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Autosomal Dominant Polycystic Kidney (ADPKD)

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Kidney disorders with autosomal dominant inheritance and characterized by multiple CYSTS in both KIDNEYS with progressive deterioration of renal function.

Also Known As:

ADPKD; Polycystic Kidney Disease, Autosomal Dominant; Polycystic Kidney, Autosomal Dominant; Adult Polycystic Kidney Disease; Adult Polycystic Kidney Disease Type 1; Adult Polycystic Kidney Disease Type 2; Polycystic Kidney, Type 1 Autosomal Dominant Disease; Polycystic Kidney, Type 2 Autosomal Dominant Disease; Kidney, Polycystic, Autosomal Dominant

Networked: 1352 relevant articles (67 outcomes, 218 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Autosomal Dominant Polycystic Kidney (ADPKD)
2.	Cysts
3.	Infection
4.	Pain (Aches)
5.	Chronic Kidney Failure (Chronic Renal Failure)

Experts

1.	Torres, Vicente E: 52 articles (11/2015 - 01/2003)
2.	Harris, Peter C: 32 articles (08/2015 - 05/2002)
3.	Schrier, Robert W: 28 articles (09/2015 - 04/2002)
4.	Grantham, Jared J: 26 articles (07/2015 - 04/2002)
5.	Wallace, Darren P: 23 articles (12/2015 - 04/2002)
6.	Gansevoort, Ron T: 23 articles (10/2015 - 11/2010)
7.	Chapman, Arlene B: 22 articles (07/2015 - 01/2003)
8.	Somlo, Stefan: 22 articles (05/2014 - 03/2002)
9.	Zhou, Jing: 16 articles (11/2015 - 03/2002)
10.	Meijer, Esther: 16 articles (10/2015 - 11/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Autosomal Dominant Polycystic Kidney:

