Medscape is available in 5 Language Editions – Choose your Edition here.


Polycystic Kidney Disease

  • Author: Roser Torra, MD, PhD; Chief Editor: Vecihi Batuman, MD, FACP, FASN  more...
Updated: Feb 22, 2016

Practice Essentials

Autosomal dominant polycystic kidney disease (ADPKD) is a multisystemic and progressive disorder characterized by cyst formation and enlargement in the kidney (see the image below) and other organs (eg, liver, pancreas, spleen). Up to 50% of patients with ADPKD require renal replacement therapy by 60 years of age.

Polycystic kidney. Polycystic kidney.

Signs and symptoms

Pain—in the abdomen, flank, or back—is the most common initial complaint, and it is almost universally present in patients with ADPKD. Dull aching and an uncomfortable sensation of heaviness may result from a large polycystic liver.

The pain can be caused by any of the following:

  • Enlargement of one or more cysts
  • Bleeding: May be confined inside the cyst or lead to gross hematuria with passage of clots or a perinephric hematoma
  • UTI (eg, acute pyelonephritis, infected cysts, perinephric abscess)
  • Nephrolithiasis and renal colic
  • Rarely, a coincidental hypernephroma

See Presentation for more detail.


Examination in patients with ADPKD may demonstrate the following:

  • Hypertension: One of the most common early manifestations of ADPKD, [1, 2] in which increased diastolic BP is the rule; clinical course in ADPKD is usually more severe early on, then becomes less problematic as the renal insufficiency progresses
  • Palpable, bilateral flank masses: In advanced ADPKD
  • Nodular hepatomegaly: In severe polycystic liver disease
  • Rarely, symptoms related to renal failure (eg, pallor, uremic fetor, dry skin, edema)


Routine laboratory studies include the following:

  • Serum chemistry profile, including calcium and phosphorus
  • CBC count from cysts
  • Urinalysis
  • Urine culture
  • Uric acid determination
  • Intact PTH assay

Genetic testing may be performed, in which the major indication is for genetic screening in young adults with negative ultrasonographic findings who are being considered as potential kidney donors.[3]


Staging of renal failure is by GFR, as follows:

  • Stage 1: GFR above 90 mL/min
  • Stage 2: GFR 60-90 mL/min
  • Stage 3: GFR 30-60 mL/min
  • Stage 4: GFR 15-30 mL/min
  • Stage 5: GFR below 15 mL/min

Imaging studies

Radiologic studies used in the evaluation of ADPKD include the following:

  • Ultrasonography: Technique of choice for patients with ADPKD and for screening patients' family members; useful for exploring abdominal extrarenal features of ADPKD (eg, liver cysts, pancreatic cysts)
  • CT scanning: Not routine; useful in doubtful pediatric cases or in complicated cases (eg, kidney stone, suspected tumor)
  • MRI: Not routine; helpful in distinguishing renal cell carcinoma from simple cysts; criterion standard to help determine renal volume for clinical trials when testing drugs for ADPKD; best imaging tool to monitor kidney size after treatment to assess progress
  • MRA: Not routine; preferred imaging technique for diagnosing intracranial aneurysms

Ultrasonographic diagnostic criteria for ADPKD1 are as follows[4] :

  • At least 2 cysts in 1 kidney or 1 cyst in each kidney in an at-risk patient younger than 30 years
  • At least 2 cysts in each kidney in an at-risk patient aged 30-59 years
  • At least 4 cysts in each kidney for an at-risk patient aged 60 years or older

Ultrasonographic diagnostic criteria for ADPKD in patients with a family history but unknown genotype are as follows[5] :

  • Three or more (unilateral or bilateral) renal cysts in patients aged 15-39 years
  • Two or more cysts in each kidney in patients aged 30-59 years

Fewer than 2 renal cysts in the findings provides a negative predictive value of 100% and can be considered sufficient for ruling out disease in at-risk individuals older than 40 years.

