Editing Autosomal dominant polycystic kidney disease (section)

==Treatment==
Currently, the only pharmacological treatment available for ADPKD consists in reducing the speed in gain of total kidney volume (TKV) with vasopressin receptor 2 (V2) antagonists (i.e. tolvaptan).<ref>{{cite web |url=https://www.lecturio.com/concepts/autosomal-dominant-polycystic-kidney-disease/ | title= Autosomal Dominant Polycystic Kidney Disease |website= The Lecturio Medical Concept Library |access-date= 3 July 2021}}</ref> 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.{{citation needed|date=June 2016}} Recent research suggests that ketogenic dietary interventions beneficially affect the progression and symptoms in individuals with ADPKD.<ref name = "Strubl_2021">{{cite journal | vauthors = Strubl S, Oehm S, Torres JA, Grundmann F, Haratani J, Decker M, Vuong S, Kaur Bhandal A, Methot N, Haynie-Cion R, Meyer F | display-authors = 6 | title = Ketogenic dietary interventions in autosomal dominant polycystic kidney disease—a retrospective case series study: first insights into feasibility, safety and effects. | journal = Clinical Kidney Journal | date = 2021  | volume = 15 | issue = 6 | pages = 1079–1092 |doi= 10.1093/ckj/sfab162 | pmid = 35664270 | pmc = 9155228 }}</ref>  Mild weight loss favorably affects pain<ref>{{cite journal | vauthors = Nowak KL, Murray K, You Z, Gitomer B, Brosnahan G, Abebe KZ, Braun W, Chapman A, Harris PC, Miskulin D, Perrone R, Torres V, Steinman T, Yu A, Chonchol M | display-authors = 6 | title = Pain and Obesity in Autosomal Dominant Polycystic Kidney Disease: A Post Hoc Analysis of the Halt Progression of Polycystic Kidney Disease (HALT-PKD) Studies | journal = Kidney Medicine | volume = 3 | issue = 4 | pages = 536–545.e1 | date = July 1, 2021 | pmid = 34401721 | pmc = 8350824 | doi = 10.1016/j.xkme.2021.03.004 }}</ref> 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 ADPKD<ref name="TP-150515-017" /> followed by Canada and Europe, which approved the drug [[tolvaptan]] for ADPKD patients in the beginning of 2015.  The USA FDA approved the use of tolvaptan in the treatment of ADPKD in 2018.<ref>{{Cite web | url=https://www.renalandurologynews.com/chronic-kidney-disease-ckd/tolvaptan-receives-fda-approved-for-treating-adpkd-in-adults/article/761347/ | title=Tolvaptan Cleared in US for ADPKD in Adults| work=Renal and Urology News| date=2018-04-26}}</ref> Tolvaptan, an [[aquaretic]] drug, is a [[vasopressin receptor]] 2 (V2) [[antagonist drug|antagonist]].<ref name="TP-150514-004" /> Pre-clinical studies had suggested that the molecule [[Cyclic AMP|cAMP]] could be involved in the enlargement of ADPKD cysts,<ref name="Hanaoka and Guggino">{{cite journal | vauthors = Hanaoka K, Guggino WB | title = cAMP regulates cell proliferation and cyst formation in autosomal polycystic kidney disease cells | journal = Journal of the American Society of Nephrology | volume = 11 | issue = 7 | pages = 1179–1187 | date = July 2000 | pmid = 10864573 | doi = 10.1681/ASN.V1171179 | doi-access = free }}</ref> 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.<ref name="TP-150519-E44">{{cite journal | vauthors = Juul KV, Bichet DG, Nielsen S, Nørgaard JP | title = The physiological and pathophysiological functions of renal and extrarenal vasopressin V2 receptors | journal = American Journal of Physiology. Renal Physiology | volume = 306 | issue = 9 | pages = F931–F940 | date = May 2014 | pmid = 24598801 | doi = 10.1152/ajprenal.00604.2013 }}</ref> Because data from the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) led by [[Mayo Clinic]] showed that total kidney volume (TKV) predicted the risk of developing [[chronic kidney disease]] in patients with ADPKD,<ref name="TP-150515-017" /><ref name="TP-150519-E39">{{cite journal | vauthors = Irazabal MV, Rangel LJ, Bergstralh EJ, Osborn SL, Harmon AJ, Sundsbak JL, Bae KT, Chapman AB, Grantham JJ, Mrug M, Hogan MC, El-Zoghby ZM, Harris PC, Erickson BJ, King BF, Torres VE | display-authors = 6 | title = Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials | journal = Journal of the American Society of Nephrology | volume = 26 | issue = 1 | pages = 160–172 | date = January 2015 | pmid = 24904092 | pmc = 4279733 | doi = 10.1681/ASN.2013101138 }}</ref> the TEMPO 3:4 trial, which enrolled patients from 129 sites worldwide from 2007 to 2009, evaluated TKV as a [[Clinical endpoint|primary end-point]] to test the efficacy of tolvaptan in ADPKD patients.<ref name="TP-150514-004" /><ref name="TP-150514-005" /> That study showed a significant decrease in the ratio of TKV increase and deterring of [[renal function]] decline in ADPKD patients after treatment with tolvaptan;<ref name="TP-150514-004" /><ref name="TP-150515-007">{{cite journal | vauthors = Kelsey R | title = Polycystic kidney disease: Tolvaptan in ADPKD-TEMPO 3:4 trial results | journal = Nature Reviews. Nephrology | volume = 9 | issue = 1 | pages = 1 | date = January 2013 | pmid = 23183839 | doi = 10.1038/nrneph.2012.236 | s2cid = 22942772 | doi-access = free }}</ref> 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.<ref name="TP-150514-005" /><ref name="TP-150515-012">{{cite journal |author= Brown T |title= Tolvaptan Not Recommended for ADPKD |journal= Medscape |year= 2013 |url= http://www.medscape.com/viewarticle/809028}}</ref>

