Which of the following conditions results primarily from mutation in the gene encoding Vitamin D receptor?

Correct Answer: B

Rationale: In pediatric nursing, understanding the etiology of various cardiovascular disorders is crucial for effective patient care. In this context, the correct answer is B) Vitamin D dependent rickets type 2. This condition primarily results from a mutation in the gene encoding the Vitamin D receptor. Vitamin D dependent rickets type 1 (Option A) is caused by mutations in the CYP27B1 gene, leading to impaired activation of Vitamin D. X-linked hypophosphatemic rickets (Option C) is caused by mutations in the PHEX gene, affecting phosphate regulation. Autosomal dominant hypophosphatemic rickets (Option D) is caused by mutations in the FGF23 gene, disrupting phosphate metabolism. Educationally, understanding the genetic basis of various rickets types is essential for nurses caring for pediatric patients with cardiovascular disorders. This knowledge helps in accurate diagnosis, treatment planning, and patient education regarding the inheritance patterns and long-term management of these conditions. By grasping the genetic underpinnings, nurses can provide holistic care and support to pediatric patients and their families.

Correct Answer: B

Rationale: Pediatric nursing students need to understand transient neonatal pustular melanosis (TNPM) to provide optimal care for newborns. Option B is incorrect because TNPM does not typically present with intracorneal or subcorneal pustules filled with eosinophils. The correct answer is A. Increased melanization of epidermal cells is characteristic of TNPM. Option C is true as the pustular phase of TNPM usually resolves within 2-3 days. Option D is also accurate as lesions of pustular melanosis do not contain bacteria. Educationally, it is vital for students to grasp the differences in neonatal skin conditions to make accurate diagnoses and provide appropriate care. Understanding the key features of TNPM, such as the absence of intracorneal or subcorneal pustules, helps students differentiate it from other skin conditions. By explaining why each option is correct or incorrect, students can deepen their knowledge and develop critical thinking skills essential for clinical practice in pediatric nursing.

Correct Answer: D

Rationale: In pediatric nursing, understanding developmental milestones is crucial for assessing normal growth and development. Bed-wetting, also known as enuresis, is a common concern for parents. The correct answer, D) 5 years in both boys and girls, is based on developmental norms. Children typically achieve nighttime continence by the age of 5. Boys and girls may vary in the age at which they achieve this milestone, but by the age of 5, most children should be able to stay dry through the night. Therefore, option D is correct as it reflects this developmental expectation. Option A) 4 years in boys and 5 years in girls is incorrect because it reverses the typical trend of girls achieving bladder control earlier than boys. Option B) 5 years in boys and 4 years in girls is also incorrect for the same reason. Option C) 4 years in both boys and girls is incorrect as it does not account for the developmental differences between boys and girls in achieving nighttime continence. Educationally, this question highlights the importance of understanding normal developmental milestones in pediatric nursing practice. By knowing these norms, nurses can provide appropriate guidance and support to parents and caregivers, helping them differentiate between normal developmental variations and potential concerns.

Correct Answer: B

Rationale: In the context of pediatric nursing and cardiovascular disorders, understanding conditions like cerebral salt wasting is crucial for providing safe and effective care to pediatric patients. In this question, the correct answer is B) High intravascular volume. Cerebral salt wasting is characterized by hyponatremia (low serum sodium levels), high urine output, and low vasopressin levels. However, the incorrect option B states high intravascular volume, which is not a characteristic of cerebral salt wasting. Low serum sodium (option A) is a hallmark feature of cerebral salt wasting as excessive renal sodium loss leads to hyponatremia. High urine output (option C) is also a common presentation in cerebral salt wasting due to the excessive excretion of sodium in the urine. Low vasopressin level (option D) is associated with cerebral salt wasting as vasopressin, an antidiuretic hormone, plays a role in regulating water retention in the body. Educationally, understanding the pathophysiology and clinical manifestations of cerebral salt wasting is essential for nurses caring for pediatric patients with cardiovascular disorders. By knowing the characteristic features of this condition, nurses can promptly recognize and intervene to prevent complications associated with hyponatremia and volume depletion in pediatric patients. This knowledge enhances patient safety and improves outcomes in pediatric nursing practice.

Correct Answer: C

Rationale: In the treatment of hyperammonemia due to certain urea cycle disorders in pediatric patients, the correct amino acid to use is C) Arginine. Arginine is essential in urea cycle metabolism as it helps in the detoxification of ammonia, a byproduct of protein metabolism. Patients with urea cycle disorders have impaired ammonia detoxification, leading to hyperammonemia. Supplementing with arginine can help in the excretion of excess ammonia and alleviate symptoms. Option A) Leucine, Option B) Valine, and Option D) Isoleucine are incorrect choices for the treatment of hyperammonemia. While these amino acids are important in protein synthesis and metabolism, they do not play a specific role in the urea cycle for ammonia detoxification. In the context of pediatric nursing and pharmacology, understanding the specific metabolic pathways and functions of amino acids is crucial for providing effective care to patients with urea cycle disorders. Administering the correct amino acid, such as arginine, can make a significant difference in managing and treating hyperammonemia in these patients.