Currently, the only pharmacological treatment available for<unk>  consists in reducing the speed in gain of total kidney volume (TKV) with vasopressin receptor 2 (V2) antagonists (i.e. tolvaptan). Tolvaptan treatment does not halt or reverse disease progression and patients still progress towards renal failure. Palliative treatment modalities involve symptomatic medications (nonopioid and opioid analgesics) for abdominal/retroperitoneal pain. Options for analgesic-resistant pain include simple or complex surgical procedures (i.e. renal cyst aspiration, cyst decortication, renal denervation and nephrectomy), which can result in complications inherent to surgery. Recent research suggests that ketogenic dietary interventions beneficially affect the progression and symptoms in individuals with<unk> . Mild weight loss favorably affects pain indicating the benefit of dietary and lifestyle changes. Aquaretic medication In 2014, Japan was the first country in the world to approve a pharmacological treatment for<unk>  followed by Canada and Europe, which approved the drug tolvaptan for<unk>  patients in the beginning of 2015. The USA FDA approved the use of tolvaptan in the treatment of<unk>  in 2018. Tolvaptan, an aquaretic drug, is a vasopressin receptor 2 (V2) antagonist. Pre-clinical studies had suggested that the molecule cAMP could be involved in the enlargement of<unk>  cysts, and studies on rodents confirmed the role of vasopressin in increasing the levels of cAMP in the kidney, which laid the basis for the conduction of clinical studies. Because data from the Consortium for Radiologic Imaging Studies of<unk>  Kidney Disease (CRISP) led by Mayo Clinic showed that total kidney volume (TKV) predicted the risk of developing chronic kidney disease in patients with<unk> , the TEMPO 3:4 trial, which enrolled patients from 129 sites worldwide from 2007 to 2009, evaluated TKV as a primary end-point to test the efficacy of tolvaptan in<unk>  patients. That study showed a significant decrease in the ratio of TKV increase and deterring of renal function decline in<unk>  patients after treatment with tolvaptan; however, because laboratory test results regarding liver function appeared elevated in a percentage of patients enrolled in that study, the approval of the drug was either delayed by regulatory agencies or, as in case of the US, altogether denied. Dietary and lifestyle interventions Research using<unk>  mouse models showed that mild food restriction strongly improved disease progression. The mechanism was shown to involve the metabolic state of ketosis, and beneficial effects could be produced by time-restricted feeding, acute fasting, a ketogenic diet, or by supplementation with the ketone beta-hydroxybutyrate in mouse, rat and cat models of<unk> . A ketogenic diet regimen not only halted further disease progression but led to partial reversal of renal cystic disease in a rat model. The metabolic state of ketosis may be beneficial in<unk>  because renal cyst cells in<unk>  have a metabolic defect similar to the Warburg effect in cancer that makes them highly dependent on glucose, and unable to metabolize fatty acids and ketones. Consistent with this, serum glucose levels positively correlate with faster disease progression in<unk>  patients. Also, individuals with<unk>  and type 2 diabetes have significantly larger total kidney volume (TKV) than those with<unk>  alone, and overweight or obesity associate with faster progression in early-stage<unk> . A retrospective case series study showed that<unk>  disease symptoms - including pain, hypertension and renal function - improved among 131 patients who implemented ketogenic diets for an average duration of 6 months. Dietary intake of sodium is associated with worse renal function decline in<unk> , and limiting sodium intake is generally recommended to patients. Dietary protein intake was not found to correlate with<unk>  progression. Increased water intake is thought to be beneficial in<unk>  and is generally recommended.
Autosomal dominant polycystic kidney disease
Autosomal recessive polycystic kidney disease
Medullary cystic kidney disease
Glomerulocystic kidney disease
Medullary sponge kidney
Juvenile nephronophthisis
Horseshoe kidney
Multicystic dysplastic kidney