Regarding cisapride:

Correct Answer: B

Rationale: Cisapride is a medication that acts as a prokinetic agent, commonly used in the treatment of gastrointestinal motility disorders. The correct answer, B) Fatal arrhythmias have been reported, is the right choice because cisapride has been associated with serious cardiac side effects, including fatal arrhythmias. This led to the withdrawal of cisapride from many markets due to safety concerns. Option A) It is licensed for use in children, is incorrect because due to its significant cardiac side effects, cisapride is not recommended for use in children. Option C) Concurrent administration with erythromycin is not advised, is incorrect because the interaction between cisapride and erythromycin can result in serious cardiac side effects, and this combination is contraindicated. Option D) It commonly causes constipation, is incorrect because constipation is not a common side effect of cisapride. In an educational context, it is essential for healthcare providers, especially nurses, to be aware of the potential risks associated with medications, such as cisapride, to ensure patient safety. Understanding the pharmacological properties, indications, contraindications, and adverse effects of medications is crucial for providing safe and effective care to pediatric patients with cardiovascular conditions.

Correct Answer: A

Rationale: Elevated levels of CPK (creatine phosphokinase) are seen in Duchenne muscular dystrophy (DMD) because DMD is a genetic disorder characterized by progressive muscle degeneration and weakness. CPK is an enzyme found in muscle cells, and its levels rise when there is muscle damage or breakdown, which occurs in DMD due to the absence of dystrophin protein. Dermatomyositis is an inflammatory muscle disease that can also lead to muscle damage, but CPK levels are typically not as elevated in dermatomyositis compared to DMD. Spinal muscular atrophy is a genetic disorder affecting motor neurons, not muscle cells, so CPK levels would not be significantly elevated in this condition. Malignant hyperthermia is a rare, life-threatening condition triggered by certain medications during anesthesia, and it does not directly cause muscle breakdown leading to elevated CPK levels. In an educational context, understanding the relationship between specific conditions and laboratory findings like CPK levels is crucial for healthcare professionals, especially nurses working with pediatric patients. This knowledge helps in proper diagnosis, monitoring disease progression, and providing appropriate care interventions. It also highlights the importance of recognizing subtle differences between conditions to make accurate clinical judgments.

Correct Answer: C

Rationale: In pediatric pharmacology, understanding the role of the complement system in immune function is crucial. Defects in the complement system can lead to increased susceptibility to certain infections. In this case, the correct answer is C) Recurrent meningococcal infection. Meningococcal infections are known to be associated with complement deficiencies, making this option the most likely indicator of a defect in the complement system. Option A) Recurrent folliculitis is less likely to be associated with complement system defects as folliculitis is primarily a bacterial infection of the hair follicles and is not a common presentation of complement deficiencies. Option B) Facial swelling following trauma is also less likely to be related to complement system defects, as trauma-induced swelling is more indicative of local tissue response to injury rather than a systemic immune deficiency. Option D) Pneumocystis carinii pneumonia is typically associated with T-cell immunodeficiencies rather than complement system defects. The complement system plays a lesser role in defense against Pneumocystis carinii compared to T-cell mediated immunity. Educationally, this question highlights the importance of recognizing different clinical presentations associated with complement deficiencies in pediatric patients. Understanding these associations can help healthcare providers in identifying and managing underlying immune deficiencies in children with recurrent infections.

Correct Answer: D

Rationale: In the context of pharmacology, understanding the proteins found in muscle fibers is crucial for nurses caring for pediatric patients with cardiovascular conditions. The correct answer is D) Desmin. Desmin is a type III intermediate filament protein that plays a key structural role in muscle cells, providing support and organization to the muscle fibers. It is specifically found in muscle cells, contributing to their contractile function and integrity. Option A) Vimentin is a type III intermediate filament protein found in connective tissue cells, not muscle fibers. Option B) Saccharin is an artificial sweetener and not a protein found in muscle fibers. Option C) Keratin is a structural protein found in epithelial cells like skin and hair, not in muscle fibers. Understanding the specific proteins found in muscle fibers is essential for nurses to comprehend the pathophysiology of cardiovascular conditions in pediatric patients. This knowledge helps in assessing muscle function, anticipating complications, and providing appropriate care interventions. By grasping the role of proteins like Desmin in muscle fibers, nurses can better understand the impact of cardiovascular medications and treatments on muscle function in pediatric patients.

Correct Answer: A

Rationale: Marfan's syndrome is a genetic disorder that affects the body's connective tissue, primarily in the cardiovascular, ocular, and skeletal systems. The gene mutation typically associated with Marfan's syndrome is FBN1, which encodes for fibrillin-1, a crucial component of microfibrils in the extracellular matrix. Mutations in FBN1 lead to structural weakness in connective tissue, resulting in the characteristic features of Marfan's syndrome. Option A (FBN1) is correct because mutations in this gene are well-documented in individuals with Marfan's syndrome. Option B (ACTA2) is associated with a different condition called familial thoracic aortic aneurysm and dissection, not Marfan's syndrome. Option C (SIX2) is related to developmental abnormalities in the kidney and not associated with Marfan's syndrome. Option D (TGFBR2) mutations are linked to Loeys-Dietz syndrome, another connective tissue disorder distinct from Marfan's syndrome. Understanding the genetic basis of Marfan's syndrome is crucial for healthcare providers, especially in pediatrics, as early diagnosis and management can significantly impact patient outcomes. Recognizing the specific gene mutation associated with Marfan's syndrome helps in providing targeted interventions and genetic counseling to affected individuals and their families.