12,323 results match your criteria Polycystic Kidney Disease


Survival Advantage of African American Dialysis Patients with End-Stage Renal Disease Causes Related to APOL1.

Cardiorenal Med 2019 Apr 17;9(4):212-221. Epub 2019 Apr 17.

Kidney Disease Branch, NIDDK, Bethesda, Maryland, USA,

Background: Observational studies show that African American (AA) dialysis patients have longer survival than European Americans. We hypothesized that apolipoprotein L1 (APOL1) genetic variation, associated with nephropathy in AAs, contributes to the survival advantage in AA dialysis patients.

Methods: We examined the association between race and mortality among 37,097 adult dialysis patients, including 54% AAs and 46% European Americans from a large dialysis organization (entry period from July 2001 to June 2006, follow-up through June 2007), within each cause of end-stage renal disease (ESRD) category associated with APOL1 renal risk variants using Cox proportional hazard models. Read More

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https://www.karger.com/Article/FullText/496472
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http://dx.doi.org/10.1159/000496472DOI Listing
April 2019
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Endothelin-1 as a therapeutic target in autosomal dominant polycystic kidney disease
.

Clin Nephrol 2019 Apr 16. Epub 2019 Apr 16.

Aims: Endothelin-1 (ET-1) is associated with the pathophysiology of autosomal dominant polycystic kidney disease (ADPKD) via cyst progression. Elevated concentrations of ET-1 in ADPKD correlate with many phenotypic changes in the kidney such as renal cyst development, interstitial fibrosis, and glomerulosclerosis. In addition, an imbalance between renal ET and ET receptors possibly leads to more severe disease progression. Read More

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http://dx.doi.org/10.5414/CN109598DOI Listing

Mutation analyses by next-generation sequencing and multiplex ligation-dependent probe amplification in Japanese autosomal dominant polycystic kidney disease patients.

Clin Exp Nephrol 2019 Apr 15. Epub 2019 Apr 15.

Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan.

Background: Autosomal dominant polycystic kidney disease (ADPKD), one of the most common hereditary kidney diseases, causes gradual growth of cysts in the kidneys, leading to renal failure. Owing to the advanced technology of next-generation sequencing (NGS), genetic analyses of the causative genes PKD1 and PKD2 have been improved.

Methods: We performed genetic analyses of 111 Japanese ADPKD patients using hybridization-based NGS and long-range (LR)-PCR-based NGS. Read More

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http://dx.doi.org/10.1007/s10157-019-01736-3DOI Listing

Sexually dimorphic regulation of behavioral states by dopamine in .

J Neurosci 2019 Apr 15. Epub 2019 Apr 15.

Department of Life Sciences, Graduate School of Arts and Sciences, The University of Tokyo, Tokyo, 153-8902, Japan.

Sex differences in behavior allow animals to effectively mate and reproduce. However, the mechanism by which biological sex regulates behavioral states, which underlie the regulation of sex-shared behaviors such as locomotion, is largely unknown. In this study, we studied sex differences in the behavioral states of and found that males spend less time in a low locomotor activity state than hermaphrodites and that dopamine generates this sex difference. Read More

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http://www.jneurosci.org/lookup/doi/10.1523/JNEUROSCI.2985-1
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http://dx.doi.org/10.1523/JNEUROSCI.2985-18.2019DOI Listing
April 2019
5 Reads

The Role of Next Generation Sequencing in the Differential Diagnosis of Caroli's Syndrome.

Balkan J Med Genet 2018 Dec 31;21(2):49-53. Epub 2018 Dec 31.

Clinical Institute of Medical Genetics, University Medical Centre Ljubljana, Ljubljana, Slovenia.

We report the case of a 41-year-old man on conservative treatment for more than 20 years for chronic renal insufficiency, chronic hepatitis and recurrent cholangitis. Following lengthy and extensive diagnostics, the differential diagnosis included primary sclerosing cholangitis and Caroli's disease (CD). To solve the diagnostic challenge, next generation sequencing (NGS) was performed to distinguish between the disorders possibly present in the patient. Read More

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http://dx.doi.org/10.2478/bjmg-2018-0027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454240PMC
December 2018
1 Read

[Psychological Assessment of a sample of women with ADPKD: quality of life, body image, anxiety and depression].

G Ital Nefrol 2019 Apr;36(2)

Primario Servizio di Psicologia Clinica e della Salute, IRCCS Ospedale San Raffaele, Milano, Italia; Università Vita Salute San Raffale, Milano, Italia.

Introduction: The Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a chronic renal disease that has not yet been the subject of psychological research. There are only a few studies related to the consequences and complications of this pathology on female patients, although women affected by this disease present serious problems.

Aim: The purpose of this study is to perform a psychological assessment (quality of life, anxiety, depression, body image) on a sample of 37 women with ADPKD. Read More

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[F-FDG positron emission tomography in non-oncological renal pathology: Current indications and perspectives].

