Article ; Online: Emerging clinical perspectives in cystic fibrosis liver disease.
Current opinion in pulmonary medicine
2021 Volume 27, Issue 6, Page(s) 593–599
Abstract: Purpose of review: Liver disease (CFLD) as a complication of cystic fibrosis is recognized as a more severe disease phenotype in both children and adults. We review recent advances in understanding the disease mechanism and consider the implications of ... ...
Abstract | Purpose of review: Liver disease (CFLD) as a complication of cystic fibrosis is recognized as a more severe disease phenotype in both children and adults. We review recent advances in understanding the disease mechanism and consider the implications of new strategies for the diagnosis and management of cystic fibrosis in those with evidence of clinically significant liver disease. Recent findings: Evidence suggests that the prevalence of CFLD has not declined with the introduction of newborn screening. Furthermore, children with CFLD, who have been diagnosed with cystic fibrosis following newborn screening continue to have a much higher mortality rate compared with those with no liver disease. There is further data suggesting noncirrhotic obliterative portal venopathy as the predominant pathological mechanism in the majority of children and young adults receiving a liver transplantation. Little progress has been made in developing an accurate noninvasive test for early diagnosis or monitoring disease progression in CFLD. The benefit of new modulator therapies is not well understood in those with established CFLD, whereas the risk of hepatotoxicity as a complication of treatment must be carefully monitored. Summary: Better understanding of the pathophysiology of CFLD would allow a standardized approach to diagnosis, with the potential to improve outcomes for those with CFLD. |
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MeSH term(s) | Cystic Fibrosis/complications ; Cystic Fibrosis/therapy ; Disease Progression ; Humans ; Liver Cirrhosis ; Liver Diseases/etiology ; Liver Transplantation ; Phenotype |
Language | English |
Publishing date | 2021-08-30 |
Publishing country | United States |
Document type | Journal Article ; Review |
ZDB-ID | 1285505-4 |
ISSN | 1531-6971 ; 1070-5287 ; 1078-1641 |
ISSN (online) | 1531-6971 |
ISSN | 1070-5287 ; 1078-1641 |
DOI | 10.1097/MCP.0000000000000824 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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