Indications for MRA are as follows[6, 7] :

  • Family history of stroke or intracranial aneurysms
  • Development of symptoms suggesting an intracranial aneurysm
  • Job or hobby in which a loss of consciousness may be lethal
  • Past history of intracranial aneurysms

See Workup for more detail.


No specific medication is available for ADPKD. However, pharmacotherapy is necessary to accomplish the following:

  • Control blood pressure: Drugs of choice are ACEIs (eg, captopril, enalapril, lisinopril) or ARBs (eg, valsartan, telmisartan, losartan, irbesartan, candesartan, olmesartan)
  • Control abnormalities related to renal failure: Drugs to maintain electrolyte levels (eg, calcium carbonate, calcium acetate, sevelamer, lanthanum carbonate, calcitriol [possibly], diuretics, blood pressure medications)
  • Treat urinary tract infections
  • Treat cyst infections: Gyrase inhibitors (eg, ciprofloxacin, chloramphenicol, clindamycin, levofloxacin); dihydrofolic acid inhibitors (TMX/SMP)
  • Treat hematuria: Possibly analgesic plus copious oral hydration
  • Reduce abdominal pain produced by enlarged kidneys
  • Prevent cardiac valve infection in patients with intrinsic valve disease

Surgical option

Surgical intervention in ADPKD includes the following:

  • Surgical drainage: Usually in conjunction with ultrasonographically guided puncture; in cases of infected renal/hepatic cysts not responding to conventional antibiotics
  • Open-/fiberoptic-guided surgery: For excision/drainage of the outer walls of cysts to ablate symptoms
  • Nephrectomy: Last resort for pain control in patients with inaccessible cysts in the renal medullae; bilateral nephrectomy in patients with severe hepatic involvement
  • Partial hepatectomy: To manage massive hepatomegaly
  • Liver transplantation: In cases of portal hypertension due to polycystic liver or hepatomegaly with nonresectable areas

Patients with ADPKD who progress to end-stage renal disease may require the following procedures:

  • Hemodialysis
  • Peritoneal dialysis
  • Renal transplantation

See Treatment and Medication for more detail.



Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common inherited disorders in humans. It is the most frequent genetic cause of renal failure in adults, accounting for 6-8% of patients on dialysis in the United States.

ADPKD is a multisystemic and progressive disorder characterized by the formation and enlargement of cysts in the kidney (as seen in the image below) and other organs (eg, liver, pancreas, spleen). Clinical features usually begin in the third to fourth decade of life, but cysts may be detectable in childhood and in utero.[8]

Polycystic kidney. Polycystic kidney.

Pain—in the abdomen, flank, or back—is the most common initial complaint, and it is almost universally present in patients with ADPKD (see Presentation). Ultrasonography is the diagnostic procedure of choice (see Workup). Medical therapy is needed to control problems such as hypertension, urinary tract infections, hematuria, and pain. Surgical drainage of cysts may be indicated. Patients who progress to end-stage renal disease (ESRD) may require dialysis or renal transplantation (see Treatment).

For a discussion of ADPKD in children, see Pediatric Polycystic Kidney Disease.



The main feature of ADPKD is a bilateral progressive increase in the number of cysts, which may lead to ESRD. Hepatic cysts, cerebral aneurysms, and cardiac valvular abnormalities also may occur.[9, 10]

Although ADPKD is a systemic disease, it shows a focal expression because less than 1% of nephrons become cystic. In ADPKD, each epithelial cell within a renal tubule harbors a germ-line mutation, yet only a tiny fraction of the tubules develop renal cysts.

It is currently held that the cells are protected by the allele inherited from the parent without ADPKD. When this allele is inactivated by a somatic event (mutation or otherwise) within a solitary renal tubule cell, the cell divides repeatedly until a cyst develops, with an aberrant growth program causing endless expansion. The severity of ADPKD is thought to be a direct consequence of the number of times and the frequency with which this cystogenic process occurs within the kidneys over the life of the patient. However this hypothesis is hard to understand in neonatal cases.