Nephrol Ther 2019 Apr 11. Epub 2019 Apr 11.

Service de néphrologie, centre hospitalier universitaire de Liège, avenue Hippocrate 13, 4000 Liège, Belgique; Groupe interdisciplinaire de géno-protéomique appliquée (GIGA), sciences cardiovasculaires, université de Liège, Liège, Belgique. Electronic address:

Positron emission tomography combined with computed tomography (PET/CT) is a nuclear imaging technique which provides anatomical and functional information. PET/CT is increasingly used in non-oncological nephrology since conventional radiological approaches after injection of contrast agents are relatively contra-indicated in patients with chronic kidney disease (CKD). PET/CT after i. Read More

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http://dx.doi.org/10.1016/j.nephro.2018.11.007DOI Listing

[In-hospital management of acute complicated urinary tract infections].

Nephrol Ther 2019 Apr;15 Suppl 1:S27-S32

Unité de bactériologie, groupe hospitalier Henri-Mondor, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil cedex, France; Université Paris-Est-Créteil-Val-de-Marne, avenue du Général-de-Gaulle, 94010 Créteil cedex, France.

Upper tract urinary tract infections that require hospitalization have been the focus of national recommendations in 2018 by the French society of infectious diseases (Spilf). We here propose to discuss several complex-challenging situations: severe infection with sepsis, pyelonephritis in the pregnant woman, management of infections involving multiresistant bacteria and infection in polycystic kidney disease. Read More

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http://dx.doi.org/10.1016/j.nephro.2019.02.002DOI Listing

Caffeine Accelerates Cystic Kidney Disease in a Pkd1-Deficient Mouse Model.

Cell Physiol Biochem 2019 ;52(5):1061-1074

Nephrology Division, Universidade Federal de São Paulo, São Paulo, Brazil.

Background/aims: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive cyst formation and growth, leading to end-stage renal disease. A higher kidney volume is predictive of a more accelerated decline in renal function. This study aimed to examine the effects of caffeine, a phosphodiesterase inhibitor, on the progression of cystic kidney disease in a mouse model orthologous to human disease (Pkd1:Nestin). Read More

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http://dx.doi.org/10.33594/000000072DOI Listing
January 2019
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Clinical significance of incidentally discovered renal cysts in pediatric patients.

Abdom Radiol (NY) 2019 Apr 10. Epub 2019 Apr 10.

Department of Radiology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA.

Purpose: To determine the clinical significance of incidentally discovered renal cysts in pediatric patients and identify imaging predictors of autosomal dominant polycystic kidney disease (ADPKD).

Methods: A retrospective search of radiology reports from 2000 to 2016 was performed to identify patients < 18 years old with an imaging exam identifying at least one renal cyst and a ≥ 1-year follow-up renal imaging exam for cyst evaluation and/or diagnosis of ADPKD. Cysts with clear solid mass components were excluded. Read More

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http://dx.doi.org/10.1007/s00261-019-02017-zDOI Listing
April 2019
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Early Urinary Biomarkers in Pediatric Autosomal Dominant Polycystic Kidney Disease (ADPKD): No Evidence in the Interest of Urinary Neutrophil Gelatinase-Associated Lipocalin (uNGAL).

Front Pediatr 2019 22;7:88. Epub 2019 Mar 22.

Pediatric Nephrology Unit, Department of Pediatrics, Children's Hospital, Geneva University Hospital, Geneva, Switzerland.

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is increasingly diagnosed during childhood by the presence of renal cysts in patients with a positive familial history. No curative treatment is available and early detection and diagnosis confronts pediatricians with the lack of early markers to decide whether to introduce renal-protective agents and prevent the progression of renal failure. Neutrophil Gelatinase-Associated Lipocalin (NGAL) is a tubular protein that has been recently proposed as an early biomarker of renal impairment in the ADPKD adult population. Read More

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http://dx.doi.org/10.3389/fped.2019.00088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439434PMC
March 2019
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CT of Kidney Volume in Autosomal Dominant Polycystic Kidney Disease: Accuracy, Reproducibility, and Radiation Dose.

Radiology 2019 Apr 9:181830. Epub 2019 Apr 9.

From the Division of Nephrology (M.U.B., T.W.Y., A.L.), Department of Radiology (C.J.H., H.S.), and Centre for Heart Lung Innovation (D.M.V.), University of British Columbia, #700 - 1380 Burrard St, Vancouver, BC, Canada V6Z 2H3; and British Columbia Provincial Renal Agency, Vancouver, Canada (M.U.B., A.R., A.L.).

Background Total kidney volume (TKV) assessment is valuable in autosomal dominant polycystic kidney disease (ADPKD) but the reference standard method of MRI planimetry requires access to MRI and time-consuming interpretation. Purpose To determine whether accurate TKV measurements comparable to the resource-intensive reference standard of MRI planimetry can be obtained by using alternate methods including dose-reducing CT protocols and time-saving measurement equations. Materials and Methods In this prospective study conducted September 2016 to June 2017, adult participants with ADPKD underwent one MRI and two CT examinations. Read More

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http://dx.doi.org/10.1148/radiol.2019181830DOI Listing
April 2019
2 Reads

Prostatic cyst in autosomal dominant polycystic kidney disease: unusual association.

BMJ Case Rep 2019 Apr 5;12(4). Epub 2019 Apr 5.

Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Prostate cyst, as an extrarenal manifestation in patients with autosomal dominant polycystic kidney disease, although infrequent, nevertheless goes beyond tenuous concomitance and may rarely contribute to recurrent urinary tract infection or outflow obstruction and mostly remains asymptomatic. In this context, we report a case of incidentally detected, an asymptomatic prostatic cyst in a patient of autosomal dominant polycystic kidney disease. Read More

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http://dx.doi.org/10.1136/bcr-2018-228617DOI Listing
April 2019
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ATP release into ADPKD cysts via pannexin-1/P2X7 channels decreases ENaC activity.

Biochem Biophys Res Commun 2019 Apr 2. Epub 2019 Apr 2.

Division of Hypertension and Vascular Research, Henry Ford Health System, Detroit, MI, USA. Electronic address:

Genetic predisposition is necessary for polycystic kidney disease (PKD) initiation, although there are other, incompletely identified downstream processes that are required for cyst growth. Their characterization may provide a unique opportunity for clinical interventions. One of the poorly studied phenomena in PKD is high ATP content in cysts. Read More

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http://dx.doi.org/10.1016/j.bbrc.2019.03.177DOI Listing
April 2019
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Octreotide-LAR in later-stage autosomal dominant polycystic kidney disease (ALADIN 2): A randomized, double-blind, placebo-controlled, multicenter trial.

PLoS Med 2019 Apr 5;16(4):e1002777. Epub 2019 Apr 5.

Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent genetically determined renal disease. In affected patients, renal function may progressively decline up to end-stage renal disease (ESRD), and approximately 10% of those with ESRD are affected by ADPKD. The somatostatin analog octreotide long-acting release (octreotide-LAR) slows renal function deterioration in patients in early stages of the disease. Read More

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http://dx.doi.org/10.1371/journal.pmed.1002777DOI Listing
April 2019
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[Genetic testing and pregnancy outcome of 337 fetuses with urinary system anomalies].

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2019 Apr;36(4):306-309

Maternal and Child Health Care Hospital of Guangxi Zhuang Autonomous Region, Nanning, Guangxi 530003, China. Email:

Objective: To explore the genetic basis and pregnancy outcome of fetuses with urinary system anomalies.

Methods: Ultrasonographic features, genetic testing and pregnancy outcomes of 337 fetuses with urinary system anomalies identified by prenatal ultrasonograhy were collected for analysis.

Results: Ultrasonographic features of the fetuses were mainly characterized by hydronephrosis or hydronephrosis, polycystic kidney disease, and renal dysplasia. Read More

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http://dx.doi.org/10.3760/cma.j.issn.1003-9406.2019.04.004DOI Listing
April 2019
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Structure and function of polycystins: insights into polycystic kidney disease.

Nat Rev Nephrol 2019 Apr 4. Epub 2019 Apr 4.

Université Côte d'Azur, Centre National de la Recherche Scientifique, Institut National de la Santé et de la Recherche Médicale, Institut de Pharmacologie Moléculaire et Cellulaire, Labex ICST, Valbonne, France.

Mutations in the polycystins PC1 or PC2 cause autosomal dominant polycystic kidney disease (ADPKD), which is characterized by the formation of fluid-filled renal cysts that disrupt renal architecture and function, ultimately leading to kidney failure in the majority of patients. Although the genetic basis of ADPKD is now well established, the physiological function of polycystins remains obscure and a matter of intense debate. The structural determination of both the homomeric PC2 and heteromeric PC1-PC2 complexes, as well as the electrophysiological characterization of PC2 in the primary cilium of renal epithelial cells, provided new valuable insights into the mechanisms of ADPKD pathogenesis. Read More

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http://www.nature.com/articles/s41581-019-0143-6
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http://dx.doi.org/10.1038/s41581-019-0143-6DOI Listing
April 2019
5 Reads

Bioelectrical impedance analysis as a nutritional assessment tool in Autosomal Dominant Polycystic Kidney Disease.

PLoS One 2019 4;14(4):e0214912. Epub 2019 Apr 4.

Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.

Objective: Autosomal dominant polycystic kidney disease (ADPKD) patients with massive organomegaly suffer from pressure-related complications including malnutrition. In this study, we analyzed the efficacy of segmental bioelectrical impedance analysis (BIA) for objective and quantitative nutritional assessment in ADPKD patients.

Design And Methods: We conducted a cross-sectional study, to evaluate the clinical utility of segmental BIA for assessing the nutritional status of ADPKD patients. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0214912PLOS
April 2019
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Serum klotho is inversely associated with metabolic syndrome in chronic kidney disease: results from the KNOW-CKD study.

BMC Nephrol 2019 Apr 3;20(1):119. Epub 2019 Apr 3.

Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-Ro, Jongno-Gu, Seoul, 03080, Korea.

Background: Metabolic syndrome (MS) is prevalent in chronic kidney disease (CKD). Klotho, a protein linked to aging, is closely associated with CKD. Each component of MS and klotho has an association. Read More

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http://dx.doi.org/10.1186/s12882-019-1297-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6446407PMC

Hemodialysis product and hip fracture in hemodialysis patients: A nationwide cohort study in Japan.

Ther Apher Dial 2019 Apr 2. Epub 2019 Apr 2.

Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Science, Niigata, Japan.

Some have raised concerns that longer and more frequent hemodialysis would be associated with bone fractures due to excess phosphate removal. We examined the effects of Hemodialysis Product on hip fracture incidence among Japanese hemodialysis patients using registry data of the Japanese Society of Dialysis Therapy. During a one-year study period, 1,411 hip fractures occurred among 135,984 patients. Read More

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http://dx.doi.org/10.1111/1744-9987.12807DOI Listing
April 2019
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Optimal imaging conditions for the diagnosis of pleuroperitoneal communication.

BMJ Case Rep 2019 Mar 31;12(3). Epub 2019 Mar 31.

Department of Internal Medicine, Ashikaga Red Cross Hospital, Ashikaga, Tochigi, Japan.

A 70-year-old woman with end-stage renal disease caused by a polycystic kidney disease developed massive right-sided pleural effusion 10 days after the initiation of peritoneal dialysis (PD). Although pleuroperitoneal communication (PPC) was suspected, computed tomographic peritoneography on usual breath holding did not show leakage. Therefore, we instructed her to strain with maximal breathing, which caused a jet of contrast material to stream from the peritoneal cavity into the right pleural cavity and allowed the identification of the exact site of the diaphragm defect. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22894
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http://dx.doi.org/10.1136/bcr-2018-228940DOI Listing
March 2019
2 Reads

In Vivo Imaging of Small Molecular Weight Peptides for Targeted Renal Drug Delivery: A Study in Normal and Polycystic Kidney Diseased Mice.

J Pharmacol Exp Ther 2019 Apr 1. Epub 2019 Apr 1.

GlaxoSmithKline

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a leading monogenetic cause of end stage renal disease (ESRD) with limited therapeutic repertoire. A targeted drug delivery strategy that directs a small molecule to renal niches around cysts could increase safety margins of agents that slow the progression of ADPKD but are poorly tolerated due to extra-renal toxicity. Herein, we determined whether previously characterized lysine- and glutamic acid-based, megalin-binding peptides can achieve renal specific localization in the Juvenile Cystic Kidney (JCK) mouse model of polycystic kidney disease and if the distribution is altered compared to control mice. Read More

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http://dx.doi.org/10.1124/jpet.119.257022DOI Listing
April 2019
4 Reads

A PKD1L3 splice variant in taste buds is not cleaved at the G protein-coupled receptor proteolytic site.

Biochem Biophys Res Commun 2019 May 27;512(4):812-818. Epub 2019 Mar 27.

Department of Biological Sciences, St. John's University, 8000 Utopia Parkway, Queens, New York, 11439, USA. Electronic address:

Mutations in polycystin proteins PKD1 and TRPP2 lead to autosomal dominant polycystic kidney disease. These two proteins form a receptor-ion channel complex on primary cilia. PKD1 undergoes an autoproteolysis at the N terminal G-protein-coupled receptor proteolytic site (GPS), which is essential for the function of PKD1. Read More

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http://dx.doi.org/10.1016/j.bbrc.2019.03.099DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467730PMC
May 2019
2 Reads

Factors influencing the clinical outcome of preimplantation genetic testing for polycystic kidney disease.

Hum Reprod 2019 Mar 30. Epub 2019 Mar 30.

Centre for Medical Genetics, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, Belgium.

Study Question: What are the factors influencing the success rate for couples undergoing preimplantation genetic testing (PGT) for polycystic kidney disease (PKD)?

Summary Answer: In our study cohort, the live birth delivery rate is significantly associated with female age while the male infertility accompanying autosomal dominant PKD (ADPKD) does not substantially affect the clinical outcome.

What Is Known Already: While women with ADPKD have no specific fertility problems, male ADPKD patients may present with reproductive system abnormalities and infertility.

