Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)

The introduction of Cystic Fibrosis Trans Regulatory modulator (CFTRm) drugs has seen a transformation in Cystic Fibrosis (CF) treatment. This has led to a significant improvement in lung function and quality of life with the potential for a real impact on life expectancy.Transient mild to moderate...

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Autores principales:	Mahan Salehi, Mubashar Iqbal, Asha Dube, Amer AlJoudeh, Frank Edenborough
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Hepatic necrosis CFTR modulators Severe transaminitis Liver injury Adverse effect Diseases of the respiratory system RC705-779
Acceso en línea:	https://doaj.org/article/82aa5a8243be4feda1590e50d4520ca0
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spelling	oai:doaj.org-article:82aa5a8243be4feda1590e50d4520ca02021-11-14T04:32:39ZDelayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)2213-007110.1016/j.rmcr.2021.101553https://doaj.org/article/82aa5a8243be4feda1590e50d4520ca02021-01-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S221300712100215Xhttps://doaj.org/toc/2213-0071The introduction of Cystic Fibrosis Trans Regulatory modulator (CFTRm) drugs has seen a transformation in Cystic Fibrosis (CF) treatment. This has led to a significant improvement in lung function and quality of life with the potential for a real impact on life expectancy.Transient mild to moderate hepatic transaminitis is a well-known side effect of CFTRm drugs, which often improves on cessation and may not recur following the re-institution of the drug. We describe a case of transaminitis developing nine months after the initiation of Kaftrio, which progressed to liver necrosis despite stopping Kaftrio and took many months to resolve.The patient had experienced significant improvement in lung function and overall health while on Kaftrio and deteriorated when it was stopped. He was keen to restart; however, Kaftrio was not reinstated due to the potential risk of acute liver failure.Mahan SalehiMubashar IqbalAsha DubeAmer AlJoudehFrank EdenboroughElsevierarticleHepatic necrosisCFTR modulatorsSevere transaminitisLiver injuryAdverse effectDiseases of the respiratory systemRC705-779ENRespiratory Medicine Case Reports, Vol 34, Iss , Pp 101553- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Hepatic necrosis CFTR modulators Severe transaminitis Liver injury Adverse effect Diseases of the respiratory system RC705-779
spellingShingle	Hepatic necrosis CFTR modulators Severe transaminitis Liver injury Adverse effect Diseases of the respiratory system RC705-779 Mahan Salehi Mubashar Iqbal Asha Dube Amer AlJoudeh Frank Edenborough Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)
description	The introduction of Cystic Fibrosis Trans Regulatory modulator (CFTRm) drugs has seen a transformation in Cystic Fibrosis (CF) treatment. This has led to a significant improvement in lung function and quality of life with the potential for a real impact on life expectancy.Transient mild to moderate hepatic transaminitis is a well-known side effect of CFTRm drugs, which often improves on cessation and may not recur following the re-institution of the drug. We describe a case of transaminitis developing nine months after the initiation of Kaftrio, which progressed to liver necrosis despite stopping Kaftrio and took many months to resolve.The patient had experienced significant improvement in lung function and overall health while on Kaftrio and deteriorated when it was stopped. He was keen to restart; however, Kaftrio was not reinstated due to the potential risk of acute liver failure.
format	article
author	Mahan Salehi Mubashar Iqbal Asha Dube Amer AlJoudeh Frank Edenborough
author_facet	Mahan Salehi Mubashar Iqbal Asha Dube Amer AlJoudeh Frank Edenborough
author_sort	Mahan Salehi
title	Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)
title_short	Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)
title_full	Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)
title_fullStr	Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)
title_full_unstemmed	Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)
title_sort	delayed hepatic necrosis in a cystic fibrosis patient taking elexacaftor/tezacaftor/ivacaftor (kaftrio)
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/82aa5a8243be4feda1590e50d4520ca0
work_keys_str_mv	AT mahansalehi delayedhepaticnecrosisinacysticfibrosispatienttakingelexacaftortezacaftorivacaftorkaftrio AT mubashariqbal delayedhepaticnecrosisinacysticfibrosispatienttakingelexacaftortezacaftorivacaftorkaftrio AT ashadube delayedhepaticnecrosisinacysticfibrosispatienttakingelexacaftortezacaftorivacaftorkaftrio AT ameraljoudeh delayedhepaticnecrosisinacysticfibrosispatienttakingelexacaftortezacaftorivacaftorkaftrio AT frankedenborough delayedhepaticnecrosisinacysticfibrosispatienttakingelexacaftortezacaftorivacaftorkaftrio
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Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)

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