Autosomal-dominant polycystic kidney disease (ADPKD) is the most prevalent inherited renal disease, characterized by multiple cysts that can eventually lead to kidney failure. Studies investigating the role of primary cilia and polycystins have significantly advanced our understanding of the pathogenesis of PKD. This review will present clinical and basic aspects of ADPKD, review current concepts of PKD pathogenesis, evaluate potential therapeutic targets, and highlight challenges for future clinical studies.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4422974 | PMC |
| http://dx.doi.org/10.1152/physiol.00032.2014 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Tolvaptan and Autosomal Dominant Polycystic Kidney Disease Progression in Individuals Aged 18-35 Years: A Pooled Database Analysis.
Kidney Med
January 2025
Otsuka Pharmaceutical Development & Commercialization, Inc, Rockville, MD.
Rational & Objective: Data are limited regarding the long-term efficacy of tolvaptan in adults aged 18-35 years with autosomal dominant polycystic kidney disease (ADPKD) at increased risk of rapid progression. We assessed the effects of tolvaptan within a larger population of younger adults and over longer follow-up than individual clinical trials could provide.
Study Design: Pooled database study.
Gut microbiota and kidney function in autosomal dominant polycystic kidney disease participants in Cameroon: a cross-sectional study.
BMC Nephrol
January 2025
Faculty of Medicine and Biomedical Sciences, University of Yaoundé 1, Yaoundé, Cameroon.
Background And Hypothesis: Gut dysbiosis characterized by an imbalance in pathobionts (Enterobacter, Escherichia and Salmonella) and symbionts (Bifidobacterium, Lactobacillus and Prevotella) can occur during chronic kidney disease (CKD) progression. We evaluated the associations between representative symbionts (Bifidobacterium and Lactobacillus) and pathobionts (Enterobacteriaceae) with kidney function in persons with autosomal dominant polycystic kidney disease (ADPKD).
Methods: In this cross-sectional study, 29 ADPKD patients were matched to 15 controls at a 2:1 ratio.
Genetic Assessment of Living Kidney Transplant Donors: A Survey of Canadian Practices.
Can J Kidney Health Dis
January 2025
Multiorgan Transplant Program, Division of Nephrology, Department of Medicine, McGill University Health Centre, Montreal, QC, Canada.
Background: Kidney failure is a prevalent condition with tendency for familial clustering in up to 27% of the affected individuals. Living kidney donor (LKD) transplantation is the optimal treatment option; however, in Canada, more than 45% of LKDs are biologically related to their recipients which subjects recipients to worse graft survival and donors to higher future risk of kidney failure. Although not fully understood, this observation could be partially explained by genetic predisposition to kidney diseases.
View Article and Find Full Text PDFGene therapy in polycystic kidney disease: A promising future.
J Transl Int Med
December 2024
Division of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University (Naval Medical University), Shanghai 200003, China.
Polycystic kidney disease (PKD) is a genetic disorder marked by numerous cysts in the kidneys, progressively impairing renal function. It is classified into autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD), with ADPKD being more common. Current treatments mainly focus on symptom relief and slowing disease progression, without offering a cure.
View Article and Find Full Text PDFTherapeutic Potential of Ketogenic Interventions for Autosomal-Dominant Polycystic Kidney Disease: A Systematic Review.
Nutrients
December 2024
Centre for Diabetes, Obesity and Endocrinology Research (CDOER), Westmead Institute for Medical Research, Westmead, Sydney, NSW 2145, Australia.
Background: Recent findings have highlighted that abnormal energy metabolism is a key feature of autosomal-dominant polycystic kidney disease (ADPKD). Emerging evidence suggests that nutritional ketosis could offer therapeutic benefits, including potentially slowing or even reversing disease progression. This systematic review aims to synthesise the literature on ketogenic interventions to evaluate the impact in ADPKD.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!