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Cystic fibrosis (also known as CF or mucoviscidosis) is an autosomal recessive genetic disorder affecting most critically the lungs, and also the pancreas, liver, and intestine.
DIOS was previously known as meconium ileus equivalent, a name which highlights its similarity to the intestinal obstruction seen in newborn infants with cystic fibrosis. [2] DIOS tends to occur in older individuals with pancreatic insufficiency .
Fibrosing colonopathy is a disease that arises in people with cystic fibrosis treated with high doses of pancreatic enzyme supplements. [1] [2] Symptoms are non-specific with abdominal pain, abdominal swelling, vomiting, and constipation. [1]
Cystic Fibrosis Lifestyle Foundation (CFLF) [26] seeks to create therapies that engage adolescents and young adults with cystic fibrosis as active agents in their healthcare, physically, psychologically, and spiritually, thereby enabling attitudes and lifestyles that create stronger and longer lives for themselves. Founded by Brian Callanan
[12] [3] [13] Cystic fibrosis eventually results in severe bronchiectasis in nearly all cases. [14] The cause in 10–50% of those without cystic fibrosis is unknown. [3] The mechanism of disease is breakdown of the airways due to an excessive inflammatory response. [3] Involved airways become enlarged and thus less able to clear secretions. [3]
Clubbing is associated with lung cancer, lung infections, interstitial lung disease, cystic fibrosis, or cardiovascular disease. [5] Clubbing may also run in families, [5] and occur unassociated with other medical problems. [6] [7] Clubbing has been recognized as a sign of disease since the time of Hippocrates. [5]
Cystic fibrosis: More than 1,800 mutations in the CFTR gene have been found [71] but the majority of these have not been associated with cystic fibrosis. [72] Most of these mutations either substitute one amino acid (a building block of proteins) for another amino acid in the CFTR protein or delete a small amount of DNA in the CFTR gene. The ...
Adult-onset Still's disease [4] ... Cystic fibrosis [4] Epidermolysis ... Schnitzler syndrome [4] Symptoms. Signs and symptoms of amyloidosis can vary depending on ...
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