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Medicine General ac7e1ec8

Which of the following systems is least likely to be affected in cystic fibrosis

A
Respiratory
B
Genitourinary
C
Hepatobiliary
D
Endocrine
High-Yield Explanation
Answer is D (Endocrine): Cystic fibrosis is typically charachterized by Exocrine Pancreatic insufficiency. Endocrine Pancreatic insufficiency is not seen as a tyical manifestation of cystic fibrosis. Charachteristic Classical Manifestations of Cystic Fibrosis (Typical System Involvement) Respiratory system (Sinopulmonary disease) Pulmonary infection Persistant colonization/infection with typical cystic fibrosis pathogens, including Staphylococcus aureus, nontypeable, Hemophilus influenzae, mucoid and nonmucoid Pseudomonas aeruginosa, Burkholderia cepase Chronic obstructive Pulmonary Disease Bronchiectasis is typical Airway obstruction manifested by wheezing and air trapping Chronic cough & sputum production Digital clubbing Persistent chest radiograph abnormalities (eg. Bronchiectasis, atelectasis, infiltrates, hyperinflation) Nasal Polyps radiographic or computed tomographic abnormalities of paranasal sinuses Gastrointestinal System (Intestinal; Hepatobiliary; Pancreatic) Intestinal meconium ileus, distal intestinal obstruction syndrome, rectal prolapse. Pancreatic Pancreatic exocrine deficiency Hepatobiliary Hepatic disease manifested by clinical or histologic evidence of focal biliary cirrhosis, or multilobular cirrhosis Nutritional Failure to thrive (protein-calorie malnutrition), hypoproteinemia, edema, complications secondary to fat-soluble vitamin deficiency Genitourinary System (Male Infeility) Male urogenital abnormalites resulting in obstructive azoospermia are typical Metabolic Abnormalities are typically charachterized by salt depletion syndrome, (Hyponatremia, Hypocholeremia and Hypokalemic Metabolic Alkalosis)

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