All Stories

  1. HYDROchlorothiazide versus placebo to PROTECT polycystic kidney disease patients and improve their quality of life: study protocol and rationale for the HYDRO-PROTECT randomized controlled trial
  2. An Artificial Intelligence Generated Automated Algorithm to Measure Total Kidney Volume in ADPKD
  3. Combining genotype with height-adjusted kidney length predicts rapid progression of ADPKD
  4. Macrophage subpopulation identity in Drosophila is modulated by apoptotic cell clearance and related signalling pathways
  5. Liver transplant recipients with polycystic liver disease have longer waiting times but better long-term clinical outcomes than those with liver disease due to other causes: A retrospective cross-sectional study
  6. Rare disease gene association discovery from burden analysis of the 100,000 Genomes Project data
  7. Macrophage subpopulation identity inDrosophilais modulated by apoptotic cell clearance and related signalling pathways
  8. Identification of renal cyst cells of type I Nephronophthisis by single-nucleus RNA sequencing
  9. The Common PKD1 p.(Ile3167Phe) Variant Is Hypomorphic and Associated with Very Early Onset, Biallelic Polycystic Kidney Disease
  10. Barriers and facilitators to the implementation of guidelines in rare diseases: a systematic review
  11. OVERTURE: A Worldwide, Prospective, Observational Study of Disease Characteristics in Patients With ADPKD
  12. Patient Perspectives on ADPKD
  13. Venglustat, a Novel Glucosylceramide Synthase Inhibitor, in Patients at Risk of Rapidly Progressing ADPKD: Primary Results of a Double-Blind, Placebo-Controlled, Phase 2/3 Randomized Clinical Trial
  14. Hypertension in young adults with autosomal dominant polycystic kidney disease: a case for early screening?
  15. The association of urinary epidermal growth factors with ADPKD disease severity and progression
  16. Diet and Polycystic Kidney Disease: Nutrients, Foods, Dietary Patterns, and Implications for Practice
  17. WCN23-0391 THE CLINICAL CHARACTERISTICS AND KIDNEY OUTCOME IN CHINESE PATIENTS WITH TYPICAL AND ATYPICAL POLYCYSTIC KIDNEY DISEASE
  18. Chronic kidney disease as cardiovascular risk factor in routine clinical practice: a position statement by the Council of the European Renal Association
  19. Chronic kidney disease as cardiovascular risk factor in routine clinical practice: a position statement by the Council of the European Renal Association
  20. The STAGED-PKD 2-Stage Adaptive Study With a Patient Enrichment Strategy and Treatment Effect Modeling for Improved Study Design Efficiency in Patients With ADPKD
  21. P73 Patients with polycystic liver disease have longer waiting times but better clinical outcomes following liver transplantation in the UK
  22. A founderUMODvariant is a common cause of hereditary nephropathy in the British population
  23. Regional variation in tolvaptan prescribing across England: national data and retrospective evaluation from an expert centre
  24. Chronic kidney disease as cardiovascular risk factor in routine clinical practice: a position statement by the Council of the European Renal Association
  25. Patients with polcystic liver disease have longer waiting times but better clinical outcomes following liver transplantation in the UK
  26. Barriers and facilitators to the implementation of guidelines in rare diseases: A systematic review
  27. Flank pain has a significant adverse impact on quality of life in ADPKD: the CYSTic-QoL study
  28. Research priorities for autosomal dominant polycystic kidney disease: a UK priority setting partnership
  29. An update on the use of tolvaptan for autosomal dominant polycystic kidney disease: consensus statement on behalf of the ERA Working Group on Inherited Kidney Disorders, the European Rare Kidney Disease Reference Network and Polycystic Kidney Disease I...
  30. FC029: A Multivariate Model Identifies Genotype, Hypertension and Kidney Length as Independent Baseline Predictors of Disease Progression in a Longitudinal Autosomal Dominant Polycystic Kidney Disease Patient Cohort
  31. MO012: Development of an Accurate Automated Segmentation Algorithm to Measure Total Kidney Volume in ADPKD Suitable for Clinical Application (The Cystvas Study)
  32. MO015: The Interaction of Gender and Genotype in the Development of Polycystic Liver Disease in ADPKD
  33. MO030: Familial clustering of a rare UMOD variant in undiagnosed hereditary nephropathy suggests the presence of a common ancestral founder mutation
  34. Can ketogenic dietary interventions slow disease progression in ADPKD: what we know and what we don't
  35. From WEDA to EDTA to ERA: 60 years of supporting European nephrology and counting
  36. Individualized everolimus treatment for tuberous sclerosis-related angiomyolipoma promotes treatment adherence and response
  37. Monoallelic IFT140 pathogenic variants are an important cause of the autosomal dominant polycystic kidney-spectrum phenotype
  38. Metformin induces lactate accumulation and accelerates renal cyst progression in Pkd1-deficient mice
  39. An update on the use of tolvaptan for autosomal dominant polycystic kidney disease: consensus statement on behalf of the ERA Working Group on Inherited Kidney Disorders, the European Rare Kidney Disease Reference Network and Polycystic Kidney Disease I...
