Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic nephropathy, and tolvaptan is the only therapy available. However, tolvaptan slows but does not stop disease progression, is marred by polyuria, and most patients worldwide lack access. This and recent preclinical research findings on the glucose-dependency of cyst-lining cells have renewed interest in the dietary management of ADPKD. We now review the current dietary recommendations for ADPKD patients according to clinical guidelines, the evidence base for those, and the potential impact of preclinical studies addressing the impact of diet on ADPKD progression. The clinical efficacy of tolvaptan has put the focus on water intake and solute ingestion as modifiable factors that may impact tolvaptan tolerance and ADPKD progression. By contrast, dietary modifications suggested to ADPKD patients, such as avoiding caffeine, are not well supported and their impact is unknown. Recent studies have identified a chronic shift in energy production from mitochondrial oxidative phosphorylation to aerobic glycolysis (Warburg effect) as a contributor to cyst growth, rendering cyst cells exquisitely sensitive to glucose availability. Therefore, low calorie or ketogenic diets have delayed preclinical ADPKD progression. Additional preclinical data warn of potential negative impact of excess dietary phosphate or oxalate in ADPKD progression.
Highlights
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic nephropathy and is a sizable contributor to end-stage kidney disease (ESRD) among those younger than70-year-old [1,2]
We review the current dietary recommendations which are common to all chronic kidney disease (CKD) patients and specific for ADPKD patients according to clinical guidelines, the evidence base for those recommendations, and the Nutrients 2019, 11, 1576; doi:10.3390/nu11071576
Similar baseline values were observed in the HALT-PKD randomized clinical trials (RCTs) of renin-angiotensin system (RAS) blockade and blood pressure control, but in these trials sodium intake decreased to 3.5–3.8 g/d (145–160 mmol/d) during the trial and higher sodium intake was associated with faster increase in total kidney volume (TKV) or decrease in glomerular filtration rate (GFR) in study A and B, respectively, but results were not consistent across both post-hoc analyses [98]
Summary
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic nephropathy and is a sizable contributor to end-stage kidney disease (ESRD) among those younger than. Some causes of CKD require specific dietary modifications, as already mentioned for diabetic kidney disease. Decreased intracellular calcium and increased cAMP levels promote cell proliferation and fluid secretion, the drivers of cyst growth [18,19,20]. This information led to the development of therapeutic approaches for ADPKD. The V2R blocker tolvaptan is the only approved drug for ADPKD, following the demonstration in placebo-controlled clinical trials of a protective effect against renal cyst growth, as assessed by the total kidney volume (TKV) and against the progressive loss of GFR [21]. Somatostatin decreases cAMP production and somatostatin agonists have been tested clinically, with less success than tolvaptan [22,23], potentially because of the downregulation of receptors associated with cyst growth [24]
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