Continuing Medical Education Questions: December 2021

Abstract

Article Title: Cystic Fibrosis Transmembrane Conductance Regulator Modulator Use is Associated with Reduced Pancreatitis Hospitalizations in Subjects with Cystic Fibrosis

LEARNING OBJECTIVE

After this activity, the participant will be able to identify the role of cystic fibrosis transmembrane conductance regulator (CFTR), recognize the impact of CFTR malfunction in the pancreas, and explain the effect of CFTR modulators in the pancreas.

QUESTION 1

Which of the following statements best describe the cystic fibrosis transmembrane conductance regulator (CFTR) ion channel?

• A. CFTR ion channel is primarily expressed on the apical plasma membrane, and it regulates Na + and H₂O secretion
• B. CFTR ion channel is primarily expressed on the apical plasma membrane, and it regulates Cl- and HCO3- secretion
• C. CFTR ion channel is primarily expressed on the basolateral plasma membrane, and it regulates Na + and H₂O secretion
• D. CFTR ion channel is primarily expressed on the basolateral plasma membrane, and it regulates Cl- and HCO3- secretion

QUESTION 2

Based on this article, which of the following best describes acute pancreatitis in cystic fibrosis?

• A. Acute pancreatitis does not occur in pancreas sufficient (PS) patients
• B. Acute pancreatitis occurs more often in pancreas insufficient (PI) patients
• C. Acute pancreatitis occurs in both PS and PI patients
• D. Acute pancreatitis occurs in PI patients with undetectable fecal elastase

QUESTION 3

An 8-year-old male with history of asthma, recurrent sinusitis and respiratory tract infections was hospitalized a fourth time this year with recurrent acute pancreatitis. He has had 10 attacks of acute pancreatitis over the past 2 years. Physical examination revealed that his height and weight was fifth percentile for his age but was otherwise unremarkable. His fecal elastase is 100. The patient underwent genetic testing and was found to have F508del and R117H, consistent with a diagnosis of cystic fibrosis. The pediatrician decides to start him on a CFTR modulator. Based on this article, how do CFTR modulators impact risk of acute pancreatitis (AP) hospitalization?

• A. CFTR modulators reduce risk of AP hospitalization in PS patients alone
• B. CFTR modulators reduce risk of AP hospitalization more in PI patients
• C. CFTR modulators reduce risk of AP in both PS and PI patients
• D. CFTR modulators do not reduce risk of AP hospitalization