===Dietary and lifestyle interventions===
Research using ADPKD mouse models showed that mild food restriction strongly improved disease progression.<ref>{{cite journal | vauthors = Kipp KR, Rezaei M, Lin L, Dewey EC, Weimbs T | title = A mild reduction of food intake slows disease progression in an orthologous mouse model of polycystic kidney disease | journal = American Journal of Physiology. Renal Physiology | volume = 310 | issue = 8 | pages = F726–F731 | date = April 2016 | pmid = 26764208 | pmc = 4835927 | doi = 10.1152/ajprenal.00551.2015 }}</ref> 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 ADPKD.<ref name="rat model">{{cite journal | vauthors = Torres JA, Kruger SL, Broderick C, Amarlkhagva T, Agrawal S, Dodam JR, Mrug M, Lyons LA, Weimbs T | display-authors = 6 | title = Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease | journal = Cell Metabolism | volume = 30 | issue = 6 | pages = 1007–1023.e5 | date = December 2019 | pmid = 31631001 | pmc = 6904245 | doi = 10.1016/j.cmet.2019.09.012 }}</ref><ref name="ketosis">{{cite journal | vauthors = Carney EF | title = Ketosis slows the progression of PKD | journal = Nature Reviews. Nephrology | volume = 16 | issue = 1 | pages = 1 | date = January 2020 | pmid = 31654043 | doi = 10.1038/s41581-019-0226-4 | s2cid = 204886698 | doi-access = free }}</ref>  A ketogenic diet regimen not only halted further disease progression but led to partial reversal of renal cystic disease in a rat model.<ref name="ketosis"/> The metabolic state of ketosis may be beneficial in ADPKD because renal cyst cells in ADPKD have a metabolic defect similar to the [[Warburg effect (oncology)|Warburg effect]] in cancer that makes them highly dependent on glucose, and unable to metabolize fatty acids and ketones.<ref name="rat model" /><ref>{{cite journal | vauthors = Nowak KL, Hopp K | title = Metabolic Reprogramming in Autosomal Dominant Polycystic Kidney Disease: Evidence and Therapeutic Potential | journal = Clinical Journal of the American Society of Nephrology | volume = 15 | issue = 4 | pages = 577–584 | date = April 2020 | pmid = 32086281 | pmc = 7133124 | doi = 10.2215/CJN.13291019 }}</ref><ref>{{cite journal | vauthors = Padovano V, Podrini C, Boletta A, Caplan MJ | title = Metabolism and mitochondria in polycystic kidney disease research and therapy | journal = Nature Reviews. Nephrology | volume = 14 | issue = 11 | pages = 678–687 | date = November 2018 | pmid = 30120380 | doi = 10.1038/s41581-018-0051-1 | s2cid = 52033674 }}</ref> Consistent with this, serum glucose levels positively correlate with faster disease progression in ADPKD patients.<ref>{{cite journal | vauthors = Torres JA, Kruger SL, Broderick C, Amarlkhagva T, Agrawal S, Dodam JR, Mrug M, Lyons LA, Weimbs T | display-authors = 6 | title = Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease | journal = Cell Metabolism | volume = 30 | issue = 6 | pages = 1007–1023.e5 | date = December 2019 | pmid = 31631001 | pmc = 6904245 | doi = 10.1016/j.cmet.2019.09.012 | ref = glucose levels }}</ref> Also, individuals with ADPKD and type 2 diabetes have significantly larger total kidney volume (TKV) than those with ADPKD alone,<ref>{{cite journal | vauthors = Reed B, Helal I, McFann K, Wang W, Yan XD, Schrier RW | title = The impact of type II diabetes mellitus in patients with autosomal dominant polycystic kidney disease | journal = Nephrology, Dialysis, Transplantation | volume = 27 | issue = 7 | pages = 2862–2865 | date = July 2012 | pmid = 22207329 | pmc = 3398061 | doi = 10.1093/ndt/gfr744 }}</ref> and overweight or obesity associate with faster progression in early-stage ADPKD.<ref>{{cite journal | vauthors = Nowak KL, You Z, Gitomer B, Brosnahan G, Torres VE, Chapman AB, Perrone RD, Steinman TI, Abebe KZ, Rahbari-Oskoui FF, Yu AS, Harris PC, Bae KT, Hogan M, Miskulin D, Chonchol M | display-authors = 6 | title = Overweight and Obesity Are Predictors of Progression in Early Autosomal Dominant Polycystic Kidney Disease | journal = Journal of the American Society of Nephrology | volume = 29 | issue = 2 | pages = 571–578 | date = February 2018 | pmid = 29118087 | pmc = 5791072 | doi = 10.1681/ASN.2017070819 }}</ref> A retrospective case series study showed that ADPKD disease symptoms - including pain, hypertension and renal function - improved among 131 patients who implemented ketogenic diets for an average duration of 6 months.<ref name = "Strubl_2021" />

