From the Guidelines
Polycystic kidney disease (PKD) management should prioritize blood pressure control, typically using ACE inhibitors like lisinopril (10-40 mg daily) or ARBs such as losartan (25-100 mg daily), and consider the use of tolvaptan in appropriate patients, as recommended by the KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD) 1.
Key Management Strategies
- Blood pressure control is crucial to slow disease progression
- ACE inhibitors or ARBs are recommended for blood pressure management
- Tolvaptan may be considered for slowing cyst growth in appropriate ADPKD patients, but requires liver function monitoring
- Patients should maintain adequate hydration, limit caffeine and alcohol, follow a low-sodium diet, and avoid NSAIDs
- Regular monitoring includes blood pressure checks, kidney function tests, and imaging studies every 1-3 years
- Genetic counseling is recommended for family planning, as ADPKD has a 50% chance of transmission to children
Diagnosis and Screening
- The KDIGO 2025 guideline recommends the use of the Mayo Imaging Classification (MIC) to predict future decline in kidney function and the timing of kidney failure 1
- Genetic testing is recommended for infants with very-early-onset symptomatic disease and for children with a negative family history and progressive disease 1
- Ultrasonography is the current radiological method of choice for screening, but a negative scan cannot rule out ADPKD in childhood 1
Lifestyle Modifications
- Patients with ADPKD should be encouraged to achieve a low dietary salt intake, as recommended for all children 1
- Lifestyle measures, such as maintaining a healthy weight and exercising regularly, may also be beneficial in slowing disease progression, although the evidence is limited 1
From the Research
Overview of Polycystic Kidney Disease
- Polycystic kidney disease (PKD) is a chronic, progressive hereditary condition characterized by abnormal development and growth of cysts in the kidneys and other organs 2.
- Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by kidney cyst formation and progressive kidney function loss 3.
Dietary Interventions for PKD
- Dietary interventions such as caloric restriction, intermittent fasting, and ketogenic diet have recently emerged as potential strategies to induce metabolic reprogramming and slow ADPKD progression 3.
- A ketogenic diet, calorie restriction, intermittent fasting, and time-restricted feeding can reduce aerobic glycolysis and inhibit the mTOR pathway, producing a reduction in cyst cell proliferation, a reduction in kidney volume, and helping to preserve kidney function 4.
- Patients with PKD are advised to follow a dietary regimen that aims to achieve or maintain an ideal body weight and includes high fluid intake, low sodium, and limited concentrated sweets 3.
Importance of Sodium Restriction
- Dietary salt restriction is beneficial to the management of autosomal dominant polycystic kidney disease (ADPKD) 5.
- Urinary sodium excretion is associated with the rate of total kidney volume increase, and sodium restriction can slow the progression of ADPKD 5.
Barriers to Adherence
- Barriers to adherence to prescribed therapies for PKD include personal beliefs and confusion due to unclear recommendations 6.
- Patients with PKD are motivated and willing to incorporate blood pressure control, exercise, low-salt diet, and high volume water intake into their daily routines, but may need specific recommendations on how to implement these 6.
Future Research Directions
- Extensive clinical research is necessary to establish the effectiveness and long-term effects of dietary interventions and supplementation in ADPKD 3.
- Future research should prioritize well-designed clinical trials to determine the benefits and safety of dietary interventions and supplementation in ADPKD 3.