1.	tolvaptan FDA Link 05/01/2008 - "A phase 3, placebo-controlled, double-blind study in 18- to 50-yr-old patients with autosomal dominant polycystic kidney disease and preserved renal function but relatively rapid progression, as indicated by a total kidney volume >750 ml, has been initiated and will determine whether tolvaptan is effective in slowing down the progression of this disease." 10/01/2015 - "Although the use of tolvaptan requires careful consideration and balancing of benefits and risks, current evidence suggests that tolvaptan is a promising new treatment option for patients with ADPKD. " 10/01/2015 - "To evaluate the safety and efficacy of tolvaptan, we performed a subgroup analysis in the participating Japanese ADPKD patient population. " 11/01/2015 - "Clinical Pattern of Tolvaptan-Associated Liver Injury in Subjects with Autosomal Dominant Polycystic Kidney Disease: Analysis of Clinical Trials Database." 10/01/2015 - "In the large phase III TEMPO 3:4 trial in adults with ADPKD, 3 years' treatment with oral tolvaptan significantly reduced growth in total kidney volume and slowed renal function decline relative to placebo. "
2.	Sirolimus (Rapamycin)FDA Link 12/01/2009 - "Inhibition of mTOR by rapamycin or one of its analogues represents a potentially novel treatment for autosomal dominant polycystic kidney disease. " 12/01/2009 - "The efficacy of mammalian target of rapamycin (mTOR) inhibitors is currently tested in patients affected by autosomal dominant polycystic kidney disease. " 01/01/2015 - "Pulsed oral sirolimus in advanced autosomal-dominant polycystic kidney disease (Vienna RAP Study): study protocol for a randomized controlled trial." 09/15/2014 - "In human ADPKD studies, sirolimus, a mammalian target of rapamycin complex 1 (mTORC1) inhibitor, had little therapeutic effect. " 01/01/2014 - "The objective of our study was to conduct a meta-analysis of randomized controlled trials (RCTs) to present an objective appraisal of the efficacy and safety of sirolimus therapy in patients with ADPKD. "
3.	Somatostatin (Somatotropin Release-Inhibiting Factor)IBA 07/01/2005 - "Safety and efficacy of long-acting somatostatin treatment in autosomal-dominant polycystic kidney disease." 07/01/2005 - "In ADPKD patients, 6-month somatostatin therapy is safe and may slow renal volume expansion. " 10/01/2013 - "Polycystic kidney disease: Trial of a long-acting somatostatin analogue for autosomal dominant polycystic kidney disease." 05/01/2011 - "Several clinical trials published over the last year now show that somatostatin analogues when given for 6-12 months in patients with ADPKD and PCLD decrease total liver volume, attenuate polycystic kidney volume, and improve perception of health. " 11/02/2013 - "Effect of longacting somatostatin analogue on kidney and cyst growth in autosomal dominant polycystic kidney disease (ALADIN): a randomised, placebo-controlled, multicentre trial."
4.	Vasopressin Receptors (Arginine Vasopressin Receptor)IBA 08/01/2011 - "Therapeutic potential of vasopressin V2 receptor antagonist in a mouse model for autosomal dominant polycystic kidney disease: optimal timing and dosing of the drug." 08/01/2011 - "The renoprotective effect of vasopressin V2 receptor antagonist (V2RA) is currently being tested in a clinical trial in early autosomal dominant polycystic kidney disease (ADPKD). " 01/01/2004 - "OPC31260, a V2 receptor antagonist, lowers renal cAMP and markedly inhibits cystogenesis in four animal models of polycystic kidney disease, three of which are orthologous to human diseases (PCK rat, ARPKD; pcy mouse, adolescent nephronophthisis; Pkd2WS25/- mouse, ADPKD). " 11/15/2012 - "In autosomal dominant polycystic kidney disease (ADPKD), binding of AVP to the V2 receptor (V2R) increases cAMP and accelerates cyst growth by stimulating cell proliferation and Cl(-)-dependent fluid secretion. " 04/01/2015 - "Several drugs designed to slow or arrest the progression of ADPKD have shown promise in preclinical models and clinical trials, including vasopressin receptor antagonists and somatostatin analogs. "
5.	Polycystic liver diseaseIBA 05/01/2015 - "We included 43 ADPKD patients with polycystic liver disease (84% female, median age 50 years, mean eGFR 63 ml/min/1.73 m(2) ). " 08/01/2014 - "Autosomal-dominant polycystic kidney disease is frequently complicated by polycystic liver disease. " 04/08/2014 - "Polycystic livers are seen in the rare inherited disorder isolated polycystic liver disease (PCLD) and are recognized as the most common extrarenal manifestation in autosomal dominant polycystic kidney disease. " 01/07/2013 - "PCLDs may exist isolated (i.e., autosomal dominant polycystic liver disease, ADPLD) or in combination with renal cystogenesis (i.e., autosomal dominant polycystic kidney disease and autosomal recessive polycystic liver disease). " 12/27/2012 - "A review of literature on polycystic liver disease in patients with ADPKD and current management strategies are presented. "