Dietary intake of sodium is associated with worse renal function decline in ADPKD,<ref>{{cite journal | vauthors = Kramers BJ, Koorevaar IW, Drenth JP, de Fijter JW, Neto AG, Peters DJ, Vart P, Wetzels JF, Zietse R, Gansevoort RT, Meijer E | display-authors = 6 | title = Salt, but not protein intake, is associated with accelerated disease progression in autosomal dominant polycystic kidney disease | journal = Kidney International | volume = 98 | issue = 4 | pages = 989–998 | date = October 2020 | pmid = 32534051 | doi = 10.1016/j.kint.2020.04.053 | s2cid = 219637514 | doi-access = free | hdl = 2066/225147 | hdl-access = free }}</ref> and limiting sodium intake is generally recommended to patients. Dietary protein intake was not found to correlate with ADPKD progression.<ref>{{cite journal | vauthors = Kramers BJ, Koorevaar IW, Drenth JP, de Fijter JW, Neto AG, Peters DJ, Vart P, Wetzels JF, Zietse R, Gansevoort RT, Meijer E | display-authors = 6 | title = Salt, but not protein intake, is associated with accelerated disease progression in autosomal dominant polycystic kidney disease | journal = Kidney International | volume = 98 | issue = 4 | pages = 989–998 | date = October 2020 | pmid = 32534051 | doi = 10.1016/j.kint.2020.04.053 | s2cid = 219637514 | first8 = Priya | first6 = A.G. | first7 = D.J.M. | doi-access = free | hdl = 2066/225147 | hdl-access = free }}</ref>