  40. TAMEing ADPKD with metformin: safe and effective?
  41. An Nphp1 knockout mouse model targeting exon 2–20 demonstrates characteristic phenotypes of human nephronophthisis
  42. MO023FLANK PAIN HAS A MAJOR NEGATIVE IMPACT ON HEALTH-RELATED QUALITY OF LIFE IN ADPKD: THE CYSTIC I STUDY
  43. Biallelic inheritance of hypomorphic PKD1 variants is highly prevalent in very early onset polycystic kidney disease
  44. Drug repurposing in autosomal dominant polycystic kidney disease: back to the future with pioglitazone
  45. Establishing a Core Outcome Set for Autosomal Dominant Polycystic Kidney Disease: Report of the Standardized Outcomes in Nephrology–Polycystic Kidney Disease (SONG-PKD) Consensus Workshop
  46. An international cohort study of autosomal dominant tubulointerstitial kidney disease due to mutations identifies distinct clinical subtypes
  47. Coronavirus-associated kidney outcomes in COVID-19, SARS, and MERS: a meta-analysis and systematic review
  48. The Controversial Role of Fibrosis in Autosomal Dominant Polycystic Kidney Disease
  49. ‘A sword of Damocles’: patient and caregiver beliefs, attitudes and perspectives on presymptomatic testing for autosomal dominant polycystic kidney disease: a focus group study
  50. Core Outcome Domains for Trials in Autosomal Dominant Polycystic Kidney Disease: An International Delphi Survey
  51. Polycystin-1 regulates ARHGAP35-dependent centrosomal RhoA activation and ROCK signaling
  52. Clinical spectrum, prognosis and estimated prevalence of DNAJB11-kidney disease
  53. Global microRNA profiling in human urinary exosomes reveals novel disease biomarkers and cellular pathways for autosomal dominant polycystic kidney disease
  54. Post-translational modifications of the polycystin proteins
  55. Range and Variability of Outcomes Reported in Randomized Trials Conducted in Patients With Polycystic Kidney Disease: A Systematic Review
  56. The positive effect of selective prostaglandin E2 receptor EP2 and EP4 blockade on cystogenesis in vitro is counteracted by increased kidney inflammation in vivo
  57. Publisher Correction: Whole-genome sequencing of a sporadic primary immunodeficiency cohort
  58. Cellular signaling in PKD: foreword
  59. SO092REDUCED QUALITY OF LIFE IN ADPKD PATIENTS WITH CKD STAGE 1-3: THE CYSTIC I QUALITY OF LIFE STUDY
  60. Whole-genome sequencing of a sporadic primary immunodeficiency cohort
  61. Ankhd1 enhances polycystic kidney disease development via promoting proliferation and fibrosis
  62. SAT-442 Identification of Exosome MicroRNAs as Novel Biomarkers for Rapid Disease Progression in Autosomal Dominant Polycystic Kidney Disease
  63. Detection and characterization of mosaicism in autosomal dominant polycystic kidney disease
  64. A high throughput zebrafish chemical screen reveals ALK5 and non-canonical androgen signalling as modulators of the pkd2−/− phenotype
  65. Long-acting somatostatin analogue treatments in autosomal dominant polycystic kidney disease and polycystic liver disease: a systematic review and meta-analysis
  66. Tuberous Sclerosis Complex (TSC): Expert Recommendations for Provision of Coordinated Care
  67. SAT-100 “A SWORD OF DAMOCLES”: PATIENT AND CAREGIVER BELIEFS, ATTITUDES AND PERSPECTIVES ON GENETIC SCREENING AND TESTING FOR AUTOSOMAL POLYCYSTIC KIDNEY DISEASE - FOCUS GROUP STUDY
  68. SAT-334 GENETIC TESTING OF FAMILIES WITH VERY EARLY ONSET POLYCYSTIC KIDNEY DISEASE REVEALS THE FUNCTIONAL SIGNIFICANCE OF HYPOMORPHIC VARIANTS
  69. Small-molecule allosteric activators of PDE4 long form cyclic AMP phosphodiesterases
  70. FO026GLOBAL MICRORNA PROFILING IN HUMAN URINARY EXOSOMES REVEALS NEW DISEASE BIOMARKERS AND CELLULAR PATHWAYS FOR AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD)
  71. SaO005CLINICAL PRESENTATION AND PROGNOSIS OF DNAJB11-ASSOCIATED NEPHROPATHY: AN INTERNATIONAL COLLABORATIVE STUDY
  72. Renal monocyte chemoattractant protein-1: an emerging universal biomarker and therapeutic target for kidney diseases?
