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Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis

Peter N Freswick1, Elizabeth K Reid2, Maria R Mascarenhas2

  • 1Helen DeVos Children's Hospital, Grand Rapids, MI 49503, USA.

Nutrients
|April 12, 2022
PubMed

Insights

Cystic fibrosis (CF) often causes pancreatic insufficiency and nutritional issues. Early pancreatic enzyme replacement therapy (PERT) is crucial for managing CF complications, despite its complexities.

Area of Science:

  • Gastroenterology
  • Pulmonology
  • Nutritional Science

Background:

  • Cystic fibrosis is primarily viewed as a pulmonary condition.
  • Pancreatic insufficiency is a common and serious complication in CF patients.
  • Nutritional deficits and comorbidities significantly impact CF patient health.

Purpose of the Study:

  • To highlight the importance of suspecting cystic fibrosis in patients with pancreatic insufficiency symptoms.
  • To emphasize the urgent need for initiating pancreatic enzyme replacement therapy (PERT).
  • To discuss the multifaceted considerations surrounding PERT for patients, dietitians, and clinicians.

Main Methods:

  • Literature review on cystic fibrosis, pancreatic insufficiency, and PERT.
  • Analysis of clinical guidelines and expert recommendations.
  • Synthesis of information regarding PERT administration, efficacy, and challenges.

Main Results:

  • High index of suspicion for CF is vital in patients presenting with pancreatic insufficiency.
  • Prompt initiation of PERT is critical for mitigating nutritional complications.
  • PERT involves diverse administration methods and potential for therapy failure and complications.

Conclusions:

  • Cystic fibrosis management requires a multidisciplinary approach addressing both pulmonary and gastrointestinal aspects.
  • Effective PERT is essential for improving nutritional status and overall outcomes in CF patients with pancreatic insufficiency.
  • Ongoing clinical attention to PERT nuances, including administration and potential complications, is necessary.

Keywords:
PERTcystic fibrosisnutritionpancreatic enzymespancreatic insufficiency

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