The hyperplastic cells cause an out-pocketing of the tubule wall, with the formation of a saccular cyst that fills with fluid derived from glomerular filtrate that enters from the afferent tubule segment. Progressive expansion eventually causes most of the emerging cysts to separate from the parent tubule, leaving an isolated sac that fills with fluid by transepithelial secretion. This isolated cyst expands relentlessly as a result of continued proliferation of the mural epithelium together with the transepithelial secretion of sodium chloride and water into the lumen.

The expanding fluid-filled tumor masses elicit secondary and tertiary changes within the renal interstitium evinced by thickening and lamination of the tubule basement membranes, infiltration of macrophages, and neovascularization. Fibrosis within the interstitium begins early in the course of the disease.

Cellular proliferation and fluid secretion may be accelerated by cyclic adenosine monophosphate (cAMP) and growth factors, such as epidermal growth factor (EGF). In summary, cysts function as autonomous structures and are responsible for progressive kidney enlargement in ADPKD.

Approximately 85-90% of patients with ADPKD have an abnormality on the short arm of chromosome 16 (ie, ADPKD type 1 [ADPKD1]). A second defect, termed ADPKD type 2 (ADPKD2), is responsible for 10-15% of ADPKD cases and is found on the long arm of chromosome 4. A third genotype may exist, but no genomic locus is assigned.

PKD1 and PKD2 are expressed in most organs and tissues of the human body. The proteins that are encoded by PKD1 and PKD2, polycystin 1 and polycystin 2, seem to function together to regulate the morphologic configuration of epithelial cells. The polycystins are expressed in development as early as the blastocyst stage and are expressed in a broad array of terminally differentiated tissues. The functions of the polycystins have been scrutinized to the greatest extent in epithelial tissues of the kidneys and liver and in vascular smooth muscle (see Etiology).

A decrease in urine-concentrating ability is an early manifestation of ADPKD. The cause is not known. Plasma vasopressin levels are increased; this increase may represent the body's attempt to compensate for the reduced concentrating capacity of the kidneys and could contribute to the development of renal cysts, hypertension, and renal insufficiency.[11]


Renal cysts in ADPKD are associated with excessive angiogenesis evinced by fragile vessels stretched across their distended walls. When traumatized, these vessels may leak blood into the cyst, causing it to expand rapidly, resulting in excruciating pain. If bleeding continues, then the cyst may rupture into the collecting system, causing gross hematuria. Alternatively, the cyst may rupture into the subcapsular compartment and eventually dissect through the renal capsule to fill the retroperitoneal space.



ADPKD is a hereditary disorder. The pattern of inheritance is autosomal dominant. Because the disorder occurs equally in males and females, each offspring has a 50% chance of inheriting the responsible mutation and, hence, the disease.

ADPKD is a genetically heterogeneous condition that involves at least 2 genes. PKD1 is located on 16p13.3 and accounts for most ADPKD cases. PKD2 is located on 4q21-q22 and accounts for 15% of ADPKD cases.

Polycystin 1 and 2

PKD1 codes for a 4304–amino acid protein (polycystin 1). The function of polycystin 1 is not yet fully defined, but this protein interacts with polycystin 2 and is involved in cell cycle regulation and intracellular calcium transport. Polycystin 1 localizes in the primary cilia of renal epithelial cells, which function as mechanosensors and chemosensors.

PKD2 codes for a 968–amino acid protein (polycystin 2) that is structurally similar to polycystin 1 and co-localizes to the primary cilia of renal epithelial cells. It is a member of the family of voltage-activated calcium channels.

Polycystin 1 and polycystin 2 are highly conserved ubiquitous transmembrane proteins. In the kidney, they are located in the epithelial cells of the renal tubules—in particular, in the primary cilia at the luminal side of the tubules, as well as in other areas of the renal cell epithelium.

Polycystin 1 is a large protein with a long extracellular N-terminal region, 11 transmembrane domains, and a short intracellular C-terminal tail. Polycystin 2 is structurally related to the transient receptor potential (TRP) channel family, and it is known to function as a nonselective cation channel permeable to Ca2+.

