Acute pancreatitis in pancreatic-insufficient cystic fibrosis patients treated with CFTR modulators.
J Cyst Fibros
; 22(4): 777-779, 2023 07.
Article
em En
| MEDLINE
| ID: mdl-36914434
ABSTRACT
Cystic fibrosis transmembrane conductance regulator modulator therapy is associated with substantial clinical benefit and improved quality of life in patients with cystic fibrosis (CF). While their effect on lung function has been clearly reported, we are still in the process of unraveling the full impact they have on the pancreas. We present two cases of pancreatic-insufficient CF patients who presented with acute pancreatitis shortly after commencing elexacaftor/tezacaftor/ivacaftor modulator therapy. Both patients were treated with ivacaftor for 5 years prior to elexacaftor/tezacaftor/ivacaftor initiation, but had no previous episodes of acute pancreatitis. We suggest that highly effective modulator combination therapy may restore additional pancreatic acinar activity, resulting in the development of acute pancreatitis in the interim until ductal flow is improved. This report adds to the growing evidence for possible restoration of pancreatic function in patients receiving modulator therapy, and highlights that treatment with elexacaftor/tezacaftor/ivacaftor may be associated with acute pancreatitis until ductal flow is restored, even in pancreatic-insufficient CF patients.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Pancreatite
/
Fibrose Cística
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
Idioma:
En
Revista:
J Cyst Fibros
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Israel