6.	Proteins (Proteins, Gene)IBA 02/01/2013 - "Genome wide association and candidate studies of multiplex families with IAs without ADPKD have identified a number of genes/proteins that may be risk factors for the development of IAs. " 12/01/2002 - "The PKD1 and PKD2 genes responsible for ADPKD and their respective encoded proteins polycystin-1 and polycystin-2 are under intense study and clues are developing as to their function and roles in the disease process. " 08/01/2015 - "However, neither the functions of these proteins nor the molecular mechanisms of ADPKD pathogenesis are well understood. " 06/01/2015 - "Impressively, these proteins are involved in biological processes that are closely related to the pathogenic state of tubular epithelial cells in ADPKD, demonstrating the possibility to monitor the status of patients using urinary EVs." 05/01/2015 - "Recent evidence suggests that the polycystin proteins, which are dysfunctional in ADPKD, are found in ciliated choroid plexus cells that are involved with regulation of cerebrospinal fluid homeostasis. "
7.	CreatinineIBA 09/01/2011 - "This retrospective singlecenter study was undertaken to evaluate patient/graft survival function vis-a-vis serum creatinine (SCr), rejection episodes and mortality in ADPKD vs matched control patients. " 01/01/2009 - "Forty-nine patients with ADPKD (age 35.9 +/- 11.1 years) with serum creatinine <1.35 mg/dl and 50 healthy controls (36.7 +/- 9.2 years) were enrolled for the study. " 12/01/1990 - "The present study was undertaken to examine whether the renal structural abnormalities are greater in hypertensive (HBP) versus normotensive (NBP) male and female patients with ADPKD who were matched within gender groups for age, body surface area, serum creatinine concentration (males HBP 1.2 +/- 0.02 vs. NBP 1.1 +/- 0.03 mg/dl. " 01/01/2015 - "The serum creatinine (Scr) levels in patients with stage I ADPKD were within normal limits 1 and 3 years postoperatively and did not differ significantly (P > 0.05). " 09/01/2014 - "ADPKD patients with creatinine clearance ≧ 50 mL/min/1.73 m(2) were divided into high (H-, n = 18) and free (F-, n = 16) water-intake groups, mainly according to their preference. "
8.	Biological Markers (Surrogate Marker)IBA 01/01/2013 - "In conclusion, the performance of peptidomic biomarker scores is superior to any other biochemical markers of ADPKD and the proteomic biomarker patterns are a promising tool for prognostic evaluation of ADPKD." 09/01/2013 - "Renal function remains stable for decades in ADPKD, and thus clinically meaningful surrogate markers to assess therapeutic efficacy are needed. " 01/01/2015 - "Research in several congenital kidney and urinary tract anomalies, ureteropelvic junction obstruction and autosomal dominant polycystic kidney disease, has discovered novel biomarkers, which may help to imply the mechanisms underlying inherited disorders. " 02/01/2015 - "The aim of this study is to review the clinical and laboratory markers of ADPKD progression. " 01/01/2015 - "Further studies are needed to evaluate the property of the aforementioned biomarkers to assess disease state at early ADPKD stage."
9.	lanreotide (Somatuline)FDA Link 01/01/2012 - "The RESOLVE trial aims to assess the efficacy of lanreotide treatment in ADPKD patients with symptomatic polycystic livers. " 05/01/2015 - "Effect of lanreotide on polycystic liver and kidneys in autosomal dominant polycystic kidney disease: an observational trial." 01/01/2012 - "Rationale and design of the RESOLVE trial: lanreotide as a volume reducing treatment for polycystic livers in patients with autosomal dominant polycystic kidney disease." 05/01/2015 - "Lanreotide reduced polycystic liver and kidney volumes and decreases symptoms in ADPKD patients. " 05/01/2015 - "Chronic renal dysfunction in ADPKD may affect treatment efficacy of lanreotide and possibly enhances risk for adverse events. "
10.	Peptidyl-Dipeptidase A (Angiotensin Converting Enzyme)IBA 12/01/1999 - "As in clinical studies, angiotensin-converting enzyme inhibition may be less beneficial in ADPKD than in renal diseases characterized by predominant glomerular injury." 11/01/2006 - "Although the role for the angiotensin I-converting enzyme (ACE) insertion/deletion (I/D) polymorphism as a modifier factor in ADPKD renal deterioration has been suggested, direct evidence from genetic association studies remain inconclusive. " 09/01/2013 - "The results in period B compared with period A demonstrated an earlier age of autosomal dominant polycystic kidney disease diagnosis (29 vs 35 years, P < .001), lower mean blood pressure (129/82 vs 142/91 mm Hg, P < .001), better estimated glomerular filtration rate (63.6 vs 44.6 mL/min, P < .001), and more therapy with angiotensin-converting enzyme inhibition (42.5% vs 13.6%, P < .001). " 09/01/2007 - "Effect of statin and angiotensin-converting enzyme inhibition on structural and hemodynamic alterations in autosomal dominant polycystic kidney disease model." 12/01/2001 - "Angiotensin-converting enzyme activity and the ACE Alu polymorphism in autosomal dominant polycystic kidney disease."

