD, leading to questions about the use of this treatment in young children given its possible adverse effects.

 

 
Question posée
 
Evaluation de l’atteinte hépatique dans la mucoviscidose (CFLD) en termes de prévalence, facteurs de risque, évolution et effet de l’UDCA à partir de la cohorte française mise en place en 2004.
 
Question posée
 
L’incidence de CFLD sévère augmente seulement après l’âge de 5 ans, atteignant 10% à 30 ans. Les facteurs de risque de CFLD et de CFLD sévères sont : genre masculin, homozygotie CFTR F508del, et antécédent d’iléus méconial. Le traitement précoce par UDCA ne modifie pas l’incidence de CFLD sévère. Les patients ayant une CFLD sévère ont une altération plus marquée de la fonction respiratoire et du status nutritionnel.
 
Commentaires

Importance des informations concernant les atteintes hépatiques dans la mucoviscidose à partir d’une des plus grandes cohortes. L’efficacité limitée de l’UDCA ne doit pas faire changer notre prise en charge amis doit inciter à trouver d’autres pistes de traitement.

 
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