Polycystin 1 and polycystin 2 form heteromeric complexes and colocalize in the primary cilium of renal epithelial cells. The primary cilium is a long, nonmotile tubular structure located in the apical surface of the epithelial cells in the renal tubules. Its function was unknown for a long time. However, studies now indicate that the primary cilium may be a mechanoreceptor that senses changes in apical fluid flow and that transduces them into an intracellular Ca2+ signaling response.

This model involves the participation of polycystin 1 as a mechanical sensor of ciliary bending induced by luminal fluid flow. Bending of the cilium would cause a conformational change in polycystin 1 that would, in turn, activate the polycystin 2–associated Ca2+ channel, increasing the intracellular Ca2+ concentration and triggering intracellular signaling pathways leading to normal kidney development.[12]

A good genotype-phenotype correlation has not been well established for ADPKD1 and ADPKD2.[13]

ADPKD1 is more severe than ADPKD2. The mean age of ESRD for patients with ADPKD1 is 53 years. The mean age of ESRD for patients with ADPKD2 is 74 years.

The genetic heterogeneity of ADKPD, and the possible contribution of modifier genes, may explain the wide clinical variability in this disease, both within and between families.[14]



Worldwide, ADPKD affects approximately 4 to 7 million individuals and accounts for 7-15% of patients on renal replacement therapy.[15] In North America and Europe, ADPKD is responsible for 6-10% of ESRD cases. Approximately one per 800-1000 population carries a mutation for this condition. Approximately 85-90% of patients with ADPKD have ADPKD1; most of the remaining patients have ADPKD2.[16]

ADPKD is slightly more severe in males than in females, but the difference is not statistically significant.

Symptoms generally increase with age. Children very rarely present with renal failure from ADPKD.



The prognosis in patients with ADPKD covers a wide spectrum. Renal failure has been reported in children; conversely, individuals with ADPKD may live a normal lifespan without knowing that they have the disease. More typically, however, ADPKD causes progressive renal dysfunction, resulting in grossly enlarged kidneys and kidney failure by the fourth to sixth decade of life. There is an inverse association between the size of polycystic kidneys and the level of glomerular filtration.[17, 18]

An early study estimated that approximately 70% of patients with ADPKD would develop renal insufficiency if they survived to age 65 years. Currently, half of all patients with ADPKD require renal replacement therapy by age 60 years. Risk factors for progression include the following:

  • PKD1 genotype
  • Large kidneys
  • Several episodes of gross hematuria [19]
  • Severe and frequent kidney infections
  • Hypertension
  • Multiple pregnancies
  • Black racial background [16]
  • Male sex

The presence of more than one risk factor increases the risk of progression to ESRD.

The 2 forms of ADPKD are ADPKD1 and ADPKD2.[20] Although they share similar clinical features, renal prognosis is strikingly different.[21] ADPKD2 is a milder disease, based on the age of onset of ESRD. The median age of renal survival for individuals with ADPKD2 is 68 years, which is significantly older than for those with ADPKD1, in whom the median age of renal survival is 53 years. Although ADPKD2 is milder than ADPKD1, it has an overall impact on survival and shortens life expectancy.

A study of 180 patients with ADPK by Idrizi et al found that recurrent episodes of gross hematuria may increase the risk for more severe renal disease.[19] In the 43 study patients who experienced at least one episode of gross hematuria before age 30 years, renal survival was worse than in the other patients with ADPKD (a 10-year difference in survival). Although 60% of the study patients experienced urinary tract infections (UTIs), appropriate treatment of UTI decreased its frequency and slowed the rate of progression to renal failure.

Cardiovascular pathology and infections account for approximately 90% of deaths of those patients treated by hemodialysis or peritoneal dialysis and after renal transplantation.

Another cause of mortality is in ADPKD is subarachnoid hemorrhage from intracranial aneurysms.[22] This complication is rare and severe.

In a retrospective, observational study of 88 patients with ADPKD who died between 1981 and 1999, Rahman et al determined that almost half of the patients died of cardiovascular problems.[23] The median age of death was 60.5 years.

