Which are common manifestations of cystic fibrosis?
Salty skin.
Barrel chest.
Clubbing.
Bradycardia.
Steatorrhea.
Correct Answer : A,B,C,E
Choice A rationale
Salty skin is caused by the dysfunction of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein, which impairs chloride and water transport across epithelial cells, including those in sweat ducts. The unabsorbed chloride remains in the sweat, leading to an elevated sodium chloride concentration on the skin, a basis for the diagnostic sweat test.
Choice B rationale
Barrel chest is a consequence of chronic hyperinflation of the lungs due to air trapping, a result of progressive small-airway obstruction from thick, tenacious mucus. Over time, the increased residual volume and functional residual capacity alter the chest wall's shape, increasing its anterior-posterior diameter.
Choice C rationale
Digital clubbing is a painless, symmetric enlargement of the tips of the fingers and toes, a sign of chronic hypoxemia and pulmonary disease. In cystic fibrosis, the progressive lung destruction and resultant impaired gas exchange lead to sustained low blood oxygen levels, triggering the development of clubbing.
Choice D rationale
Bradycardia (abnormally slow heart rate) is not a common manifestation of cystic fibrosis; in fact, chronic hypoxemia and the increased metabolic demands of respiratory distress often lead to tachycardia (abnormally fast heart rate). Bradycardia is usually a late, ominous sign of severe decompensation.
Choice E rationale
Steatorrhea, the passage of foul-smelling, fatty stools, results from the failure of the exocrine pancreas to secrete sufficient digestive enzymes (lipase, protease, amylase) into the small intestine. The thick mucus blocks the pancreatic ducts, preventing the essential enzymes from reaching the chyme for proper fat absorption.
Nursing Test Bank
Naxlex Comprehensive Predictor Exams
Related Questions
Correct Answer is A
Explanation
Choice A rationale
The setting-sun sign is a clinical finding where the infant's eyes appear to be driven downward, with the sclera visible above the iris, resembling the sun setting below the horizon. This is highly indicative of hydrocephalus as the increased intracranial pressure (ICP) presses on the midbrain tectum, causing paralysis of upward gaze.
Choice B rationale
A high-pitched, shrill cry is a non-specific sign of neurological distress and is often associated with increased intracranial pressure (ICP). While it can occur with hydrocephalus, it is a less specific and less consistent finding than the setting-sun sign, which directly reflects the mechanical pressure effects on the oculomotor and associated midbrain structures.
Choice C rationale
A sunken or depressed fontanel is a classic sign of dehydration or hypovolemia due to decreased intracranial volume. Conversely, an infant with hydrocephalus, characterized by excessive cerebrospinal fluid accumulation, will typically present with a bulging, tense, and non-pulsatile anterior fontanel due to the severely elevated intracranial pressure.
Choice D rationale
Normal head circumference would be an unexpected finding in an infant with significant, uncompensated hydrocephalus. The defining clinical feature in infants is the rapid and often exaggerated increase in head circumference (macrocephaly), as the cranial sutures are not yet fused, allowing the head to expand markedly to accommodate the accumulating cerebrospinal fluid and rising pressure.
Correct Answer is D
Explanation
Choice A rationale
Administering half the dose is inappropriate and lacks a scientific basis unless ordered by the provider, as it would likely be sub-therapeutic and fail to achieve the desired effect of improving myocardial contractility and reducing heart rate. Digoxin administration should be all or none based on parameters.
Choice B rationale
The acceptable apical pulse rate for administering digoxin to an infant is typically above 90-110 beats per minute (bpm). An apical pulse of 88 bpm falls below this generally accepted cutoff, indicating potential drug toxicity or the therapeutic effect lowering the rate too much, necessitating withholding the dose.
Choice C rationale
Administering double the dose is a dangerous, unscientific action that would significantly increase the risk of digoxin toxicity, which can cause severe cardiac arrhythmias, gastrointestinal symptoms, and potential cardiac arrest, particularly in infants who are highly susceptible to small dose changes.
Choice D rationale
The normal heart rate for an infant is significantly higher than 88 bpm. Holding the dose and notifying the provider is the required action. Bradycardia in an infant receiving digoxin is the cardinal sign of potential toxicity or an excessively deep therapeutic effect, and the provider must assess the situation before further dosing.
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