Polycystic Kidney Diseases (Polycystic Kidney Disease)

Hereditary diseases that are characterized by the progressive expansion of a large number of tightly packed CYSTS within the KIDNEYS. They include diseases with autosomal dominant and autosomal recessive inheritance.

Also Known As:

Polycystic Kidney Disease; Polycystic Kidney; Kidney, Polycystic; Polycystic Kidneys; Polycystic Renal Disease; Disease, Polycystic Kidney; Disease, Polycystic Renal; Diseases, Polycystic Kidney; Diseases, Polycystic Renal; Kidney Disease, Polycystic; Kidney Diseases, Polycystic; Kidneys, Polycystic; Polycystic Renal Diseases; Renal Disease, Polycystic; Renal Diseases, Polycystic

Networked: 1968 relevant articles (67 outcomes, 169 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Cysts
2.	Chronic Kidney Failure (Chronic Renal Failure)
3.	Liver Diseases (Liver Disease)
4.	Glomerulonephritis
5.	Autosomal Dominant Polycystic Kidney (ADPKD)

Experts

1.	Torres, Vicente E: 44 articles (01/2022 - 11/2003)
2.	Harris, Peter C: 26 articles (01/2022 - 11/2003)
3.	Wallace, Darren P: 24 articles (01/2021 - 06/2003)
4.	Somlo, Stefan: 19 articles (01/2021 - 03/2002)
5.	Peters, Dorien J M: 18 articles (01/2020 - 09/2005)
6.	Phillips, Jacqueline K: 17 articles (01/2022 - 11/2011)
7.	Yamaguchi, Tamio: 16 articles (01/2022 - 02/2003)
8.	Aukema, Harold M: 16 articles (01/2021 - 02/2003)
9.	Zhou, Jing: 15 articles (11/2020 - 02/2002)
10.	Wang, Xiaofang: 14 articles (01/2022 - 11/2003)

