The most common genetic syndrome associated with Wilm's tumor is

Correct Answer: A

Rationale: The correct answer is A) Beckwith-Wiedemann syndrome. Wilms tumor, a common pediatric kidney cancer, is associated with several genetic syndromes, with Beckwith-Wiedemann syndrome being the most common. This syndrome involves overgrowth, abdominal wall defects, an increased risk of developing Wilms tumor, and other embryonal tumors. Option B) Denys-Drash syndrome is characterized by nephropathy, male pseudohermaphroditism, and Wilms tumor. However, it is less common than Beckwith-Wiedemann syndrome in association with Wilms tumor. Option C) Neurofibromatosis type 1 is associated with various tumors, but not commonly Wilms tumor. Option D) Hereditary leiomyomatosis and renal cell cancer are associated with a different type of kidney cancer, not Wilms tumor. Understanding these associations is crucial in pediatric pharmacology, as it helps healthcare providers in recognizing potential underlying genetic conditions in children with Wilms tumor. Early identification can lead to appropriate monitoring, treatment, and genetic counseling for the affected child and their family.

Correct Answer: D

Rationale: In pediatric kidney transplant recipients, the most important predictor of long-term graft survival is compliance with immunosuppressive therapy (Option D). This is because adherence to the prescribed immunosuppressive regimen is crucial in preventing rejection of the transplanted kidney. Non-compliance can lead to rejection episodes and ultimately graft failure. HLA matching (Option A) is important in reducing the risk of rejection initially, but long-term graft survival is more heavily influenced by compliance with medication. Donor age (Option B) and recipient age (Option C) may have some impact on outcomes but are not as critical as medication adherence in ensuring graft survival. Educationally, this question highlights the significant role of patient compliance in the success of kidney transplants. It emphasizes the importance of patient education, support, and monitoring in pediatric transplant care to optimize outcomes. Healthcare providers must prioritize educating patients and families on the importance of adhering to medication regimens to ensure the best possible long-term graft survival.

Correct Answer: C

Rationale: In this scenario, the correct answer is C) Calcium gluconate via slow IV push. The child with acute renal failure (ARF) is experiencing symptoms of hyperkalemia, as indicated by the widened QRS complex and premature ventricular contractions (PVCs) on the cardiac monitor. Calcium gluconate is administered to stabilize the myocardial cell membrane and counteract the effects of hyperkalemia on the heart. Option A, isotonic saline with KCl, would worsen the hyperkalemia by adding more potassium. Option B, sodium bicarbonate, is used in metabolic acidosis, not to treat hyperkalemia. Option D, oral potassium supplements, is contraindicated in the presence of hyperkalemia. Educationally, understanding the appropriate treatment for electrolyte imbalances like hyperkalemia in pediatric patients is crucial for nurses caring for children with renal disorders. Recognizing ECG changes associated with hyperkalemia and knowing the correct intervention, such as administering calcium gluconate, can prevent life-threatening cardiac complications in these vulnerable patients.

Correct Answer: C

Rationale: In the case of a child with minimal change nephrotic syndrome (MCNS) experiencing generalized edema and skin breakdown due to Lasix administration, the correct treatment plan to reduce edema would be to administer intravenous albumin (Option C). In MCNS, there is a loss of proteins like albumin in the urine, leading to hypoalbuminemia and edema. Administering albumin intravenously helps to restore colloid osmotic pressure, reducing edema by drawing fluid back into the blood vessels from the interstitial space. Options A and B, increasing Lasix dosage or adding another diuretic, would further exacerbate the issue by promoting more fluid loss without addressing the underlying cause of protein loss. Option D, eliminating all fluids and sodium from the child's diet, is inappropriate as it can lead to dehydration and electrolyte imbalances, which are especially dangerous in pediatric patients. Educationally, this scenario highlights the importance of understanding the pathophysiology of pediatric GU disorders and the rationale behind treatment choices. It emphasizes the need for a targeted approach that addresses the underlying mechanisms of the condition to provide effective and safe care for pediatric patients with nephrotic syndrome.

Correct Answer: A

Rationale: In a child with chronic renal failure (CRF), chronic hypertension is primarily due to the retention of sodium and water, making option A the correct choice. When the kidneys are compromised in CRF, they are unable to effectively regulate sodium and water balance, leading to excessive retention of these substances. This retention results in an increase in extracellular fluid volume and subsequently, elevated blood pressure. Option B, obstruction of the urinary system, is not the primary cause of chronic hypertension in CRF. While urinary obstruction can lead to kidney dysfunction and hypertension, it is not the typical mechanism in the context of CRF. Option C, accumulation of waste products, is more closely associated with the symptoms of uremia rather than chronic hypertension in CRF. While the accumulation of waste products can contribute to overall complications in CRF, it is not the primary driver of hypertension. Option D, generalized metabolic alkalosis, is not directly linked to chronic hypertension in a child with CRF. Metabolic alkalosis involves an imbalance in acid-base regulation, which can occur in various conditions but is not the main factor contributing to hypertension in the setting of CRF. Educationally, understanding the underlying mechanisms of hypertension in children with CRF is crucial for healthcare providers managing these patients. By recognizing the role of sodium and water retention in elevating blood pressure, clinicians can implement appropriate interventions to help control hypertension and mitigate associated risks in pediatric patients with CRF.