Article
Idiopathic chronic pancreatitis treated with ivacaftor in a CFTR carrier with methylmalonic acidemia.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jul 2022
Tang Tin-Yun, Cruz Vincent B, Konczal Laura L
Abstract excerpt
CFTR mutation carriers, numbering 1 in 25 among Caucasians, have an increased risk of developing chronic pancreatitis due to the underlying dysfunction of ion channels created by the mutant allele. Carriers do not frequently manifest disease due to the remaining wild-type CFTR protein sufficiently maintaining normal pancreatic homeostasis. However, additional risk factors for pancreatitis, such as organic...
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