Colchicine may be an effective drug to prevent the development of AA amyloidosis in which of the following diseases?

Correct Answer: C

Rationale: Colchicine is an anti-inflammatory medication that is known to be effective in preventing the development of AA amyloidosis, a condition characterized by the deposition of amyloid protein in organs. In this case, the correct answer is C) familial Mediterranean fever (FMF). FMF is an autosomal recessive disease characterized by recurrent episodes of fever, serositis, and arthritis. One of the complications of FMF is the development of AA amyloidosis due to chronic inflammation. Colchicine is the mainstay treatment for FMF and has been shown to reduce the frequency of acute attacks and the risk of developing AA amyloidosis. Option A) juvenile idiopathic arthritis is incorrect because colchicine is not typically used in the treatment of this condition. Juvenile idiopathic arthritis is usually managed with nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and disease-modifying antirheumatic drugs (DMARDs) depending on the subtype. Option B) ankylosing spondylitis is also incorrect as colchicine is not a standard treatment for this condition. Ankylosing spondylitis is managed with NSAIDs, physical therapy, and in some cases, biologic agents like TNF-alpha inhibitors. Option D) hyper IgD syndrome is incorrect because colchicine is not indicated for this genetic autoinflammatory disorder. Hyper IgD syndrome is managed symptomatically, and colchicine is not a primary treatment for this condition. Educationally, understanding the use of colchicine in preventing AA amyloidosis in FMF highlights the importance of appropriate medication choices based on the underlying pathophysiology of a disease. It also emphasizes the significance of recognizing the role of inflammation in certain diseases and how targeted therapies like colchicine can help prevent serious complications like amyloidosis.

Correct Answer: D

Rationale: The correct answer is D) glutaric aciduria type 1. In this scenario, the infant's history of difficult labor, macrocephaly, mild hypotonia, and intracranial bleeding following a viral illness suggests an underlying metabolic disorder like glutaric aciduria type 1. This condition can present with macrocephaly, hypotonia, and acute encephalopathy triggered by a catabolic state like a viral illness. The absence of skull fracture and normal fundoscopy point away from trauma. Option A) residual birth trauma is incorrect because the absence of skull fracture and normal fundoscopy do not support this diagnosis. Option B) arteriovenous malformation is less likely given the clinical presentation and absence of predisposing factors. Option C) acute viral encephalitis is less likely as the symptoms are more indicative of a metabolic disorder than an infectious cause. Educationally, this question highlights the importance of recognizing metabolic disorders in pediatric patients presenting with neurological symptoms. Understanding the clinical features, diagnostic clues, and appropriate management of rare conditions like glutaric aciduria type 1 is crucial for pediatric nurses to provide optimal care for infants in critical situations.

Correct Answer: B

Rationale: In the context of pediatric nursing, understanding jaundice in infants is crucial. The correct answer, option B, indicates physiologic jaundice in a term infant. Physiologic jaundice typically presents within the first 24 hours post-birth and resolves by the first week. The key characteristic is a gradual increase in serum bilirubin levels of less than 5 mg/dL per day. This pattern is considered normal as it results from the breakdown of fetal red blood cells and the immature liver's ability to process bilirubin efficiently. Option A is incorrect because jaundice within the first 24 hours is more likely to be pathological. Option C is incorrect as a high direct serum bilirubin level suggests a pathologic cause such as biliary atresia. Option D is incorrect as jaundice appearing after the first week raises concern for pathological jaundice and further investigation is warranted. Educationally, recognizing the characteristics of physiologic jaundice is vital for pediatric nurses to differentiate normal variations from potentially serious conditions. Understanding the timing, rate of rise in bilirubin levels, and associated factors can guide appropriate interventions and prevent unnecessary anxiety for caregivers. This knowledge ensures safe and effective care for neonates experiencing jaundice.

Correct Answer: B

Rationale: In this scenario, the correct answer is B) Intravenous ampicillin and gentamicin. This treatment is appropriate because the infant is showing signs of respiratory distress and sepsis, which are common complications associated with premature rupture of membranes. Ampicillin and gentamicin are broad-spectrum antibiotics that cover a wide range of potential pathogens that could be causing the sepsis. Option A) Surfactant by aerosol is not the most appropriate choice in this case as the primary issue is likely related to infection rather than respiratory distress syndrome. Option C) Intravenous steroids would not be the first-line treatment in this situation. Steroids are typically used to help with lung development in premature infants at risk for respiratory distress syndrome but are not indicated for the immediate management of sepsis. Option D) Intravenous acyclovir is used to treat viral infections, particularly herpes infections. Since there is no indication of a viral infection in this case, acyclovir would not be the most appropriate treatment. Educationally, this question highlights the importance of recognizing and managing complications in premature infants, such as sepsis, which can quickly become life-threatening. Understanding the appropriate use of antibiotics in neonatal care is crucial for pediatric nurses to provide safe and effective care to this vulnerable population.

Correct Answer: A

Rationale: In this scenario, the correct answer is A) Pneumothorax. When a preterm infant is born, especially at 7 weeks' gestation, their lungs are underdeveloped and fragile, making them susceptible to conditions like pneumothorax, which is the accumulation of air in the pleural space causing lung collapse. This condition can lead to respiratory distress due to compromised lung function. Option B) Respiratory distress syndrome (RDS) is a common condition in preterm infants where the lungs lack surfactant, leading to difficulty in breathing. While RDS is a possible cause of respiratory distress in preterm infants, in this case, the presentation is more indicative of pneumothorax. Option C) Patent ductus arteriosus is a heart condition where a blood vessel fails to close after birth, causing circulation issues. While this can lead to respiratory distress in some cases, it is less likely in this scenario compared to pneumothorax. Option D) Group B streptococcal sepsis is a bacterial infection that can affect newborns, but it typically presents with symptoms like fever, lethargy, poor feeding, and respiratory distress may not be the primary symptom, making it less likely in this case. Educationally, understanding the common complications and conditions in preterm infants is crucial for pediatric nurses to provide prompt and effective care. Knowing the specific signs and symptoms of conditions like pneumothorax can help nurses intervene quickly and improve outcomes for these vulnerable patients.