Causes of death included the following:

  • Cardiovascular problems - 46.6% of patients
  • Infection - 15.9% of patients, with 42% of these deaths resulting from septicemia
  • Central nervous system disorders - 11.36% of patients, with 60% of these deaths caused by cerebrovascular events
  • Uremia - 2.2% of patients
  • Other, miscellaneous causes - 11.36%

Patient Education

Ensure that patients are aware that this disease is hereditary and that their children have a 50% chance of acquiring the disease. Patients should also understand that although several treatments are being tested, this disease currently has no cure. Only interventions that slow the progression of renal disease (eg, adequate blood pressure control) are of benefit. Hopefully, effective specific therapy will be available in a few years.

Prenatal diagnosis is available through DNA linkage studies, if enough family members cooperate, or through a mutation search. Suggest that family members who are not screened for ADPKD have annual blood pressure checks and urine screenings for hematuria.

Contributor Information and Disclosures

Roser Torra, MD, PhD Consulting Staff, Hereditary Renal Diseases, Department of Nephrology, Fundacio Puigvert, Spain

Roser Torra, MD, PhD is a member of the following medical societies: American Society of Nephrology, International Society of Nephrology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

George R Aronoff, MD Director, Professor, Departments of Internal Medicine and Pharmacology, Section of Nephrology, Kidney Disease Program, University of Louisville School of Medicine

George R Aronoff, MD is a member of the following medical societies: American Federation for Medical Research, American Society of Nephrology, Kentucky Medical Association, National Kidney Foundation

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FACP, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Renal Section, Southeast Louisiana Veterans Health Care System

Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, International Society of Nephrology

Disclosure: Nothing to disclose.

Additional Contributors

Laura Lyngby Mulloy, DO, FACP Professor of Medicine, Chief, Section of Nephrology, Hypertension, and Transplantation Medicine, Glover/Mealing Eminent Scholar Chair in Immunology, Medical College of Georgia, Georgia Regents University

Disclosure: Nothing to disclose.

  1. Schrier RW. Renal volume, renin-angiotensin-aldosterone system, hypertension, and left ventricular hypertrophy in patients with autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2009 Sep. 20(9):1888-93. [Medline].

  2. Cadnapaphornchai MA, McFann K, Strain JD, et al. Prospective change in renal volume and function in children with ADPKD. Clin J Am Soc Nephrol. 2009 Apr. 4(4):820-9. [Medline]. [Full Text].

  3. Pei Y. Diagnostic approach in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2006 Sep. 1(5):1108-14. [Medline].

  4. Ravine D, Gibson RN, Walker RG, et al. Evaluation of ultrasonographic diagnostic criteria for autosomal dominant polycystic kidney disease 1. Lancet. 1994 Apr 2. 343(8901):824-7. [Medline].

  5. Pei Y, Obaji J, Dupuis A, Paterson AD, Magistroni R, Dicks E, et al. Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2009 Jan. 20(1):205-12. [Medline]. [Full Text].

  6. Huston J 3rd, Torres VE, Wiebers DO, et al. Follow-up of intracranial aneurysms in autosomal dominant polycystic kidney disease by magnetic resonance angiography. J Am Soc Nephrol. 1996 Oct. 7(10):2135-41. [Medline].

  7. Irazabal MV, Huston J 3rd, Kubly V, Rossetti S, Sundsbak JL, Hogan MC, et al. Extended follow-up of unruptured intracranial aneurysms detected by presymptomatic screening in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2011 Jun. 6(6):1274-85. [Medline]. [Full Text].

  8. Wilson PD. Polycystic kidney disease. N Engl J Med. 2004 Jan 8. 350(2):151-64. [Medline].

  9. Pirson Y. Extrarenal Manifestations of Autosomal Dominant Polycystic Kidney Disease. Adv Chronic Kidney Dis. 2010 Mar. 17(2):173-180. [Medline].

  10. Tufan F, Uslu B, Cekrezi B, Uysal M, Alpay N, Turkmen K, et al. Assessment of Adrenal Functions in Patients with Autosomal Dominant Polycystic Kidney Disease. Exp Clin Endocrinol Diabetes. 2010 Feb 9. [Medline].

  11. Torres VE. Vasopressin antagonists in polycystic kidney disease. Kidney Int. 2005 Nov. 68(5):2405-18. [Medline]. [Full Text].

  12. Ong AC, Wheatley DN. Polycystic kidney disease--the ciliary connection. Lancet. 2003 Mar 1. 361(9359):774-6. [Medline].

  13. Rossetti S, Harris PC. Genotype-phenotype correlations in autosomal dominant and autosomal recessive polycystic kidney disease. J Am Soc Nephrol. 2007 May. 18(5):1374-80. [Medline].

  14. Qian F, Watnick TJ, Onuchic LF, et al. The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I. Cell. 1996 Dec 13. 87(6):979-87. [Medline].

  15. Akoh JA. Current management of autosomal dominant polycystic kidney disease. World J Nephrol. 2015 Sep 6. 4 (4):468-79. [Medline].

  16. Fary Ka E, Seck SM, Niang A, et al. Patterns of autosomal dominant polycystic kidney diseases in black Africans. Saudi J Kidney Dis Transpl. 2010 Jan. 21(1):81-6. [Medline].

  17. Grantham JJ, Chapman AB, Torres VE. Volume progression in autosomal dominant polycystic kidney disease: the major factor determining clinical outcomes. Clin J Am Soc Nephrol. 2006 Jan. 1(1):148-57. [Medline].

  18. Grantham JJ, Torres VE, Chapman AB, et al. Volume progression in polycystic kidney disease. N Engl J Med. 2006 May 18. 354(20):2122-30. [Medline]. [Full Text].

  19. Idrizi A, Barbullushi M, Petrela E, et al. The influence of renal manifestations to the progression of autosomal dominant polycystic kidney disease. Hippokratia. 2009 Jul. 13(3):161-4. [Medline]. [Full Text].

  20. Hateboer N, v Dijk MA, Bogdanova N, et al. Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group. Lancet. 1999 Jan 9. 353(9147):103-7. [Medline].

  21. Torra R, Badenas C, Darnell A, et al. Linkage, clinical features, and prognosis of autosomal dominant polycystic kidney disease types 1 and 2. J Am Soc Nephrol. 1996 Oct. 7(10):2142-51. [Medline].

  22. Chauveau D, Pirson Y, Verellen-Dumoulin C, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. Kidney Int. 1994 Apr. 45(4):1140-6. [Medline].

  23. Rahman E, Niaz FA, Al-Suwaida A, et al. Analysis of causes of mortality in patients with autosomal dominant polycystic kidney disease: A single center study. Saudi J Kidney Dis Transpl. 2009 Sep-Oct. 20(5):806-10. [Medline].

  24. Baker A, King D, Marsh J, Makin A, Carr A, Davis C, et al. Understanding the physical and emotional impact of early-stage ADPKD: experiences and perspectives of patients and physicians. Clin Kidney J. 2015 Oct. 8 (5):531-7. [Medline]. [Full Text].

  25. Kistler AD, Serra AL, Siwy J, Poster D, Krauer F, Torres VE, et al. Urinary proteomic biomarkers for diagnosis and risk stratification of autosomal dominant polycystic kidney disease: a multicentric study. PLoS One. 2013. 8(1):e53016. [Medline]. [Full Text].

  26. Srivastava A, Patel N. Autosomal dominant polycystic kidney disease. Am Fam Physician. 2014 Sep 1. 90 (5):303-7. [Medline]. [Full Text].

  27. Perrone RD, Malek AM, Watnick T. Vascular complications in autosomal dominant polycystic kidney disease. Nat Rev Nephrol. 2015 Oct. 11 (10):589-98. [Medline].

  28. Sawicki M, Walecka A, Rozanski J, et al. Doppler sonography measurements of renal vascular resistance in autosomal-dominant polycystic kidney disease. Med Sci Monit. 2009 Aug. 15(8):MT101-4. [Medline].

  29. Spithoven EM, van Gastel MD, Messchendorp AL, Casteleijn NF, Drenth JP, Gaillard CA, et al. Estimation of total kidney volume in autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2015 Nov. 66 (5):792-801. [Medline].

  30. Torres VE, Grantham JJ, Chapman AB, Mrug M, Bae KT, King BF Jr, et al. Potentially Modifiable Factors Affecting the Progression of Autosomal Dominant Polycystic Kidney Disease. Clin J Am Soc Nephrol. 2011 Mar. 6(3):640-647. [Medline].

  31. Masoumi A, Reed-Gitomer B, Kelleher C, et al. Potential pharmacological interventions in polycystic kidney disease. Drugs. 2007. 67(17):2495-510. [Medline].

  32. Walz G. Therapeutic approaches in autosomal dominant polycystic kidney disease (ADPKD): is there light at the end of the tunnel?. Nephrol Dial Transplant. 2006 Jul. 21(7):1752-7. [Medline].

  33. Bolignano D, Palmer SC, Ruospo M, Zoccali C, Craig JC, Strippoli GF. Interventions for preventing the progression of autosomal dominant polycystic kidney disease. Cochrane Database Syst Rev. 2015 Jul 14. 7:CD010294. [Medline].

  34. Torres VE, Harris PC. Mechanisms of Disease: autosomal dominant and recessive polycystic kidney diseases. Nat Clin Pract Nephrol. 2006 Jan. 2(1):40-55; quiz 55. [Medline]. [Full Text].

  35. Torres VE, Harris PC. Polycystic kidney disease: genes, proteins, animal models, disease mechanisms and therapeutic opportunities. J Intern Med. 2007 Jan. 261(1):17-31. [Medline].

  36. Rozenfeld MN, Ansari SA, Mohan P, Shaibani A, Russell EJ, Hurley MC. Autosomal Dominant Polycystic Kidney Disease and Intracranial Aneurysms: Is There an Increased Risk of Treatment?. AJNR Am J Neuroradiol. 2015 Sep 3. [Medline].

  37. Schrier RW. Optimal care of autosomal dominant polycystic kidney disease patients. Nephrology (Carlton). 2006 Apr. 11(2):124-30. [Medline].

  38. Patch C, Charlton J, Roderick PJ, Gulliford MC. Use of antihypertensive medications and mortality of patients with autosomal dominant polycystic kidney disease: a population-based study. Am J Kidney Dis. 2011 Jun. 57(6):856-62. [Medline].

  39. Russell RT, Pinson CW. Surgical management of polycystic liver disease. World J Gastroenterol. 2007 Oct 14. 13(38):5052-9. [Medline].

  40. Sallee M, Rafat C, Zahar JR, et al. Cyst infections in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2009 Jul. 4(7):1183-9. [Medline]. [Full Text].

  41. Torres VE, Chapman AB, Devuyst O, Gansevoort RT, Grantham JJ, Higashihara E, et al. Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease. N Engl J Med. 2012 Nov 3. [Medline].

  42. Caroli A, Perico N, Perna A, et al, for the ALADIN study group. Effect of longacting somatostatin analogue on kidney and cyst growth in autosomal dominant polycystic kidney disease (ALADIN): a randomised, placebo-controlled, multicentre trial. Lancet. 2013 Aug 20. [Medline].

  43. Douglas D. Somatostatin analogue helpful in polycystic kidney disease. Reuters Health Information. September 03, 2013. [Full Text].

  44. Doulton TW, Saggar-Malik AK, He FJ, Carney C, Markandu ND, Sagnella GA, et al. The effect of sodium and angiotensin-converting enzyme inhibition on the classic circulating renin-angiotensin system in autosomal-dominant polycystic kidney disease patients. J Hypertens. 2006 May. 24(5):939-45. [Medline].

  45. Tahvanainen E, Tahvanainen P, Kääriäinen H, et al. Polycystic liver and kidney diseases. Ann Med. 2005. 37(8):546-55. [Medline].

Polycystic kidney.
Polycystic kidney disease and massive polycystic liver disease.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.