Study Design, Size, Duration: This retrospective cohort study involves 91 PGT cycles for PKD for 43 couples (33 couples for PKD1, 2 couples for PKD2 and 8 couples for autosomal recessive PKD (ARPKD)) from January 2005 until December 2016 with follow-up of transfers until end of 2017. Read More

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http://dx.doi.org/10.1093/humrep/dez027DOI Listing
March 2019
1 Read

Long-term trajectory of kidney function in autosomal-dominant polycystic kidney disease.

Kidney Int 2019 Mar 4. Epub 2019 Mar 4.

Legacy Good Samaritan Hospital, Portland, Oregon, USA.

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by cyst and kidney growth, which is hypothesized to cause loss of functioning renal mass and eventually end-stage kidney disease. However, the time course of decline in glomerular filtration rate (GFR) is poorly defined. The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease study is a 14-year observational cohort study of 241 adults with ADPKD. Read More

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http://dx.doi.org/10.1016/j.kint.2018.12.023DOI Listing
March 2019
1 Read

Novel increasing dose regimen of tolvaptan for autosomal dominant polycystic kidney disease in patient with low tolerability.

Nephrology (Carlton) 2018 Aug;23(8):798-799

Department of Metabolism, Endocrinology, and Molecular Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan.

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http://dx.doi.org/10.1111/nep.13196DOI Listing
August 2018
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Spinal Anesthesia for Renal Transplantation in Lung Resected Patient: A Case Report.

Tanaffos 2018 Mar;17(3):207-210

Department of Anesthesiology, Loghman Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Renal transplantation is among the definitive therapies for treatment of patients with "End-Stage Renal Disease" (ESRD). Proper anesthesia should be considered in patients who undergo renal transplantation. On the other hand, anesthesia in patients with single lung is an ever challenging issue. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6428382PMC
March 2018
1 Read

Author Correction: Hydrophobic pore gates regulate ion permeation in polycystic kidney disease 2 and 2L1 channels.

Nat Commun 2019 03 26;10(1):1452. Epub 2019 Mar 26.

National "111" Center for Cellular Regulation and Molecular Pharmaceutics, Hubei University of Technology, Wuhan, Hubei, 430068, China.

The original version of this Article contained an error in the spelling of the author David Bulkley, which was incorrectly given as David Bulkey. This has now been corrected in both the PDF and HTML versions of the Article. Read More

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http://dx.doi.org/10.1038/s41467-019-09422-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6435729PMC
March 2019
2 Reads

Oxalosis Associated With High-Dose Vitamin C Ingestion in a Peritoneal Dialysis Patient.

Am J Kidney Dis 2019 Mar 22. Epub 2019 Mar 22.

Division of Nephrology and Hypertension; Mayo Clinic; The Rare Kidney Stone Consortium; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN. Electronic address:

We report a case of systemic oxalosis involving the eyes and joints due to long-term use of high-dose vitamin C in a patient receiving maintenance peritoneal dialysis (PD). This 76-year-old woman with autosomal dominant polycystic kidney disease underwent living unrelated kidney transplantation 10 years earlier. The transplant failed 6 months before presentation, and she initiated hemodialysis therapy before transitioning to PD therapy 4 months later. Read More

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http://dx.doi.org/10.1053/j.ajkd.2019.01.022DOI Listing
March 2019
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Urinary angiotensinogen level is associated with potassium homeostasis and clinical outcome in patients with polycystic kidney disease: a prospective cohort study.

BMC Nephrol 2019 Mar 25;20(1):104. Epub 2019 Mar 25.

Department of Internal Medicine, College of Medicine, Institute of Kidney Disease Research, Yonsei University, Seoul, 03722, Republic of Korea.

Background: Guidelines for general hypertension treatment do not recommend the combined use of renin-angiotensin-aldosterone system (RAAS) inhibitors due to the risk of hyperkalemia. However, a recent clinical trial showed that polycystic kidney disease (PKD) patients had infrequent episodes of hyperkalemia despite receiving combined RAAS inhibitors. Because intrarenal RAAS is a main component for renal potassium handling, we further investigated the association between intrarenal RAAS activity and serum potassium level in patients with chronic kidney disease, particularly in PKD patients, and examined whether intrarenal RAAS activity has a prognostic role in patients with PKD. Read More

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https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882
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http://dx.doi.org/10.1186/s12882-019-1292-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6434770PMC
March 2019
5 Reads

Ultrasound Elastography to Quantify Liver Disease Severity in Autosomal Recessive Polycystic Kidney Disease.

J Pediatr 2019 Mar 19. Epub 2019 Mar 19.

Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA; Department of Radiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.

Objectives: To evaluate the diagnostic accuracy of ultrasound elastography with acoustic radiation force impulse (ARFI) to detect congenital hepatic fibrosis and portal hypertension in children with autosomal recessive polycystic kidney disease (ARPKD).

Study Design: Cross-sectional study of 25 children with ARPKD and 24 healthy controls. Ultrasound ARFI elastography (Acuson S3000, Siemens Medical Solutions USA, Inc, Malvern, Pennsylvania) was performed to measure shear wave speed (SWS) in the right and left liver lobes and the spleen. Read More

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http://dx.doi.org/10.1016/j.jpeds.2019.01.055DOI Listing
March 2019
2 Reads

Long-term Tolvaptan Treatment of Autosomal Dominant Polycystic Kidney Disease in Korea.

Electrolyte Blood Press 2018 Dec 31;16(2):23-26. Epub 2018 Dec 31.

Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.

A 22-year-old male patient was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). He received conservative treatment with an angiotensin-converting enzyme inhibitor. Two years later, oral therapy, consisting of 60 mg tolvaptan per day, was initiated. Read More

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https://synapse.koreamed.org/DOIx.php?id=10.5049/EBP.2018.16
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http://dx.doi.org/10.5049/EBP.2018.16.2.23DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414314PMC
December 2018
6 Reads

Loss of Fibrocystin Promotes Interleukin-8-Dependent Proliferation and CTGF Production of Biliary Epithelium.

J Hepatol 2019 Mar 15. Epub 2019 Mar 15.

Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University, Tokyo, Japan. Electronic address:

Background & Aims: Congenital hepatic fibrosis (CHF) is a genetic liver disease with abnormal proliferation of cholangiocyte and progressive hepatic fibrosis. Mutation of polycystic kidney and hepatic disease 1 (PKHD1) causes CHF and the dysfunction of its coding protein, fibrocystin, is associated with ductal plate malformation during fetal development and further pathological changes in CHF patients. However, the underlying molecular mechanism of CHF remains unclear, which is quite different from liver cirrhosis. Read More

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http://dx.doi.org/10.1016/j.jhep.2019.02.024DOI Listing
March 2019
8 Reads

Plasma copeptin levels predict disease progression and tolvaptan efficacy in autosomal dominant polycystic kidney disease.

Kidney Int 2019 Mar 9. Epub 2019 Mar 9.

Institute of Physiology, University of Zurich, Zurich, Switzerland; and Division of Nephrology, Université Catholique de Louvain, Brussels, Belgium.

In the TEMPO 3:4 Trial, treatment with tolvaptan, a vasopressin V2 receptor antagonist, slowed the increase in total kidney volume and decline in estimated glomerular filtration rate (eGFR) in autosomal dominant polycystic kidney disease (ADPKD). We investigated whether plasma copeptin levels, a marker of plasma vasopressin, are associated with disease progression, and whether pre-treatment copeptin and treatment-induced change in copeptin are associated with tolvaptan treatment efficacy. This post hoc analysis included 1,280 TEMPO 3:4 participants (aged 18-50 years, estimated creatinine clearance ≥60 ml/min and total kidney volume ≥750 mL) who had plasma samples available at baseline for measurement of copeptin using an automated immunofluorescence assay. Read More

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http://dx.doi.org/10.1016/j.kint.2018.11.044DOI Listing
March 2019
3 Reads

Refractory ascites and graft dysfunction in early renal transplantation.

J Bras Nefrol 2019 Mar 18. Epub 2019 Mar 18.

Serviço de Nefrologia e Transplantação Renal do Centro Hospitalar do Porto, Porto, Portugal.

The occurrence of ascites after Renal Transplant (RT) is infrequent, and may be a consequence of surgical or medical complications. Case report: 61 year-old, male, history of arterial hypertension, tongue carcinoma and alcoholic habits 12-20g/day. He had chronic kidney disease secondary to autosomal dominant polycystic kidney disease, without hepatic polycystic disease. Read More

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http://dx.doi.org/10.1590/2175-8239-JBN-2018-0175DOI Listing
March 2019
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A role for polycystin-1 and polycystin-2 in neural progenitor cell differentiation.

Cell Mol Life Sci 2019 Mar 20. Epub 2019 Mar 20.

Institute of Molecular and Cellular Anatomy, Ulm University, Albert-Einstein-Allee 11, 89081, Ulm, Germany.

Polycystin-1 (PC1) and polycystin-2 (PC2) are transmembrane proteins encoded by the Pkd1 and Pkd2 genes, respectively. Mutations in these genes are causative for the development of autosomal-dominant polycystic kidney disease. A prominent feature of this disease is an unbalanced cell proliferation. Read More

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http://dx.doi.org/10.1007/s00018-019-03072-xDOI Listing
March 2019
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The novel gene apnoia regulates Drosophila tracheal tube size.

Dev Dyn 2019 Mar 20. Epub 2019 Mar 20.

Department of Biological Sciences, Oakland University, Rochester, Michigan.

Background: Distinct tube size is critical for the function of human tubular organs such as the lung, vascular system, and kidney. Aberrant tube sizes can lead to devastating human illnesses including polycystic kidney disease. The Drosophila trachea provides a premier genetic system to investigate the fundamental mechanisms that regulate tube size. Read More

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http://dx.doi.org/10.1002/dvdy.29DOI Listing
March 2019
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Low-dose 2-deoxyglucose and metformin synergically inhibit proliferation of human polycystic kidney cells by modulating glucose metabolism.

Cell Death Discov 2019 11;5:76. Epub 2019 Mar 11.

Department of Nephrology, Chinese PLA General Hospital, Chinese PLA Institute of Nephrology, State Key Laboratory of Kidney Diseases, National Clinical Research Center for Kidney Diseases, Beijing Key Laboratory of Kidney Diseases, Beijing, 100853 China.

Polycystic kidney disease (PKD) is a common hereditary kidney disease with abnormal proliferation and apoptosis of kidney cystic epithelial cells, eventually leading to chronic renal failure. Currently, there are no effective treatment methods. Similar to tumor cells, cystic epithelial cells have abnormal glycolysis and over-activation of proliferation signaling pathways. Read More

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http://dx.doi.org/10.1038/s41420-019-0156-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6411866PMC
March 2019
4 Reads

[Liver transplantation for polycystic liver disease: 11 cases report and literature review].

Zhonghua Yi Xue Za Zhi 2019 Mar;99(10):767-770

Liver Transplantation Center, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, China.

To explore the indications and safety of orthopedic liver transplantation for polycystic liver disease (PLD). Data of 11 patients with PLD who underwent orthotopic liver transplantation between 2004 and 2013 was retrospectively analyzed. Demographic, clinical and follow-up data were collected for statistical analysis. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0376-2491.2019.10.012DOI Listing
March 2019
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The TRPP2-dependent channel of renal primary cilia also requires TRPM3.

PLoS One 2019 18;14(3):e0214053. Epub 2019 Mar 18.

Department of Pharmacology and Systems Physiology, University of Cincinnati, Cincinnati, Ohio, United States of America.

Primary cilia of renal epithelial cells express several members of the transient receptor potential (TRP) class of cation-conducting channel, including TRPC1, TRPM3, TRPM4, TRPP2, and TRPV4. Some cases of autosomal dominant polycystic kidney disease (ADPKD) are caused by defects in TRPP2 (also called polycystin-2, PC2, or PKD2). A large-conductance, TRPP2-dependent channel in renal cilia has been well described, but it is not known whether this channel includes any other protein subunits. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0214053PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6422334PMC
March 2019
2 Reads

Caroli Disease Revisited: A Case of a Kidney Transplant Patient With Autosomal Polycystic Kidney Disease and Recurrent Episodes of Cholangitis.

Transplant Proc 2019 Mar 3;51(2):541-544. Epub 2019 Jan 3.

Division of Nephrology and Transplantation, Department of Medicine, University of Vermont Medical Center and Larner College of Medicine, Burlington, Vermont, USA. Electronic address:

Polycystic kidney disease (PKD) is a genetic disorder leading to end-stage renal disease more commonly in the fourth to sixth decades of life. Cyst formation in the kidneys and other organs such as the liver and pancreas is the main characteristic of this disease. A significant number of patients with PKD undergo kidney transplantation and receive significant immunosuppression, predisposing them to comorbidities such as infections and malignancies. Read More

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http://dx.doi.org/10.1016/j.transproceed.2018.12.025DOI Listing
March 2019
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Low HDL cholesterol as a predictor of chronic kidney disease progression: a cross-classification approach and matched cohort analysis.

Heart Vessels 2019 Mar 14. Epub 2019 Mar 14.

Department of Medicine, Kidney Center, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Emerging epidemiological evidence indicates that low serum high-density lipoprotein cholesterol (HDL-C) levels are associated with the risk of progression of chronic kidney disease (CKD). However, the differences in the influence of serum HDL-C levels on CKD progression in different subcohorts have rarely been examined in detail in previous studies. The aim of this study was to investigate the significance of low serum HDL-C levels as a predictor of disease progression in CKD patients according to sub-analyses using a cross-classified subcohort. Read More

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http://dx.doi.org/10.1007/s00380-019-01375-4DOI Listing
March 2019
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Renal expression of JAK2 is high in polycystic kidney disease and its inhibition reduces cystogenesis.

Sci Rep 2019 Mar 14;9(1):4491. Epub 2019 Mar 14.

Academic Nephrology Unit, Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, S10 2RX, United Kingdom.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common renal genetic disorder, however it still lacks a cure. The discovery of new therapies heavily depends on understanding key signalling pathways that lead to ADPKD. The JAnus Kinase and Signal Transducers and Activators of Transcription (JAK/STAT) pathway is aberrantly activated and contributes to ADPKD pathogenesis via enhancing epithelial proliferation. Read More

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http://www.nature.com/articles/s41598-019-41106-3
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http://dx.doi.org/10.1038/s41598-019-41106-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6418191PMC
March 2019
4 Reads

Increased salt intake does not worsen the progression of renal cystic disease in high water-loaded PCK rats.