Increased water intake is thought to be beneficial in ADPKD and is generally recommended.<ref name = "Torres_2019">{{cite journal | vauthors = Torres JA, Rezaei M, Broderick C, Lin L, Wang X, Hoppe B, Cowley BD, Savica V, Torres VE, Khan S, Holmes RP, Mrug M, Weimbs T | display-authors = 6 | title = Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease | journal = The Journal of Clinical Investigation | volume = 129 | issue = 10 | pages = 4506–4522 | date = July 2019 | pmid = 31361604 | pmc = 6763267 | doi = 10.1172/JCI128503 }}</ref><ref>{{cite journal | vauthors = Amro OW, Paulus JK, Noubary F, Perrone RD | title = Low-Osmolar Diet and Adjusted Water Intake for Vasopressin Reduction in Autosomal Dominant Polycystic Kidney Disease: A Pilot Randomized Controlled Trial | journal = American Journal of Kidney Diseases | volume = 68 | issue = 6 | pages = 882–891 | date = December 2016 | pmid = 27663039 | pmc = 5123924 | doi = 10.1053/j.ajkd.2016.07.023 }}</ref> The underlying beneficial mechanism of increased water intake may be related to effects on the vasopressin V2 receptor or may be due to the suppression of harmful micro-crystal formation in renal tubules by dilution of solutes such as calcium oxalate, calcium phosphate and uric acid.<ref name = "Torres_2019" /><ref>{{cite journal | vauthors = Allison SJ | title = Crystal deposition aids cystogenesis | journal = Nature Reviews. Nephrology | volume = 15 | issue = 12 | pages = 730 | date = December 2019 | pmid = 31551591 | doi = 10.1038/s41581-019-0215-7 | s2cid = 202733384 | doi-access = free }}</ref>

Dietary intake of oxalate or inorganic phosphate has been shown to accelerate PKD disease progression in several rodent models.<ref name = "Torres_2019" /> Low levels or urinary citrate – a natural antagonist of the formation of harmful crystals in kidney tubules – have been shown to associate with worse disease progression in ADPKD patients.<ref name = "Torres_2019" />

===Analgesic medication===
[[Chronic pain]] in patients with ADPKD is often refractory to conservative, noninvasive treatments, but [[Nonsteroidal anti-inflammatory drug|nonopioid analgesics]] and conservative interventions can be first used before [[opioid analgesic]]s are considered; if pain continues, then surgical interventions can target renal or hepatic cysts  to directly address the cause of pain, with surgical options including renal cyst decortication, renal [[denervation]], and [[nephrectomy]].<ref name="TP-150515-E02">{{cite journal | vauthors = Tellman MW, Bahler CD, Shumate AM, Bacallao RL, Sundaram CP | title = Management of pain in autosomal dominant polycystic kidney disease and anatomy of renal innervation | journal = The Journal of Urology | volume = 193 | issue = 5 | pages = 1470–1478 | date = May 2015 | pmid = 25534330 | doi = 10.1016/j.juro.2014.10.124 | hdl-access = free | hdl = 1805/7798 }}</ref>

