Related Experiment Video
Updated: Jan 16, 2026
Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients
Published on: February 11, 2017
Beyond the Surface: Novel Therapy Approach for Pancreatitis in the Setting of CFTR Dysfunction
Brittany A Wright1,2, Ellie Hodapp1, Douglas B Hornick3
1Department of Pharmaceutical Care, University of Iowa Health Care, Iowa City, Iowa, USA.
Objectives:
Despite cystic fibrosis transmembrane conductance regulator (CFTR) proteins being present throughout the entire body and organ systems, typical presentation of cystic fibrosis (CF) involves lung disease. We report a series of individuals that were referred to our CF clinic for evaluation following a diagnosis of chronic pancreatitis or acute recurrent pancreatitis. After evaluation, they were deemed to have CFTR dysfunction and started on highly effective modulator therapy (HEMT).
Methods:
A retrospective chart review was completed for all individuals referred to the University of Iowa Health Care Cystic Fibrosis Clinic from January 1, 2012, through December 31, 2023, for follow-up evaluation of chronic or acute recurrent pancreatitis. Along with demographic data, we quantified emergency department encounters or hospitalizations due to pancreatitis pre- and post-HEMT as well as individual tolerability of therapy.
Results:
Ten individuals met inclusion criteria. Four individuals were found to have sweat chlorides that confirmed a diagnosis of CF, and three individuals with sweat chloride values below the diagnostic threshold were further evaluated with nasal potential difference (NPD) testing and found to have results consistent with CFTR dysfunction. All 10 patients were initiated on HEMT. In the 2 years prior to HEMT initiation, there was an average of 0.33 pancreatic episodes per month (range: 0.00-1.38), compared with 0.09 pancreatic episodes per month (range: 0.00-0.50) after initiation. HEMT was generally well tolerated with one individual discontinuing due to liver function abnormalities.
Conclusions:
Our study highlights a panel of individuals that may have been overlooked for CFTR dysfunction due to a nonpulmonary primary presentation, such as pancreatitis. However, our use of additional diagnostic tools followed by HEMT utilization was able to describe individual-reported improvements in symptoms and quality of life in individuals with CFTR dysfunction.
More Related Videos
05:56Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
Published on: August 29, 2025
08:00Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
Related Concept Videos
01:29Chronic Pancreatitis II: Collaborative Care
Assessment:
01:24Cystic Fibrosis: Management
Sinus disease and chronic...
01:24Chronic Pancreatitis I: Introduction
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
01:30Acute Pancreatitis II: Clinical Manifestations and Management
01:23Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
01:27Acute Pancreatitis I: Introduction
Acute pancreatitis is characterized by rapid inflammation of the pancreas, often caused by factors like gallstone blockage or excessive alcohol consumption. Chronic pancreatitis, on the other hand, is a slow, progressive inflammation that may result from long-term alcohol abuse, obstructions in the pancreatic duct, or genetic factors.
The causes of acute pancreatitis include: