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Polycystic Kidney Diseases (Polycystic Kidney Disease)

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

Networked: 1317 relevant articles (36 outcomes, 102 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Cysts
2.	Hypertension (High Blood Pressure)
3.	Chronic Kidney Failure (Chronic Renal Failure)
4.	Liver Diseases (Liver Disease)
5.	Infection

Experts

1.	Torres, Vicente E: 26 articles (08/2015 - 11/2003)
2.	Harris, Peter C: 15 articles (08/2015 - 11/2003)
3.	Somlo, Stefan: 15 articles (05/2015 - 03/2002)
4.	Aukema, Harold M: 12 articles (01/2015 - 02/2003)
5.	Edelstein, Charles L: 11 articles (02/2015 - 01/2005)
6.	Wang, Xiaofang: 10 articles (08/2015 - 11/2003)
7.	Yamaguchi, Tamio: 10 articles (01/2015 - 02/2003)
8.	Wallace, Darren P: 10 articles (03/2014 - 06/2003)
9.	Phillips, Jacqueline K: 9 articles (09/2015 - 11/2011)
10.	Walz, Gerd: 9 articles (03/2011 - 08/2003)

Drugs and Biologics

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

1.	Sirolimus (Rapamycin)FDA Link 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. " 01/01/2009 - "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. " 07/01/2008 - "Recent studies suggested abnormalities of the mammalian target of rapamycin signaling pathway in polycystic kidney disease. " 05/01/2015 - "4D MRI of polycystic kidneys from rapamycin-treated Glis3-deficient mice." 01/01/2013 - "The mammalian target of rapamycin (mTOR) signaling pathway is aberrantly activated in polycystic kidney disease (PKD). "
2.	Polycystic liver diseaseIBA 11/01/2008 - "We designed this study with the specific aim to dissect whether pathways involved in polycystic kidney diseases are also implicated in polycystic liver disease. " 07/01/2008 - "This report describes four cases with polycystic kidney disease with or without liver involvement and one case with polycystic liver disease with corroborative radiological studies." 03/01/2010 - "Polycystic liver disease (PCLD) without polycystic kidney is infrequent in clinical setting. " 01/01/2010 - "Severe polycystic liver disease can complicate adult dominant polycystic kidney disease, a genetic disease caused by defects in polycystin-1 (Pkd1) or polycystin-2 (Pkd2). " 11/01/2005 - "Adult polycystic liver disease (APLD) is an autosomal dominant condition most commonly associated with polycystic kidney disease. "
3.	Phosphotransferases (Kinase)IBA 08/22/2013 - "NEK8/NPHP9 is a ciliary kinase associated with two renal ciliopathies in humans and mice, nephronophthisis (NPHP) and polycystic kidney disease. " 05/01/2011 - "Polycystic kidney disease (PKD) in mice can arise from defects in Nek kinases, which participate in ciliogenesis. " 12/01/2005 - "The NIMA-related kinases (Nek) Nek1 and Nek8 carry the causal mutations of two of the eight established mouse models of polycystic kidneys. " 12/01/2005 - "NIMA-related kinases defective in murine models of polycystic kidney diseases localize to primary cilia and centrosomes." 01/01/2004 - "There is also evidence of abnormal cross-talk between cAMP and MAPK pathways, that can be reproduced in wild-type cells by altering [Ca2+]i. While cAMP inhibits Ras-Raf-1-stimulated phosphorylation of ERK in normal kidney cells, it markedly increases B-Raf kinase activity and ERK phosphorylation in polycystic kidney cells. "
4.	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."
5.	Somatostatin (Somatotropin Release-Inhibiting Factor)IBA 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. " 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. " 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. " 01/01/2015 - "Thus, somatostatin (SST) analogs become potential and promising alternatives in treating human polycystic kidney disease (PKD) and polycystic liver disease (PLD) via interacting with Gαi protein-coupled SSTRs and further blocking cAMP production. "
6.	Caseins (Casein)FDA Link 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. " 07/01/2002 - "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. " 07/01/2011 - "Weanling Han:SPRD-cy rats with experimental polycystic kidney disease were given hemp-, pea- and soy protein-based diets compared with the standard AIN 93G diet with casein as the protein source. " 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. "
7.	pioglitazone (Actos)FDA Link 01/01/2009 - "These findings suggest that pioglitazone may have a unique antihypertensive effect in polycystic kidney disease, and that such an effect may promote improved survival." 01/01/2010 - "Pioglitazone Attenuates Cystic Burden in the PCK Rodent Model of Polycystic Kidney Disease." 01/01/2009 - "Effect of pioglitazone on survival and renal function in a mouse model of polycystic kidney disease." 01/01/2009 - "Pioglitazone did not alter renal function, cell proliferation, apoptosis, or cyst formation in animals with polycystic kidney disease, however it did increase survival. " 10/01/2010 - "Although pioglitazone, a PPAR-gamma (peroxisome-proliferator-activated receptor-gamma) agonist, has been shown to prolong survival in two rapidly progressive pkd1 (polycystic kidney disease 1)-knockout mice models through disparate mechanisms, these studies lacked data on therapeutic potential and long-term safety because of a short observation period. "
8.	Epidermal Growth Factor (EGF)IBA 09/15/2014 - "Epidermal growth factor (EGF) is linked to the pathogenesis of polycystic kidney disease (PKD). " 09/15/2014 - "Epidermal growth factor-induced proliferation of collecting duct cells from Oak Ridge polycystic kidney mice involves activation of Na+/H+ exchanger." 01/01/2005 - "Abnormal ENaC activity and EGF signaling are both associated with polycystic kidney disease localized to the distal nephron. " 01/01/2001 - "The role of EGF in the progression of polycystic kidney disease in bpk mice is relatively complicated, with neonatal treatment being associated with some amelioration of the renal dysfunction and extrarenal pathology without an effect on the renal pathology. " 06/01/1995 - "Biliary epithelial cells from mice with congenital polycystic kidney disease are hyperresponsive to epidermal growth factor."
9.	Soybean Proteins (Soy Protein)IBA 03/01/1998 - "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." 01/01/2000 - "Modification of polycystic kidney disease and fatty acid status by soy protein diet. " 01/01/2000 - "Modification of polycystic kidney disease and fatty acid status by soy protein diet."
10.	ChitinIBA 06/01/2003 - "The depolymerases were modular in nature and contained glycosyl hydrolase family 18 domains, chitin-binding domains, and polycystic kidney disease domains. " 01/01/2015 - "We improved catalytic efficiency of SpChiD in degradation of insoluble chitin substrates by fusing with auxiliary domains like polycystic kidney disease (PKD) domain and chitin binding protein 21 (CBP21). " 01/01/2005 - "The aim of study was to clarify whether the polycystic kidney disease (PKD) domain of chitinase A (ChiA) participates in the hydrolysis of powdered chitin. " 12/01/2005 - "Homology modelling of the chitinase of Epiphyas postvittana nucleopolyhedrovirus (EppoNPV) against Serratia marcescens chitinase A indicated that the enzyme possesses an N-terminal polycystic kidney 1 (PKD1) domain for chitin-substrate feeding and an alpha/beta TIM barrel catalytic domain characteristic of a family 18 glycohydrolase. " 01/01/2005 - "Role of the N-terminal polycystic kidney disease domain in chitin degradation by chitinase A from a marine bacterium, Alteromonas sp. strain O-7."