===Renal cyst aspiration===
Aspiration with ethanol [[sclerotherapy]] can be performed for the treatment of symptomatic simple renal cysts, but can be impractical in advanced patients with multiple cysts.<ref name="TP-150515-E03">{{cite journal | vauthors = Mohsen T, Gomha MA | title = Treatment of symptomatic simple renal cysts by percutaneous aspiration and ethanol sclerotherapy | journal = BJU International | volume = 96 | issue = 9 | pages = 1369–1372 | date = December 2005 | pmid = 16287460 | doi = 10.1111/j.1464-410X.2005.05851.x | doi-access = free }}</ref> The procedure itself consists in the percutaneous insertion of a needle into the identified cyst, under [[ultrasound]] guidance, with subsequent draining the contained liquid; the sclerotherapy is used to avoid liquid reaccumulation that can occur in the cyst, which can result in symptom recurrence.<ref name="TP-150515-E03" /><ref name="TP-150515-E07">{{cite journal | vauthors = Okeke AA, Mitchelmore AE, Keeley FX, Timoney AG | title = A comparison of aspiration and sclerotherapy with laparoscopic de-roofing in the management of symptomatic simple renal cysts | journal = BJU International | volume = 92 | issue = 6 | pages = 610–613 | date = October 2003 | pmid = 14511045 | doi = 10.1046/j.1464-410x.2003.04417.x | doi-access = free }}</ref>

===Laparoscopic cyst decortication===
Laparoscopic cyst decortication (also referred to as marsupialization) consists in the removal of one or more kidney cysts through [[laparoscopic surgery]], during which cysts are punctured, and the outer wall of the larger cysts is excised with care not to incise the renal parenchyma.<ref name="TP-150515-E04">{{cite journal | vauthors = Brown JA, Torres VE, King BF, Segura JW | title = Laparoscopic marsupialization of symptomatic polycystic kidney disease | journal = The Journal of Urology | volume = 156 | issue = 1 | pages = 22–27 | date = July 1996 | pmid = 8648810 | doi = 10.1016/s0022-5347(01)65927-5 }}</ref><ref name="TP-150515-E05">{{cite journal | vauthors = McDougall EM | title = Approach to decortication of simple cysts and polycystic kidneys | journal = Journal of Endourology | volume = 14 | issue = 10 | pages = 821–827 | date = December 2000 | pmid = 11206615 | doi = 10.1089/end.2000.14.821 }}</ref> This procedure can be useful for pain relief in patients with ADPKD, and is usually indicated after earlier cyst aspiration has confirmed that the cyst to be decorticated is responsible for pain.<ref name="TP-150515-E05" />
Nonrandomised controlled trials conducted in the '90s showed that patients with symptomatic simple renal cysts who had recurrence of symptoms after initial response to simple aspiration could be safely submitted to cyst decortication, with a mean pain-free life between 17 and 24 months after surgery.<ref name="TP-150515-E04" /><ref name="TP-150515-E06">{{cite journal | vauthors = Consonni P, Nava L, Scattoni V, Bianchi A, Spaliviero M, Guazzoni G, Bellinzoni P, Bocciardi A, Rigatti P | display-authors = 6 | title = [Percutaneous echo-guided drainage and sclerotherapy of symptomatic renal cysts: critical comparison with laparoscopic treatment] | journal = Archivio Italiano di Urologia, Andrologia | volume = 68 | issue = 5 Suppl | pages = 27–30 | date = December 1996 | pmid = 9162369 }}</ref> Laparoscopic decortication presents a 5% recurrence rate of renal cysts compared to an 82% recurrence rate obtained with sclerotherapy.<ref name="TP-150515-E07" />