Drugs and Biologics

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

1.	Tolvaptan FDA Link 01/01/2020 - "Doubts about the efficacy of tolvaptan for polycystic kidney disease." 08/24/2023 - "The Efficacy, the Treatment Response and the Aquaretic Effects of a Three-Year Tolvaptan Regimen in Polycystic Kidney Disease Patients." 01/01/2023 - "Retrospective evaluation at an expert PKD centre revealed that in a cohort demonstrating rapid estimated glomerular filtration rate (eGFR) decline, 14% would not be eligible for tolvaptan by Mayo imaging classification and more than half (57%) would not be eligible by Predicting Renal Outcome in Polycystic Kidney Disease score. " 11/06/2022 - "Tolvaptan ameliorated kidney function for one elderly autosomal dominant polycystic kidney disease patient: A case report." 01/01/2022 - "Delay in funding of tolvaptan for polycystic kidney disease in Aotearoa New Zealand."
2.	Sirolimus (Rapamycin)FDA Link 11/01/2023 - "Although rapamycin is a very effective drug for rodents with polycystic kidney disease (PKD), it is not encouraging in the clinical trials due to the suboptimal dosages compelled by the off-target side effects. " 10/28/2009 - "Rapamycin has been shown to have beneficial effects in rodent models of polycystic kidney disease, prompting the initiation of pilot clinical trials with human patients. " 08/01/2009 - "Therefore, at present, polycystic kidney disease is the most promising therapeutic application for sirolimus in non-transplant renal diseases, and further studies are needed to clarify its role in other situations." 01/01/2018 - "Both unconjugated rapamycin and FC-rapa are similarly effective on polycystic kidneys in this model. " 05/01/2015 - "4D MRI of polycystic kidneys from rapamycin-treated Glis3-deficient mice."
3.	Somatostatin (Somatotropin Release-Inhibiting Factor)IBA 01/01/2020 - "Long-acting somatostatin analogs have also been effective in slowing the rates of growth of polycystic kidneys and liver. " 11/01/2009 - "Treatment with somatostatin analogues slowed kidney growth in patients with polycystic kidney disease (PKD) and reduced liver and kidney volume in a PKD rodent model. " 01/01/2016 - "Short-term studies have shown that somatostatin analogues are effective in patients with polycystic kidney and liver disease. " 01/01/2014 - "In recent clinical studies, somatostatin analogues have demonstrated efficacy in isolated polycystic liver disease and, to a lesser extent, in polycystic kidney disease. " 10/01/2013 - "Polycystic kidney disease: Trial of a long-acting somatostatin analogue for autosomal dominant polycystic kidney disease."
4.	1-dodecylpyridoxal (PLD)IBA 01/01/2020 - "Polycystic kidney (PCK) rats, a model of PLD, were randomly divided into a sedentary group and a long-term exercise group, which underwent treadmill running for 12 wk (28 m·min, 60 min·d, 5 d·wk). " 01/01/2016 - "PLD patients scored higher on the PLD-Q total score than general controls (NL, 42 vs. 17; US, 40 vs. 13 points) and polycystic kidney disease patients without PLD (22 points). " 01/01/2016 - "To test discriminative validity, we compared PLD-Q total scores of patients with different PLD severity stages (Gigot classification) and PLD-Q total scores of PLD patients with general controls and polycystic kidney disease patients without PLD. " 10/01/2012 - "Polycystic kidney (PKD) and liver (PLD) diseases cause significant morbidity and mortality. " 03/01/2012 - "Cdc25A expression was analyzed in livers of control and polycystic kidney (PCK) rats, control and polycystic kidney 2 (Pkd2(ws25/-)) mice, healthy individuals, and patients with PLD. "
5.	Soybean Proteins (Soy Protein)IBA 05/06/2016 - "Studies conducted in animal models have helped to identify the underlying molecular mechanisms that may play a role in the positive effects of soy protein on renal parameters in polycystic kidney disease, metabolically-induced kidney dysfunction and age-associated progressive nephropathy. " 01/01/1998 - "We undertook a study to determine whether soy protein feeding would ameliorate renal injury in the Han:SPRD-cy rat model of polycystic kidney disease (PKD). " 07/01/2009 - "A model in which a beneficial soy protein effect has been consistently demonstrated is the Han:SPRD-cy rat model of polycystic kidney disease. " 01/01/2007 - "Late dietary intervention limits benefits of soy protein or flax oil in experimental polycystic kidney disease." 01/01/1998 - "Soy protein modification of rat polycystic kidney disease."
6.	Paclitaxel (Taxol)FDA LinkGeneric 12/01/1997 - "Efficacy of taxol in the orpk mouse model of polycystic kidney disease." 05/01/1997 - "This dramatic moderation of polycystic kidney disease progression has been postulated to be a result of paclitaxel's ability to stabilize microtubules. " 03/01/1997 - "Woo et al (Nature 368:750-753) reported that parenteral administration of paclitaxel arrested the striking renal enlargement and prolonged life in C57BL/6J-cpk/cpk mice with a rapidly progressive form of polycystic kidney disease (PKD). " 03/01/1997 - "The effect of paclitaxel on the progression of polycystic kidney disease in rodents." 04/21/1994 - "Taxol inhibits progression of congenital polycystic kidney disease."
7.	Anti-Bacterial Agents (Antibiotics)IBA 10/01/1981 - "The concentrations of several antibiotics were measured in the cyst fluid of six adult patients with polycystic kidney disease. " 06/01/1989 - "Because of improved treatments such as antibiotic and hemodialysis techniques the five-year survival rate of patients, with polycystic kidney diseases, may be increased." 01/01/1984 - "Treatment with oral antibiotics proved successful in 13 episodes but 2 patients with polycystic kidneys were admitted to hospital for intravenous therapy. " 10/01/1981 - "Cyst fluid antibiotic concentrations in polycystic kidney disease: differences between proximal and distal cysts." 01/01/2022 - "Efficacy of Prolonged Antibiotic Therapy for Renal Cyst Infections in Polycystic Kidney Disease."
8.	Chloramphenicol (Chloromycetin)FDA Link 03/01/1976 - "Comparison of renal excretion of chloramphenicol in patients with chronic pyelonephritis, glomerulonephritis and polycystic kidneys." 04/04/1975 - "[Renal chloramphenicol clearance in patients with chronic pyelonephritis, glomerulonephritis or polycystic kidneys (author's transl)]." 05/01/1978 - "In patients with chronic renal failure due to glomerulonephritis, pyelonephritis or polycystic kidneys the urinary clearance of free chloramphenicol (C(CHL)) was depressed proportionally to GFR (C(In)). " 03/01/1976 - "In patients with chronic pyelonephritis, glomerulonephritis and polycystic kidneys, decrease in renal clearance of chloramphenicol (CCHL) is related to a decrease in the clearance of inulin (Cin). "
9.	TOR Serine-Threonine KinasesIBA 01/01/2018 - "Inhibition of mammalian target of rapamycin (mTOR) slows polycystic kidney disease (PKD) progression in animal models, but randomized controlled trials failed to prove efficacy of mTOR inhibitor treatment. " 05/01/2012 - "Inhibition of the mammalian target of rapamycin (mTOR) shows beneficial effects in animal models of polycystic kidney disease (PKD); however, two clinical trials in patients with autosomal dominant PKD failed to demonstrate a short-term benefit in either the early or progressive stages of disease. " 07/01/2008 - "Recent studies suggested abnormalities of the mammalian target of rapamycin signaling pathway in polycystic kidney disease. " 11/01/2018 - "Mammalian target of rapamycin inhibitors in a patient with polycystic kidney disease-1-tuberous sclerosis-2 contiguous gene syndrome." 01/01/2013 - "The mammalian target of rapamycin (mTOR) signaling pathway is aberrantly activated in polycystic kidney disease (PKD). "
10.	Caseins (Casein)FDA Link 07/01/2002 - "Effect on histology: Low casein and soy feeding ameliorates Han: SPRD-cy rat polycystic kidney disease reducing both tubular remodeling and interstitial inflammation and fibrosis, while flax seed diet effect appears to be through moderation of associated interstitial nephritis. " 07/01/2002 - "We undertook several studies to determine if feeding with low protein casein, soy diet and flax seed diet would ameliorate renal injury in Han:SPRD-cy rat model of polycystic kidney disease. " 07/01/2001 - "Polycystic kidney disease induced in F(1) Sprague-Dawley rats fed para-nonylphenol in a soy-free, casein-containing diet." 02/01/1999 - "The effects of dietary soy protein compared to casein were examined in male and female CD1-pcy/pcy (pcy) mice with polycystic kidney disease. " 01/01/1998 - "Genistein supplementation of a casein-based diet in study 2 did not reduce the renal enlargement and cyst development associated with progression of polycystic kidney disease. "

