Pancreatic involvement in cystic fibrosis
Yesim Ozen Alahdab1, Deniz G Duman
1Department of Gastroenterology, Marmara University School of Medicine, Istanbul, Turkey - yesimalahdab@yahoo.com.
Insights
Cystic fibrosis (CF) affects the pancreas, causing exocrine and endocrine dysfunction. This review details CF pancreatic disease types, clinical presentation, diagnosis, and genetics.
Area of Science:
- Gastroenterology
- Genetics
- Pediatrics
Background:
- Cystic fibrosis (CF) is a genetic disorder impacting multiple organs, notably the pancreas.
- Improved patient care allows for observation across all age groups.
- Pancreatic involvement is a significant aspect of CF pathology.
Purpose of the Study:
- To review the pancreatic manifestations of cystic fibrosis.
- To outline the disease types, clinical features, and diagnostic approaches.
- To discuss the genetic underpinnings of CF-related pancreatic disease.
Main Methods:
- Literature review of cystic fibrosis and pancreatic involvement.
- Synthesis of information on clinical presentation and diagnosis.
- Analysis of genetic factors contributing to pancreatic complications.
Main Results:
- CF frequently impacts the exocrine pancreas, leading to dysfunction.
- Endocrine pancreatic function can also be compromised.
- Pancreatic parenchymal damage, including pancreatitis, may occur.
Conclusions:
- Pancreatic involvement is a critical component of cystic fibrosis management.
- Understanding the spectrum of pancreatic disease in CF is essential for patient care.
- Genetic factors play a key role in the development of pancreatic complications in CF.
Abstract:
Cystic fibrosis (CF) is an important genetic disorder that usually manifests in early childhood. Better understanding and improved care of CF patients in our current practice with pancreatic enzyme replacement therapy and nutritional support has enabled physicians to see them more frequently from any age groups. After the lungs, pancreas is the most important organ involved with CF. Its most frequently affected component is the exocrine pancreas while endocrine functions may also be deranged. Parenchymal damage as acute or chronic pancreatitis may also develop. Herein, we aimed to outline the types of the disease, clinical picture, diagnosis and genetic aspect of the CF linked with the pancreatic involvement.
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