  73. Identifying patient‐important outcomes in polycystic kidney disease: An international nominal group technique study
  74. Imaging of Kidney Cysts and Cystic Kidney Diseases in Children: An International Working Group Consensus Statement
  75. A rapid high-performance semi-automated tool to measure total kidney volume from MRI in autosomal dominant polycystic kidney disease
  76. Implementing core outcomes in kidney disease: report of the Standardized Outcomes in Nephrology (SONG) implementation workshop
  77. Imaging of kidney cysts and cystic kidney diseases in children. Consensus paper by an ad hoc committee
  78. Magnetic resonance imaging biomarkers for chronic kidney disease: a position paper from the European Cooperation in Science and Technology Action PARENCHIMA
  79. Linear and Nonlinear Estimated GFR Slopes in ADPKD Patients Reaching ESRD
  80. A model to predict disease progression in patients with autosomal dominant polycystic kidney disease (ADPKD): the ADPKD Outcomes Model
  81. Tolvaptan slows disease progression in late-stage ADPKD
  82. European ADPKD Forum multidisciplinary position statement on autosomal dominant polycystic kidney disease care
  83. Standardised Outcomes in Nephrology—Polycystic Kidney Disease (SONG-PKD): study protocol for establishing a core outcome set in polycystic kidney disease
  84. Targeting new cellular disease pathways in autosomal dominant polycystic kidney disease
  85. Targeting new cellular disease pathways in autosomal dominant polycystic kidney disease
  86. The Sorting Nexin 3 Retromer Pathway Regulates the Cell Surface Localization and Activity of a Wnt-Activated Polycystin Channel Complex
  87. Making sense of polycystic kidney disease
  88. TO033VERY EARLY-ONSET AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE DUE TO BI-ALLELIC MUTATIONS IN PKD1 AND PKD2
  89. Parallel microarray profiling identifies ErbB4 as a determinant of cyst growth in ADPKD and a prognostic biomarker for disease progression
  90. STAT5 drives abnormal proliferation in autosomal dominant polycystic kidney disease
  91. Development of a rapid semi-automated tool to measure total kidney volume in autosomal dominant polycystic kidney disease
  92. Autosomal dominant polycystic kidney disease: recent advances in clinical management
  93. SO052DEVELOPMENT OF A RAPID SEMI-AUTOMATED TOOL TO MEASURE TOTAL KIDNEY VOLUME IN AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE
  94. SO053SELECTIVE PROSTAGLANDIN E2 RECEPTOR BLOCKADE FOR THE TREATMENT OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE
  95. The Cyclic AMP Signaling Pathway and Direct PKA Phosphorylation Regulate Polycystin-2 (TRPP2) Channel Function
  96. Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA Working Groups on Inherited Kidney Disorders and European Renal Best Practice
  97. New onset diabetes after kidney transplantation in patients with autosomal dominant polycystic kidney disease: systematic review protocol: Figure 1
  98. A polycystin-centric view of cyst formation and disease: the polycystins revisited
  99. Autosomal dominant polycystic kidney disease
  100. Autosomal dominant polycystic kidney disease
  101. The cAMP Signaling Pathway and Direct Protein Kinase A Phosphorylation Regulate Polycystin-2 (TRPP2) Channel Function
  102. The Polycystin-1, Lipoxygenase, and α-Toxin Domain Regulates Polycystin-1 Trafficking
  103. Increased psychosocial risk, depression and reduced quality of life living with autosomal dominant polycystic kidney disease
  104. Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference
  105. Department of Error
  106. Backbone assignment and secondary structure of the PLAT domain of human polycystin-1
  107. Autosomal dominant polycystic kidney disease: the changing face of clinical management
  108. FP064ASSESSING THE LONG TERM OUTCOMES OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD) USING THE ADPKD OUTCOMES MODEL: A UK CASE STUDY
  109. FP364INCREASED PSYCHOSOCIAL BURDEN AND ADVERSE QUALITY OF LIFE IN AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE
  110. Genetic Testing in the Assessment of Living Related Kidney Donors at Risk of Autosomal Dominant Polycystic Kidney Disease
  111. SuO042POLYCYSTIN-1 TRAFFICKING IS REGULATED BY CAMP DEPENDENT PHOSPHORYLATION OF THE PLAT DOMAIN
  112. Endothelin and Tubulointerstitial Renal Disease
  113. Analysis of data from the ERA-EDTA Registry indicates that conventional treatments for chronic kidney disease do not reduce the need for renal replacement therapy in autosomal dominant polycystic kidney disease
