Cystic fibrosis is associated with EPI

Most patients with cystic fibrosis (CF) have EPI1-3

CF is an autosomal recessive disease due to a mutated CFTR gene on the long arm of chromosome 7. The CF mutation results in abnormal sodium and chloride transport. CF is associated with viscous secretions that obstruct the pancreatic duct, leading to pancreatic ductal obstruction and pancreatitis, pancreatic fibrosis, and EPI.

90% of children with CF will have EPI by age one
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