Guidelines

What causes pancreatitis in cystic fibrosis?

What causes pancreatitis in cystic fibrosis?

Pancreatic damage in CF is characterised by widespread loss of acinar cells with fatty replacement and interstitial fibrosis. Pancreatic disease in CF varies from complete loss of exocrine and endocrine function to nearly normal pancreatic function. About 90% of the CF patients show exocrine pancreatic insufficiency.

What is a CF variant?

CF is a disease that causes thick, sticky mucus to build up. This mucus can lead to problems with breathing and lung infections. This mucus can also make it harder for the body to break down food.

How many variants of CF are there?

Scientists have found more than 1,700 different mutations in the CFTR gene that can cause CF.

Does CF cause pancreatitis?

Pancreatitis is a rare complication among patients with CF. It occurred for 1.24% (95% CI: 1.02–1.46%) of a large CF cohort. Pancreatitis mainly occurs during adolescence and young adulthood. It is much more common among patients with CF and PS (10.3%), but it can occur among patients with PI (0.5%).

What are the side effects of cystic fibrosis?

It leads to severe respiratory and digestive problems as well as other complications such as infections and diabetes. Cystic fibrosis is caused by a defective protein that results from mutations in the CFTR gene. While there are approximately 2,000 known mutations of the CFTR gene, the most common mutation is the F508del mutation.

Do you have to have one gene mutation to have cystic fibrosis?

The type of gene mutation is associated with the severity of the condition. Children need to inherit one copy of the gene from each parent in order to have the disease. If children inherit only one copy, they won’t develop cystic fibrosis. However, they will be carriers and possibly pass the gene to their own children.

Who is the FDA commissioner for cystic fibrosis?

Today’s landmark approval is a testament to these efforts, making a novel treatment available to most cystic fibrosis patients, including adolescents, who previously had no options and giving others in the cystic fibrosis community access to an additional effective therapy,” said acting FDA Commissioner Ned Sharpless, M.D.

What happens if you have only one copy of cystic fibrosis?

If children inherit only one copy, they won’t develop cystic fibrosis. However, they will be carriers and could pass the gene to their own children. Because cystic fibrosis is an inherited disorder, it runs in families, so family history is a risk factor.