RESPIRATORY
FIBROTIC LUNG DISEASE
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Question
[CLICK ON ANY CHOICE TO KNOW THE RIGHT ANSWER]
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nodular cystic fibrosis
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Classic Cystic Fibrosis
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CFTR-related disease
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Atypical Cystic Fibrosis
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Detailed explanation-1: -What is atypical CF? Atypical CF is a milder form of the CF disorder, which is a result of mutations of the CFTR gene. Individuals with atypical CF usually have 1 severe mutation and 1 less common mutation25 or abnormality of trinucleotide repeats on their other CFTR gene.
Detailed explanation-2: -Cystic fibrosis is an inherited disease characterized by the buildup of thick, sticky mucus that can damage many of the body’s organs. The disorder’s most common signs and symptoms include progressive damage to the respiratory system and chronic digestive system problems.
Detailed explanation-3: -Impaired CFTR functioning directly reduces ductal epithelial chloride secretion and indirectly increases sodium absorption through lack of CFTR’s inhibitory effect on the apical sodium channel. The result is dehydrated mucus and a widened, negative transepithelial potential difference.