Which organ systems are affected in cystic fibrosis?

Prepare for the PAEA Pediatrics EOR Exam. Study with flashcards and multiple-choice questions, each question has hints and explanations. Ace your exam!

Cystic fibrosis primarily impacts several organ systems due to a malfunction in the CFTR gene, which is responsible for the transport of chloride ions across epithelial cell membranes. This defect leads to thick, sticky mucus accumulation, particularly in the lungs, pancreas, liver, intestines, and reproductive tracts.

In the lungs, the thick mucus obstructs airflow and creates an environment conducive to bacterial infections, leading to chronic respiratory issues. The pancreas is affected because the thick secretions block the release of digestive enzymes, causing malabsorption of nutrients. Similarly, the liver can develop complications as the thick bile can lead to biliary duct obstruction. In the intestines, the thick mucus affects the gastrointestinal tract's normal function, leading to difficulties with digestion and nutrient absorption. The reproductive tract is also impacted, especially in males, where congenital absence of the vas deferens can lead to infertility.

Cystic fibrosis does not primarily affect the heart, kidneys, skin and joints, or eyes and ears, which is why the other options do not represent the systemic impact of this hereditary condition. Understanding the organ systems affected helps in managing and anticipating complications associated with cystic fibrosis effectively.

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