Autosomal dominant polycystic kidney disease is the most common hereditary human kidney disease. All the following are true EXCEPT

Correct Answer: D

Rationale: In this question, option D, "right-sided valvular heart disease," is the correct answer because it is NOT associated with autosomal dominant polycystic kidney disease (ADPKD). The presence of valvular heart disease is not a typical feature of ADPKD. Option A, "gross or microscopic hematuria," is a common manifestation of ADPKD due to the development of cysts in the kidneys leading to bleeding within the urinary tract. Option B, "may be seen in neonates," is true because ADPKD can be diagnosed at any age, including in neonates if there is a family history of the disease. Option C, "abnormal renal sonography in the absence of symptoms," is also true as ADPKD can be detected through imaging studies like renal sonography even before symptoms manifest, making it a valuable diagnostic tool. In an educational context, understanding the clinical manifestations and diagnostic methods for pediatric genitourinary disorders like ADPKD is crucial for nurses and healthcare professionals caring for pediatric patients. This knowledge helps in early identification, appropriate management, and improving patient outcomes. By reviewing and understanding these concepts, nurses can provide better care and support to children with genitourinary disorders and their families.

Correct Answer: C

Rationale: Nephrotic syndrome is a kidney disorder characterized by increased glomerular permeability, leading to proteinuria, hypoalbuminemia, edema, and hyperlipidemia. One of the complications of nephrotic syndrome is a hypercoagulable state due to changes in coagulation factor levels. This alteration leads to an increased risk of thrombosis, especially venous thromboembolism, in affected individuals. Choice A, hemodilution, is not the correct answer because nephrotic syndrome causes hemoconcentration rather than hemodilution due to the loss of protein-rich fluid into the urine. This loss leads to an increase in blood viscosity, contributing to the hypercoagulable state. Choice B, decreased platelet number, is incorrect as nephrotic syndrome does not typically cause thrombocytopenia. Instead, it is associated with platelet dysfunction and an increased risk of thrombosis. Choice D, decrease in hepatic production of fibrinogen, is also incorrect. In nephrotic syndrome, there is actually an increase in the production of hepatic acute-phase proteins, including fibrinogen, contributing to the hypercoagulable state. Educationally, understanding the pathophysiology of nephrotic syndrome and its complications, such as hypercoagulability, is crucial for healthcare professionals managing pediatric patients with this condition. Recognizing the risk factors for thrombosis in nephrotic syndrome and implementing appropriate monitoring and preventive measures are essential in providing comprehensive care to these patients.

Correct Answer: B

Rationale: In pediatric nephrotic syndrome, the correct answer is B) restrict sodium intake after achieving remission. This is crucial because excessive sodium can lead to fluid retention, edema, and a relapse of the condition. Limiting sodium helps maintain the delicate fluid balance in these children, reducing the risk of complications. Option A) restricting activities is incorrect because children should be encouraged to be active to promote overall health, unless there are specific medical reasons to restrict certain activities. Option C) considering the child chronically ill may not be necessary if they have achieved remission. It is important to support them in leading a normal life while managing their condition. Option D) maintaining an unrestricted diet is incorrect because excessive sodium intake can worsen the condition. Children with nephrotic syndrome should follow a balanced diet with restrictions on sodium and possibly other nutrients depending on their individual needs. In an educational context, understanding the dietary management of pediatric nephrotic syndrome is essential for nurses and healthcare providers caring for these children. By recognizing the importance of sodium restriction post-remission, healthcare professionals can help prevent disease relapse and promote better outcomes for their pediatric patients.

Correct Answer: D

Rationale: In the treatment of cystinosis, the correct answer is D) angiotensin-converting enzyme inhibitors. These medications are used to help manage hypertension that can develop as a complication of chronic renal failure, which is common in patients with cystinosis. Angiotensin-converting enzyme inhibitors work by dilating blood vessels, reducing blood pressure, and reducing the workload on the heart. Option A) cystine eye drops are not the appropriate treatment for correcting the metabolic abnormalities associated with Fanconi syndrome or chronic renal failure in cystinosis. These eye drops are used to treat eye manifestations of cystinosis, such as corneal cystine crystal deposits. Option B) oral cysteine is not the correct treatment either. While cysteine is a component of cystine, it is not the primary focus of treatment for the systemic metabolic abnormalities in cystinosis. Option C) prostaglandin synthesis inhibitors are not typically used in the treatment of cystinosis. These medications are more commonly used for conditions like arthritis or to manage certain types of glaucoma. Educationally, understanding the rationale behind the correct answer helps students grasp the importance of tailoring treatments to address specific aspects of a disease process. In pharmacology, it is crucial to select the most appropriate medication based on the underlying pathophysiology of the condition being treated. This question highlights the importance of considering comorbidities and complications when managing pediatric genitourinary disorders like cystinosis.

Correct Answer: D

Rationale: In pediatric genitourinary disorders, understanding urinary indices is crucial for proper diagnosis and management. In the context of type IV renal tubular acidosis (RTA), the correct answer, option D (aminoaciduria), is not typically seen. Type IV RTA is characterized by hyperkalemia, metabolic acidosis, and decreased urinary ammonium excretion. Aminoaciduria, the presence of amino acids in the urine, is not a classic feature of type IV RTA. Option A, urinary pH being alkaline or acidic, is a common finding in patients with type IV RTA due to impaired acid secretion in the distal tubules. Elevated sodium levels, as in option B, can be seen in type IV RTA due to the impaired sodium reabsorption in the distal nephron. Inappropriately low potassium levels, as in option C, are also a characteristic feature of type IV RTA due to impaired potassium secretion in the distal nephron. Educationally, understanding these urinary indices and their implications in pediatric patients with genitourinary disorders is essential for nurses and healthcare providers to recognize and manage these conditions effectively. It is vital for nurses preparing for the NCLEX exam to grasp these concepts to provide safe and competent care to pediatric patients with renal disorders.