PLoS One 2019 14;14(3):e0207461. Epub 2019 Mar 14.

Department of Clinical Nutrition, Faculty of Health Science, Suzuka University of Medical Science, Suzuka, Mie, Japan.

The anti-diuretic hormone arginine vasopressin is thought to be a detrimental factor in polycystic kidney disease (PKD). We previously reported that high water intake (HWI) reduced urine osmolality and urinary arginine vasopressin, improved renal function, and reduced the kidney/body weight ratio in PCK rats, an orthologous model of human PKD. In PKD patients, however, it is reported that HWI increases total kidney volume, urine volume, and urine sodium excretion, which could be a consequence of high salt intake. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0207461PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6417693PMC
March 2019
2 Reads

[Polycystic kidney disease: 10 key messages].

Authors:
Éric Thervet

Rev Prat 2018 May;68(5):570

Service de néphrologie, département hypertension artérielle, prévention et prise en charge des affections rénales et cardiovasculaires, Hôpital européen Georges-Pompidou, Assistance publique-Hôpitaux de Paris, université Paris-Descartes Paris, France.

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May 2018
1 Read

[Management of a patient with polycystic kidney disease].

Authors:
Dominique Joly

Rev Prat 2018 May;68(5):564-569

Service de néphrologie, hôpital Necker-Enfants malades, AP-HP, Paris, France.

Management of a patient with polycystic kidney disease. In patients with autosomal dominant polycystic kidney disease, the nephrologist is typically asked to treat hypertension, urological complications (pain, stones, infections), progressive renal failure and its consequences, and finally initiate renal replacement therapy (hemodialysis, peritoneal dialysis, kidney transplant) for those reaching end-stage renal failure, most often in the second part of their life. We should now consider a more modern vision: in at risk subjects, an early diagnosis (around 20 years of age) will help to provide dietary advice (water intake, salt and protein intake), detect hypertension, fight against cardiovascular risk factors, detect intracranial aneurysms; in women, to give advice on contraception, pregnancies, and to detect massive polycystic liver disease; finally, to discuss advances in cystic blocking research and to propose the first of these, tolvaptan, to eligible patients. Read More

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May 2018
6 Reads

[Polycystic kidney disease: diagnosis and progressive profile, prognostic elements].

Authors:
Yannick Le Meur

Rev Prat 2018 May;68(5):559-563

Service de néphrologie, CHU La Cavale blanche, université de Brest, Brest, France.

Polycystic kidney disease: diagnosis, progressive profile, prognostic elements. Autosomal dominant polycystic kidney disease can lead to end-stage renal disease but is also complicated by infectious, digestive, cardiovascular and hepatic problems. The diagnosis is easy in the presence of a family history, but its exclusion is sometimes difficult. Read More

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[Polycystic kidney disease].

Rev Prat 2018 May;68(5):556-558

Service de néphrologie, hémodialyse et transplantation rénale, hôpital la Cavale Blanche, CHRU de Brest, Brest, France.

Mechanisms and genetics in polycystic kidney disease. Mutations of PKD1 or PKD2 are identified in more than 90% of the patients affected by Autosomal Dominant Polycystic Kidney Disease (ADPKD). The severity of the renal disease is strongly influenced by the gene involved and the mutation type; the combination of genetic and clinical factors allows stratifying the risk to develop end-stage renal disease. Read More

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May 2018
3 Reads

Unexpected activities in regulating ciliation contribute to off-target effects of targeted drugs.

Clin Cancer Res 2019 Mar 13. Epub 2019 Mar 13.

Molecular Therapeutics, Fox Chase Cancer Center

Purpose: For many tumors, signaling exchanges between cancer cells and other cells in their microenvironment influence overall tumor signaling. Some of these exchanges depend on expression of the primary cilium on non-transformed cell populations, as extracellular ligands including Sonic Hedgehog (SHH), PDGFRa, and others function through receptors spatially localized to cilia. Cell ciliation is regulated by proteins that are themselves therapeutic targets. Read More

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http://dx.doi.org/10.1158/1078-0432.CCR-18-3535DOI Listing
March 2019
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Ciliotherapy: Remote Control of Primary Cilia Movement and Function by Magnetic Nanoparticles.

ACS Nano 2019 Mar 15;13(3):3555-3572. Epub 2019 Mar 15.

Department of Biomedical & Pharmaceutical Sciences, Chapman University School of Pharmacy (CUSP), Harry and Diane Rinker Health Science Campus , Chapman University , Irvine , California 92618 , United States.

Patients with polycystic kidney disease (PKD) are characterized with uncontrolled hypertension. Hypertension in PKD is a ciliopathy, an abnormal function and/or structure of primary cilia. Primary cilia are cellular organelles with chemo and mechanosensory roles. Read More

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http://dx.doi.org/10.1021/acsnano.9b00033DOI Listing
March 2019
4 Reads