===Neurolysis===
A novel treatment of specifically the chronic pain experienced by many with ADPKD is [[Celiac plexus neurolysis]].<ref name="pmid28159317">{{cite journal | vauthors = Casteleijn NF, van Gastel MD, Blankestijn PJ, Drenth JP, de Jager RL, Leliveld AM, Stellema R, Wolff AP, Groen GJ, Gansevoort RT | display-authors = 6 | title = Novel treatment protocol for ameliorating refractory, chronic pain in patients with autosomal dominant polycystic kidney disease | journal = Kidney International | volume = 91 | issue = 4 | pages = 972–981 | date = April 2017 | pmid = 28159317 | doi = 10.1016/j.kint.2016.12.007 | doi-access = free }}</ref><ref>{{cite journal | vauthors = Nitschke AM, Ray CE | title = Percutaneous neurolytic celiac plexus block | journal = Seminars in Interventional Radiology | volume = 30 | issue = 3 | pages = 318–321 | date = September 2013 | pmid = 24436554 | pmc = 3773031 | doi = 10.1055/s-0033-1353485 }}</ref>  This involves the chemical ablation of the [[celiac plexus]], to cause a temporary degeneration of targeted nerve fibers. When the nerve fibers degenerate, it causes an interruption in the transmission of nerve signals.  This treatment, when successful, provides significant pain relief for a period ranging from a few days to over a year.  The procedure may be repeated when the affected nerves have healed and the pain returns.<ref>{{cite journal |title=Response to repeat echoendoscopic celiac plexus neurolysis in pancreatic cancer patients: A machine learning approach |url=https://pubmed.ncbi.nlm.nih.gov/31375433/ |journal=Pancreatology |year=2019 |pmid=31375433 |access-date=5 January 2023|last1=Facciorusso |first1=A. |last2=Del Prete |first2=V. |last3=Antonino |first3=M. |last4=Buccino |first4=V. R. |last5=Muscatiello |first5=N. |volume=19 |issue=6 |pages=866–872 |doi=10.1016/j.pan.2019.07.038 |s2cid=199389236 }}</ref>

===Nephrectomy===
Many ADPKD patients experience symptomatic sequelae in consequence of the disease, such as cyst [[hemorrhage]], [[flank pain]], recurrent [[infection]]s, [[nephrolithiasis]], and symptoms of mass effect (i.e., early [[satiety]], [[nausea]] and vomiting, and abdominal discomfort), from their enlarged kidneys.<ref name="TP-150518-E08">{{cite journal | vauthors = Alam A, Perrone RD | title = Management of ESRD in patients with autosomal dominant polycystic kidney disease | journal = Advances in Chronic Kidney Disease | volume = 17 | issue = 2 | pages = 164–172 | date = March 2010 | pmid = 20219619 | doi = 10.1053/j.ackd.2009.12.006 }}</ref><ref name="TP-150518-E09">{{cite journal | vauthors = Wagner MD, Prather JC, Barry JM | title = Selective, concurrent bilateral nephrectomies at renal transplantation for autosomal dominant polycystic kidney disease | journal = The Journal of Urology | volume = 177 | issue = 6 | pages = 2250–4; discussion 2254 | date = June 2007 | pmid = 17509331 | doi = 10.1016/j.juro.2007.01.146 }}</ref><ref name="TP-150518-E13">{{cite journal | vauthors = Cristea O, Yanko D, Felbel S, House A, Sener A, Luke PP | title = Maximal kidney length predicts need for native nephrectomy in ADPKD patients undergoing renal transplantation | journal = Canadian Urological Association Journal | volume = 8 | issue = 7–8 | pages = 278–282 | date = July 2014 | pmid = 25210553 | pmc = 4137014 | doi = 10.5489/cuaj.2128 }}</ref> In such cases, [[nephrectomy]] can be required due to intractable symptoms or when in the course of preparing for [[kidney transplantation]], the native kidneys are found to impinge upon the [[true pelvis]] and preclude the placement of a donor [[allograft]].<ref name="TP-150518-E09" /><ref name="TP-150518-E13" /><ref name="TP-150518-E10">{{cite journal | vauthors = Fuller TF, Brennan TV, Feng S, Kang SM, Stock PG, Freise CE | title = End stage polycystic kidney disease: indications and timing of native nephrectomy relative to kidney transplantation | journal = The Journal of Urology | volume = 174 | issue = 6 | pages = 2284–2288 | date = December 2005 | pmid = 16280813 | doi = 10.1097/01.ju.0000181208.06507.aa | s2cid = 25363382 }}</ref><ref name="TP-150518-E11">{{cite journal | vauthors = Cohen D, Timsit MO, Chrétien Y, Thiounn N, Vassiliu V, Mamzer MF, Legendre C, Méjean A | display-authors = 6 | title = [Place of nephrectomy in patients with autosomal dominant polycystic kidney disease waiting for renal transplantation] | journal = Progres en Urologie | volume = 18 | issue = 10 | pages = 642–649 | date = November 2008 | pmid = 18971106 | doi = 10.1016/j.purol.2008.06.004 }}</ref> Additionally, native nephrectomy may be undertaken in the presence of suspected malignancy, as renal cell carcinoma (RCC) is two to three times more likely in the ADPKD population in [[Kidney failure|end-stage kidney disease]] (ESKD) than in the ESKD patients without ADPKD.<ref name="TP-150518-E13" /><ref name="TP-150518-E12">{{cite journal | vauthors = Hajj P, Ferlicot S, Massoud W, Awad A, Hammoudi Y, Charpentier B, Durrbach A, Droupy S, Benoît G | display-authors = 6 | title = Prevalence of renal cell carcinoma in patients with autosomal dominant polycystic kidney disease and chronic renal failure | journal = Urology | volume = 74 | issue = 3 | pages = 631–634 | date = September 2009 | pmid = 19616833 | doi = 10.1016/j.urology.2009.02.078 }}</ref> Although the indications for nephrectomy in ADPKD may be related to kidney size, the decision to proceed with native nephrectomy is often undertaken on an individual basis, without specific reference to kidney size measurements.<ref name="TP-150518-E13" />

