A young school-age child with a history of a waddling gait and frequent falls is brought into the hospital for diagnostic testing. When explaining the diagnostic testing to the parents, the nurse should provide information based on which understanding of the underlying disease pathology?
Systemic autoimmune vasculopathy.
Muscle fiber degeneration.
Autonomic neuropathy.
Impaired neuron function.
The Correct Answer is B
The clinical presentation of a waddling gait and frequent falls in a school-age child is a classic hallmark of Duchenne Muscular Dystrophy (DMD). This X-linked recessive disorder is characterized by the absence of dystrophin, a protein vital for maintaining the structural integrity of muscle cell membranes. Without dystrophin, the repetitive stress of muscle contraction causes microscopic tears in the sarcolemma, leading to progressive necrosis and fibrosis of the muscle tissue.
Rationale:
A. Systemic autoimmune vasculopathy refers to conditions like Juvenile Dermatomyositis, which involves inflammation of the blood vessels and muscles. While this can cause weakness, it typically presents with skin rashes (Grotton papules) and acute systemic symptoms rather than the slow, progressive waddling gait seen in genetic dystrophies.
B. Muscle fiber degeneration is the core pathological process of muscular dystrophy. As the muscle fibers break down, they are gradually replaced by fatty and connective tissue (pseudohypertrophy). This leads to a loss of muscle strength, starting in the proximal muscles of the pelvic girdle, which forces the child to use a waddling gait and the Gowers' sign (using the hands to "climb up" the legs) to stand.
C. Autonomic neuropathy involves damage to the nerves that regulate involuntary body functions, such as heart rate, blood pressure, and digestion. While certain neurological conditions can cause motor issues, autonomic dysfunction does not manifest as the primary muscle wasting or mechanical gait changes described in this pediatric scenario.
D. Impaired neuron function is the hallmark of neurodegenerative or neuromuscular junction disorders, such as Spinal Muscular Atrophy (SMA) or Myasthenia Gravis. In those cases, the problem lies in the signal being sent to the muscle. In muscular dystrophy, the neurons are healthy, but the muscle fiber itself is structurally defective and unable to respond to the signals.
Nursing Test Bank
Naxlex Comprehensive Predictor Exams
Related Questions
Correct Answer is ["100"]
Explanation
Brief introduction:
Surgical prophylaxis with antibiotics like cefazolin is a standard clinical practice designed to reduce the risk of surgical site infections. To ensure therapeutic tissue levels at the time of the initial incision, the medication must be timed and dosed accurately. Dosing is weight-dependent to account for the volume of distribution, particularly in larger patients.
Rationale:
Step 1: Convert weight to pounds
90 kg × 2.2 = 198 lb
Step 2: Determine correct protocol
198 lb is less than 265.5 lb, so use:
2 g cefazolin in 100 mL over 1 hour
Step 3: Identify formula
mL/hr = total volume ÷ time
Step 4: Insert values
= 100 mL ÷ 1 hr
Step 5: Calculate
= 100 mL/hr
Correct Answer is D
Explanation
Brief introduction:
Type 1 diabetes involves absolute insulin deficiency due to pancreatic beta-cell destruction. In severe hyperglycemia, metabolic shifts and gastric stasis often trigger emesis. Immediate airway protection is vital to prevent aspiration pneumonia and respiratory compromise during acute vomiting episodes.
Rationale:
A. Providing an emesis basin is a secondary supportive measure. While it helps contain gastric contents, it does not address the immediate physiological threat of airway obstruction or pulmonary aspiration. The nurse must prioritize patient positioning to ensure that vomitus is expelled safely from the oropharynx.
B. Checking the serum glucose level is necessary for ongoing monitoring of hyperglycemic states. However, this assessment provides data rather than immediate life-saving intervention. In an acute vomiting episode, protecting the ventilatory status takes precedence over checking biochemical markers that were recently confirmed at 420 mg/dL.
C. Hanging a bag of normal saline is essential for correcting osmotic diuresis and dehydration. Although fluid resuscitation is a cornerstone of managing severely elevated glucose, it is not the priority action when a client is actively vomiting. Airway management always supersedes circulatory support in the clinical hierarchy.
D. Turning the client to a lateral position is the priority nursing action. This maneuver uses gravity to prevent the inhalation of gastric contents into the tracheobronchial tree. In an older adult, maintaining airway patency during emesis is the first step in preventing life-threatening aspiration and ensuring safety.
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