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 B
Explanation
Choice A rationale
Kawasaki disease (KD) is an acute systemic vasculitis often leading to an inflammatory state characterized by fever, conjunctivitis, and rash. The child is expected to be tachycardic and often hypertensive (due to inflammation and hyperdynamic state), or may be hypotensive in later shock, but bradycardia and hypotension are not typical diagnostic findings in the acute phase.
Choice B rationale
The presence of strawberry tongue (erythema and prominent papillae) and peeling (desquamation) of the palms and soles (especially in the subacute phase) are two of the key mucocutaneous findings that fulfill the American Heart Association's diagnostic criteria for Kawasaki disease. This systemic inflammatory response targets epithelial and vascular tissues.
Choice C rationale
Petechiae, small pinpoint hemorrhages, are often associated with conditions like meningococcemia, septicemia, or platelet disorders such as Idiopathic Thrombocytopenic Purpura (ITP). While a non-specific maculopapular rash or perineal desquamation can occur in Kawasaki disease (KD), petechiae are not considered a classic or defining diagnostic criterion.
Choice D rationale
Splinter hemorrhages are small lines of blood under the fingernails or toenails, classically associated with infective endocarditis (microemboli lodging in the nail bed capillaries). While Kawasaki disease can lead to carditis, splinter hemorrhages are not a specific assessment finding that supports the initial diagnosis of this particular systemic vasculitis.
Correct Answer is A
Explanation
Choice A rationale
During a tonic-clonic seizure, loss of consciousness and uncontrolled muscle movements carry a high risk of aspiration if the child vomits or has excessive oral secretions. Turning the child to the side (recovery position) allows gravity to drain secretions and prevents the tongue from obstructing the posterior pharynx, which is the most critical immediate safety intervention.
Choice B rationale
While benzodiazepines (like rectal or intranasal diazepam or midazolam) are used to stop seizures, this is done via a non-oral route as the child is typically unconscious and unable to safely swallow during a seizure, posing a severe aspiration risk. Oral administration is contraindicated during the acute seizure event itself.
Choice C rationale
Inserting a tongue blade or any object into the mouth during a seizure is strictly contraindicated. The child's jaw may clamp down forcefully, causing injury to the child's teeth, gums, or jaw, or causing the object to break and obstruct the airway, leading to far more harm than benefit.
Choice D rationale
Restraining the child during a seizure is inappropriate and dangerous. The forceful muscle contractions can cause injury (fractures or dislocations) if movement is restricted against the convulsive forces. The nurse's role is to clear the area and protect the head and limbs from striking hard objects.
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