Therapies and Procedures

1.	Nephrectomy 07/01/2001 - "They uniformly noticed improved preoperative pulmonary and gastrointestinal symptoms Hand assisted laparoscopic nephrectomy in patients with autosomal dominant polycystic kidney disease makes bilateral nephrectomy a reasonable option. " 02/01/1996 - "To the best of our knowledge, the two nephrectomy specimens in this study represent the largest kidneys removed laparoscopically to date and the first laparoscopic nephrectomies in ADPKD patients." 11/01/2015 - "The objective of our analysis was to evaluate graft survival in patients with ADPKD in which we conduct pretransplant nephrectomy. " 05/01/2015 - "We retrospectively reviewed all laparoscopic bilateral nephrectomies performed for ADPKD at our institution from 1 January 2000 to 31 December 2012. " 01/01/2015 - "Laparoscopic Nephrectomy versus Open Nephrectomy for Patients with Autosomal Dominant Polycystic Kidney Disease: A Systematic Review and Meta-Analysis."
2.	Transplants (Transplant) 07/01/2002 - "The better outcome of female ADPKD graft recipients compared to the male group may be related to a gender-dependent disease severity, possibly due to hormonal effects. " 09/15/1991 - "While relatives are attractive as renal donors due to their availability and the improved transplant success associated with living-related donors, they may coincidentally be at risk for ADPKD. " 06/01/2011 - "This study provides important new insights that could lead to a better care for renal transplant patients with ADPKD." 11/01/2015 - "Eighty-seven patients underwent a kidney transplant owing to ADPKD; 62% (n = 54) were male, with an average age of 55.22 years. " 11/01/2015 - "This retrospective study including renal transplant patients secondary to ADPKD in our hospital between January 2000 and December 2012. "
3.	Renal Dialysis (Hemodialysis) 01/01/2008 - "Thus, the aim of the present study was to investigate both the frequency and clinical profile of ADPKD among hemodialysis patients in south of Brazil. " 02/01/2014 - "Successful pregnancy in a patient with autosomal dominant polycystic kidney disease on long-term hemodialysis." 01/01/2014 - "[Autosomal dominant polycystic kidney disease in hemodialysis patients in Southern Brazil]." 10/01/2012 - "Transcatheter renal artery embolization improves lung function in patients with autosomal dominant polycystic kidney disease on hemodialysis." 01/01/2012 - "A 52-year-old male with autosomal dominant polycystic kidney disease undergoing hemodialysis experienced an episode of S. "
4.	Lithotripsy (Extracorporeal Shockwave Lithotripsy) 06/01/1997 - "Thirteen patients with ADPKD and symptomatic or obstructive renal stones presented to our hospital for evaluation and treatment with extracorporeal shockwave lithotripsy (SWL). " 06/01/2013 - "Shockwave lithotripsy (SWL) for urinary calculi in ADPKD was reported to be a safe and effective treatment option. " 10/01/2010 - "The search terms used were ADPKD, nephrolithiasis, percutaneous nephrolithotomy, shockwave lithotripsy, flexible ureterorenoscopy, congenital kidney disorders, etc., separately and in various combinations. " 08/01/2009 - "Hepatolithiasis in a patient with autosomal dominant polycystic kidney disease treated by extracorporeal shock wave lithotripsy (ESWL)." 06/01/1997 - "Extracorporeal shockwave lithotripsy for urinary calculi in autosomal dominant polycystic kidney disease."
5.	Kidney Transplantation 01/01/2014 - "The objective of this study was to evaluate a longitudinal experience of kidney transplantation for ADPKD. " 01/01/2008 - "This study was undertaken to evaluate the demographics, outcome and complications of renal transplantation in patients with ADPKD. " 01/01/2016 - "Blood pressure control was studied retrospectively in 118 patients with autosomal dominant polycystic kidney disease who underwent renal transplantation between 2003 and 2013. " 05/01/2015 - "Predictive genetic testing can facilitate donor evaluation and augment living related kidney transplantation in ADPKD. " 01/01/2015 - "New onset diabetes after kidney transplantation in patients with autosomal dominant polycystic kidney disease: systematic review protocol."