Describe in detail the pathophysiological process of cystic fibrosis.

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Cysticfibrosis can occur as an inheritable genetic disorder. It affects both the respiratory and digestive systems. This is due to a mutation of CFTR, which codes for a regulator protein that’s known as Cystic Fibrosis Transmembrane Conductance Regulation (CFTR). The defective gene causes thick and sticky mucus to build up in the lung, causing inflammation and persistent infections.

This abnormal mucus can accumulate in the lungs and block airways, which could cause difficulty breathing. Due to cystic Fibrosis’ decreased ciliary transportation, sputum that contains bacteria can remain in the pulmonary alveoli. This may lead to infection.

This condition causes increased secretion viscosity in the glands that line all affected areas, including the pancreas. It also leads to poor digestion and absorption. The result is malnutrition and dehydration, as well as the inability to absorb fluids properly.

Moreover , malfunctioning CFTR proteins lead to impairment of salt balance regulation throughout body tissues thus allowing ions such sodium chloride) to accumulate on the surface epithelial cells present on all organs involved. This may lead to a disruption in normal functioning and activate certain immune pathways which could potentially cause more severe symptoms.

Altogether , it’s evident that when a person inherits two copies mutations of CFTR gene severe consequences ensue eventually leading potentially fatal complications if left untreated . A successful treatment program must include aggressive management strategies and early diagnosis to prevent such complications.

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