Cystic Fibrosis: Multidisciplinar Nakano

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Cystic Fibrosis Shaun Hale Eastern Michigan University Abstract BACKGROUND: Cystic Fibrosis is an autosomal recessive disorder that occurs in 1 in 3000 births. Mutations in the cystic fibrosis transmembrane regulator gene (CFTR), which regulates hydration of epithelial cells located throughout the body by controlling chloride and sodium transport. Defects in the chloride channel alter the transport of electrolytes across the cell membrane, creating excessive secretion of sodium and chloride. As a result, thick secretion in exocrine glands affects the lungs, pancreas, and reproductive organs. Treatment goals include minimization of pulmonary complications, adequate nutrition, and patient/family education. SEARCH METHODS: Medical databases including CINAHL and PUBMED were utilized. DATA COLLECTION & ANALYSIS: Cystic Fibrosis, treatment, etiology, plan of care, screening, testing, and nursing publication were used. MAIN RESULTS; Treatment of CF requires…show more content…
He explains that between 1,940 and 1,965 mutations are associated with CF, and 127 of them have been positively linked to impaired CFTR function significant enough to produce CF symptoms. The most commonly cited pathologic mutation is designated AF508, and it occurs in 70% of whites with CF (Nakano & Tluczek, 2014). Nicholson (2013) explains that CF is an autosomal recessive disease that requires inheritance of two mutant chromosomes in order for an individual to express physical symptoms. Those inheriting only one mutant chromosome are designated carriers. In their article entitled “Update in Cystic Fibrosis 2013,” authors Jain and Goss (2013) stress clinical manifestations that include bronchiectasis, chronic sinusitis, idiopathic pancreatitis, chronic obstructive pulmonary disease, meconium ileus, and congenital bilateral absence of the vas

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