===Dialysis===
Two modalities of [[Kidney dialysis|dialysis]] can be used in the treatment of ADPKD patients: [[peritoneal dialysis]] and [[hemodialysis]].<ref name="TP-150518-E14">{{cite journal | vauthors = Courivaud C, Roubiou C, Delabrousse E, Bresson-Vautrin C, Chalopin JM, Ducloux D | title = Polycystic kidney size and outcomes on peritoneal dialysis: comparison with haemodialysis | journal = Clinical Kidney Journal | volume = 7 | issue = 2 | pages = 138–143 | date = April 2014 | pmid = 25852862 | pmc = 4377775 | doi = 10.1093/ckj/sft171 }}</ref> Epidemiological data shows that ADPKD affects 5–13.4% of patients undergoing hemodialysis in Europe and in the United States,<ref name="TP-150518-E15">{{cite journal | vauthors = Nunes AC, Milani V, Porsch DB, Rossato LB, Mattos CB, Roisenberg I, Barros EJ | title = Frequency and clinical profile of patients with polycystic kidney disease in southern Brazil | journal = Renal Failure | volume = 30 | issue = 2 | pages = 169–173 | year = 2008 | pmid = 18300116 | doi = 10.1080/08860220701810265 | doi-access = free }}</ref><ref name="TP-150518-E16">{{cite journal | vauthors = Bleyer AJ, Hart TC | title = Polycystic kidney disease | journal = The New England Journal of Medicine | volume = 350 | issue = 25 | pages = 2622; author reply 2622 | date = June 2004 | pmid = 15201424 | doi = 10.1056/NEJM200406173502519 }}</ref><ref name="TP-150518-E17">{{cite journal | vauthors = Corradi V, Gastaldon F, Virzì GM, de Cal M, Soni S, Chionh C, Cruz DN, Clementi M, Ronco C | display-authors = 6 | title = Clinical pattern of adult polycystic kidney disease in a northeastern region of Italy | journal = Clinical Nephrology | volume = 72 | issue = 4 | pages = 259–267 | date = October 2009 | pmid = 19825331 | doi = 10.5414/CNP72259 }}</ref> and about 3% in Japan.<ref name="TP-150518-E18"/> Peritoneal dialysis has usually been contra-indicated in ADPKD patients with large kidney and liver volumes, due to expected physical difficulties in the procedure and possible complications;<ref name="TP-150518-E14" /><ref name="TP-150518-E19">{{cite journal | vauthors = Hamanoue S, Hoshino J, Suwabe T, Marui Y, Ueno T, Kikuchi K, Hazue R, Mise K, Kawada M, Imafuku A, Hayami N, Sumida K, Hiramatsu R, Hasegawa E, Sawa N, Takaichi K, Ubara Y | display-authors = 6 | title = Peritoneal Dialysis is Limited by Kidney and Liver Volume in Autosomal Dominant Polycystic Kidney Disease | journal = Therapeutic Apheresis and Dialysis | volume = 19 | issue = 3 | pages = 207–211 | date = June 2015 | pmid = 25612237 | doi = 10.1111/1744-9987.12272 | s2cid = 27836789 }}</ref> however, no difference is seen in long-term morbidity between hemodialysis and peritoneal dialysis in ADPKD.<ref name="TP-150518-E14" />