Therapies and Procedures

1.	Therapeutics 11/01/1985 - "In polycystic renal disease, leukocyte scans have advantages over standard diagnostic modalities and are very helpful in planning appropriate therapy." 06/23/2015 - "Several in vivo pre-clinical studies in Polycystic Kidney Disease (PKD) utilize orthologous rodent models to identify and study the genetic and molecular mechanisms responsible for the disease, and are very convenient for rapid drug screening and testing of promising therapies. " 10/01/2014 - "Rodent models are available to study the pathogenesis of polycystic kidney disease (PKD) and for preclinical testing of potential therapies-either genetically engineered models carrying mutations in Pkd1 or Pkd2 or models of renal cystic disease that do not have mutations in these genes. " 01/01/2021 - "The cellular pathways and potential therapeutics of Polycystic Kidney Disease." 01/01/2020 - "In this review, we focus on the role of signal transducer and activator of transcription (STAT) signaling pathways in various renal cell types in healthy kidneys as compared to polycystic kidneys, on the mechanisms of STAT regulation by PC1 and other factors, and on the possibility to target STAT signaling for PKD therapy."
2.	Nephrectomy 02/01/2007 - "Patients with massive polycystic kidneys with a kidney volume of greater than 3,500 cc are at increased risk for open conversion and they may have improved outcomes if open nephrectomy is attempted from the outset." 05/01/2013 - "Our study showed that laparoscopic nephrectomy for polycystic kidney is feasible and reproducible. " 05/01/2013 - "The aim of our study was to report our experience of laparoscopic nephrectomy in patients with polycystic kidney disease. " 11/01/2023 - "Robotic bilateral nephrectomy for large polycystic kidney disease." 07/01/2023 - "For this compressive complication of the polycystic kidney, a nephrectomy was performed after embolization of the renal artery, given the haemorrhage risk. "
3.	Kidney Transplantation 11/01/2015 - "We aimed to retrospectively assess the long-term safety and efficacy of embolization of renal arteries (ERA) in patients with polycystic kidney disease (PKD) before renal transplantation. " 01/01/2008 - "Outcome of kidney transplantation in patients with polycystic kidney disease: a single center study." 01/01/2021 - "Renal Transplantation from a Deceased Donor with Polycystic Kidney Disease." 09/01/2020 - "In this case description, we describe the first case of de novo TMA in a patient with polycystic kidney disease that occurred immediately after kidney transplantation. " 01/01/2018 - "Change in kidney volume after kidney transplantation in patients with autosomal polycystic kidney disease."
4.	Renal Dialysis (Hemodialysis) 01/01/2021 - "Recipient 1: a 49 years old man with polycystic kidney disease had been on hemodialysis for 28 months. " 01/01/2019 - "We report a 55-year-old man who began undergoing hemodialysis for polycystic kidney disease 17 years ago. " 01/01/2014 - "All the medical records of patients with polycystic kidneys who initiated hemodialysis between 1995 and 2012, in four centers that treat patients of the coverage area of the 15th regional health Paraná (Brazil), were analyzed. " 07/01/2012 - "Maintenance hemodialysis (MHD) patients with polycystic kidney disease (PKD) have better survival than non-PKD patients. " 12/01/2010 - "Blood pressure and survival in long-term hemodialysis patients with and without polycystic kidney disease."
5.	Drug Therapy (Chemotherapy) 11/01/2015 - "PCK rats develop age-related polycystic kidney disease (PKD) and liver disease and have been used to investigate pharmacotherapies to ameliorate hepatorenal lesions for patients with PKD. " 01/01/2016 - "Wilms Tumor in a Child With Bilateral Polycystic Kidneys and PHACE Syndrome: Successful Treatment Outcome Using Partial Nephrectomy and Chemotherapy." 01/01/2012 - "Agonists of PPARγ exert protective effects against various kidney diseases including diabetic nephropathy, ischemic renal injury, IgA nephropathy, chemotherapy-associated kidney damage, polycystic kidney diseases and age-related kidney diseases via both systemic and renal actions. " 01/01/2016 - "We report a 17-month-old baby with bilateral polycystic kidneys and PHACE syndrome who developed nephroblastoma in the right polycystic kidney which was treated successfully with nephron-sparing partial nephrectomy and chemotherapy. " 10/01/2011 - "Anti-VEGF drug therapy may therefore not be a treatment option for polycystic kidney disease."