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    Ati paediatrics final proctored exam 2025

    A nurse is reinforcing teaching to a newly hired nurse about cystic fibrosis. Which of the following statements should the nurse make?

    Explanation & Rationale

    A. "The life expectancy for an individual who has this disorder is 25 years of age." Advances in treatment, including improved respiratory therapies and medications like CFTR modulators, have significantly increased life expectancy. Many individuals with cystic fibrosis (CF) now live into their 40s or beyond, depending on disease severity and access to medical care. B. "This disorder is most common in individuals of Northern European descent." CF is most prevalent in individuals of Northern European ancestry, affecting about 1 in 2,500 to 3,500 newborns in this population. It is much less common in individuals of African, Asian, or Hispanic descent. C. "Heart failure will occur at some point during the progression of CF." CF primarily affects the lungs and digestive system due to thick mucus production, but heart failure is not a direct consequence of the disease. However, cor pulmonale (right-sided heart failure) can develop secondary to chronic lung disease and pulmonary hypertension in severe cases. D. "This disorder occurs when the mother has a mutation of the 7th chromosome." CF is an autosomal recessive disorder, meaning a child must inherit mutated CFTR genes from both parents (not just the mother). The mutation affects the CFTR gene located on chromosome 7, leading to defective chloride transport and thick mucus production.

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