Therapies and Procedures

1.	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. " 11/01/2015 - "Graft Survival in Patients With Polycystic Kidney Disease With Nephrectomy of Native Kidney Pretransplant." 05/01/2015 - "Massive size of polycystic kidneys is not a contraindication to attempting a laparoscopic approach to bilateral nephrectomies in an experienced, high-volume centre."
2.	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." 01/01/2015 - "Inadvertent transmission of polycystic kidney disease in kidney transplantation." 02/01/2013 - "The ipsilateral polycystic kidney was removed at the time of renal transplantation. " 09/01/2010 - "Furthermore, we consider that bilateral renal TAE is necessary for polycystic kidney patients prior to renal transplantation for a variety of reasons, including problems associated with contrast nephropathy if renal TAE for left kidney is remained after renal transplantation."
3.	Renal Dialysis (Hemodialysis) 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." 01/01/2014 - "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. " 07/01/2012 - "Mineral and bone disorders and survival in hemodialysis patients with and without polycystic kidney disease." 12/01/2010 - "Blood pressure and survival in long-term hemodialysis patients with and without polycystic kidney disease."
4.	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."
5.	Transplants (Transplant) 10/01/2015 - "Although fear of future appearance of polycystic kidney disease may reduce the rate of related living donors, our study showed that graft and patient survival rates in the living-donor group were as high as in the deceased-donor group." 10/01/2015 - "The low rate of morbidity and excellent survival rates make kidney transplant an excellent option for patients with polycystic kidney disease. " 10/01/2015 - "Patients with polycystic kidney disease are candidates for kidney transplant. " 07/01/2015 - "Fifteen-year survival of a polycystic kidney transplant." 03/01/2015 - "Factors significantly associated with referral before dialysis were the diagnosis of polycystic kidney disease, white recipient race, referral by a transplant nephrologist, and employed status. "