NCLEX Review Guide: Polycystic Kidney Disease (PKD)

Understanding PKD

• Polycystic Kidney Disease (PKD) is a genetic disorder characterized by the development of multiple fluid-filled cysts in the kidneys that gradually enlarge and compress normal kidney tissue.
• Two main types exist: Autosomal Dominant PKD (ADPKD) - most common, adult-onset; and Autosomal Recessive PKD (ARPKD) - rare, infantile form.

Signs & Symptoms

• Flank pain is the most common presenting symptom, often described as dull, aching, or sharp due to cyst expansion and bleeding.
• Hypertension occurs in 60-70% of patients before kidney function decline and is often the first sign of disease.
• Hematuria (gross or microscopic) occurs due to cyst rupture or kidney stone formation.
• Urinary tract infections are frequent due to cyst compression and urinary stasis.

Key Diagnostic Tests

• Ultrasound is the initial screening test showing enlarged kidneys with multiple cysts of varying sizes.
• CT or MRI provides detailed imaging for definitive diagnosis and monitoring disease progression.
• Genetic testing confirms diagnosis and helps with family counseling and screening.
• Laboratory studies include BUN, creatinine, GFR to assess kidney function and urinalysis for hematuria and proteinuria.

Priority Interventions

1. Blood pressure management - ACE inhibitors or ARBs are first-line to slow progression
2. Pain management with non-nephrotoxic analgesics (avoid NSAIDs)
3. Infection prevention and prompt treatment of UTIs
4. Dietary modifications: sodium restriction, adequate hydration, protein restriction if CKD present
5. Patient education on disease progression and when to seek medical attention

Long-term Complications

• End-stage renal disease (ESRD) typically develops by age 60 in ADPKD patients, requiring dialysis or transplantation.
• Cardiovascular complications include mitral valve prolapse, aortic root dilation, and increased risk of aneurysms.
• Hepatic cysts occur in 80% of patients but rarely cause symptoms or require intervention.
• Intracranial aneurysms occur in 8-10% of patients and may require screening in high-risk individuals.