The organ of digestive system most commonly involved in cystic fibrosis is

UPPSC 2017

Medical & Surgical Nursing

Easy

Appeared in: UPPSC 2017

Explanation

Cystic Fibrosis (CF) is a genetic disorder of the exocrine glands, causing the production of abnormally thick, sticky mucus.
The pancreas is the most commonly and severely affected digestive organ; in fact, the full name of the disease was once 'cystic fibrosis of the pancreas'.
Thick mucus obstructs the pancreatic ducts, preventing digestive enzymes from reaching the duodenum. This occurs in 85-90% of patients.
This blockage leads to exocrine pancreatic insufficiency, resulting in malabsorption of fats and proteins, steatorrhea, and failure to thrive.

Option B: The small intestine is affected, but often secondarily to the lack of pancreatic enzymes. Direct complications like meconium ileus (in 10-15% of newborns) are less common than pancreatic insufficiency.
Option C: The esophagus is not directly affected by the primary disease process of cystic fibrosis. Any pathology, such as varices, is a late-stage, secondary complication of liver disease.
Option D: The stomach's secretory and motor functions are not primarily affected by the CFTR mutation. It is not a major site of pathology in CF.

Clinical Relevance

Nursing practice connection: Knowing Gastrointestinal manifestations of Cystic Fibrosis helps nurses interpret findings accurately and avoid errors in routine assessment, medication administration, and patient teaching.
Nurses must meticulously assess CF patients for signs of malabsorption, including monitoring stool characteristics (for steatorrhea), tracking weight, and evaluating nutritional status.
A key nursing intervention is educating patients and families on Pancreatic Enzyme Replacement Therapy (PERT). Enzymes must be taken with all meals and snacks to facilitate digestion and nutrient absorption.
What if? If a patient with CF develops CF-related diabetes (CFRD) due to progressive pancreatic damage, the nursing care plan must expand to include blood glucose monitoring, insulin administration, and diabetes education, alongside managing the exocrine insufficiency.

How to Approach the Question

First, identify the key terms in the question: 'cystic fibrosis', 'digestive system', and 'most commonly involved'.
Recall the basic pathophysiology of cystic fibrosis: it is a disease of exocrine glands causing thick, viscous secretions.
Consider how thick secretions would impact each of the listed organs. The pancreas relies on a network of fine ducts to transport enzymes, making it extremely vulnerable to obstruction.
Evaluate the options based on the frequency and severity of involvement. Pancreatic insufficiency is a hallmark and near-universal feature of CF, making it the 'most commonly' affected organ compared to the other options.
The very name 'cystic fibrosis of the pancreas' can serve as a strong clue to the correct answer.

Concept Tested & Keywords

QjUW7Zi0N6bmYTKc7iq3vC

E6 Medicine Harrison 22e Part 2 p. 149-151

E6 Pathology-Textbook of PATHOLOGYHarsh Mohan Part 3 (515-969) p. 137-139

E6 Pathology- ROBBINS & COTRAN PATHOLOGIC BASIS OF DISEASE 10TH Ed p. 476-478