===Kidney transplant===
Kidney transplantation is accepted as the preferred treatment for ADPKD patients with ESRD.<ref name="TP-150518-E20" /> Among American patients on the kidney-transplant waiting list (as of December 2011), 7256 (8.4%) were listed due to cystic kidney disease and of the 16,055 renal transplants performed in 2011, 2057 (12.8%) were done for patients with cystic kidney disease, with 1,189 from deceased donors and 868 from living donors.<ref name="TP-150518-E21">{{cite journal | vauthors = Matas AJ, Smith JM, Skeans MA, Lamb KE, Gustafson SK, Samana CJ, Stewart DE, Snyder JJ, Israni AK, Kasiske BL | display-authors = 6 | title = OPTN/SRTR 2011 Annual Data Report: kidney | journal = American Journal of Transplantation | volume = 13 | issue = Suppl 1 | pages = 11–46 | date = January 2013 | pmid = 23237695 | pmc = 5527691 | doi = 10.1111/ajt.12019 }}</ref>

=== Novel Therapies ===
There are several novel therapies currently underway aimed at slowing the progression of disease in APKD. Alternative therapeutic options water therapy, use of lipid lowering agents, antiproliferative analogues and synthetic peptides.<ref name=":2" />

Water Therapy

Increased water intake downregulates vasopressin activity As a result water therapy has been explored as a potential therapeutic intervention for individuals with ADPKD, however studies have examining its role in ADPKD advancement remain unclear.<ref name=":2" />

HMG-CoA reductase inhibitors

The progression of ADPKD leads to decline in kidney function, with a marked decreased in glomerular filtration rate. As a result, treatment with statins is recommended in accordance with current guidelines for managing chronic kidney disease. The effect of statins on slowing ADPKD are inconclusive, with some trials showing a decrease in total kidney volume, while others showed no benefit on either total kidney volume or glomerular filtration rate.<ref name=":2" />

Somatostatin analogs

Somatostatin are artificial peptides designed to mimic the function of endogenous hormone that has many regulatory functions within the body including restraining cell proliferation. Small phase 2 studies have shown that somatostatin analogues are effective at reducing the rate of total kidney volume growth and preserving glomerular filtration rate.<ref name=":2" /> The use of somatostatin analogues may be restricted due to their side effects, which commonly include gastrointestinal issues such as diarrhea, abdominal discomfort, and gas, as well as conditions like gallstones and elevated blood sugar. 

Anti-proliferative agents

Proliferation of epithelial cells lining cyst resulting in cyst expansion and contributing to disease progression. As a result of this mechanism, antiproliferative agents such as tyrosine kinase inhibitor bosutinib. However, results in clinical trails have shown mixed results, with studies showing a decrease in total kidney volume. <ref name=":2" /><ref>{{Cite journal |last=Zhou |first=Julie Xia |last2=Torres |first2=Vicente E. |date=May 2023 |title=Autosomal Dominant Polycystic Kidney Disease Therapies on the Horizon |url=https://linkinghub.elsevier.com/retrieve/pii/S2949813923000034 |journal=Advances in Kidney Disease and Health |language=en |volume=30 |issue=3 |pages=245–260 |doi=10.1053/j.akdh.2023.01.003}}</ref>