  114. Erratum
  115. Development Of A Model To Predict Disease Progression In Autosomal Dominant Polycystic Kidney Disease (ADPKD)
  116. Genetics and Genomics of Chronic Kidney Disease
  117. Building a network of ADPKD reference centres across Europe: the EuroCYST initiative
  118. How simple are 'simple renal cysts'?
  119. Renal replacement therapy for autosomal dominant polycystic kidney disease (ADPKD) in Europe: prevalence and survival--an analysis of data from the ERA-EDTA Registry
  120. Sudden death due to subarachnoid haemorrhage in an infant with autosomal dominant polycystic kidney disease
  121. Rare inherited kidney diseases: challenges, opportunities, and perspectives
  122. RENAL DEVELOPMENT AND CYSTIC DISEASES
  123. Metabolic abnormalities in autosomal dominant polycystic kidney disease
  124. Angiopoietin-1 regulates microvascular reactivity and protects the microcirculation during acute endothelial dysfunction: Role of eNOS and VE-cadherin
  125. TRPP2 in Polycystic Kidney Disease
  126. Polycystin-1 but not polycystin-2 deficiency causes upregulation of the mTOR pathway and can be synergistically targeted with rapamycin and metformin
  127. Cosegregation of Focal Segmental Glomerulosclerosis in a Family with Familial Partial Lipodystrophy due to a Mutation in <b><i>LMNA</i></b>
  128. New treatments for autosomal dominant polycystic kidney disease
  129. Fabrication of Luminescent Monolayered Tungsten Dichalcogenides Quantum Dots with Giant Spin-Valley Coupling
  130. The Role of Phospholipase D in Modulating the MTOR Signaling Pathway in Polycystic Kidney Disease
  131. Primary cilia and renal cysts: does length matter?
  132. Fabrication and Luminescence of Monolayered Boron Nitride Quantum Dots
  133. The natural history of autosomal dominant polycystic kidney disease: 30-year experience from a single centre
  134. Hyperphosphorylation of polycystin-2 at a critical residue in disease reveals an essential role for polycystin-1-regulated dephosphorylation
  135. Pkd2 mesenteric vessels exhibit a primary defect in endothelium-dependent vasodilatation restored by rosiglitazone
  136. Cystic kidney diseases: many ways to form a cyst
  137. The ERA-EDTA Working Group on inherited kidney disorders
  138. Mechanism-Based Therapeutics for Autosomal Dominant Polycystic Kidney Disease: Recent Progress and Future Prospects
  139. 32 PKD2 mutant zebrafish display excessive developmental angiogenesis
  140. Spurious hypophosphatemia associated with monoclonal paraproteinemia
  141. Thiazolidinediones inhibit MDCK cyst growth through disrupting oriented cell division and apicobasal polarity
  142. A Single Amino Acid Residue Constitutes the Third Dimerization Domain Essential for the Assembly and Function of the Tetrameric Polycystin-2 (TRPP2) Channel
  143. What’s new in… Ciliopathies
  144. Endothelin in Polycystic Kidney Disease
  145. Protein Kinase D–mediated Phosphorylation of Polycystin-2 (TRPP2) Is Essential for Its Effects on Cell Growth and Calcium Channel Activity
  146. Towards the Integration of Genetic Knowledge into Clinical Practice
  147. A polycystin-2 (TRPP2) dimerization domain essential for the function of heteromeric polycystin complexes
  148. Structural and Molecular Basis of the Assembly of the TRPP2/PKD1 Complex
  149. Screening for intracranial aneurysms in ADPKD
  150. Pkd2 Dosage Influences Cellular Repair Responses following Ischemia-Reperfusion Injury
  151. Screening for intracranial aneurysms in ADPKD
  152. Structural and molecular basis of the assembly of the TRPP2/PKD1 complex
  153. A novel dephosphorylation‐activated conductance in a mouse renal collecting duct cell line
  154. Peroxisome Proliferator-Activated Receptor Gamma Agonists in Kidney Disease – Future Promise, Present Fears
  155. Autosomal dominant polycystic kidney disease
  156. Homophilic and heterophilic polycystin 1 interactions regulate E-cadherin recruitment and junction assembly in MDCK cells
  157. Homophilic and heterophilic polycystin 1 interactions regulate E-cadherin recruitment and junction assembly in MDCK cells
  158. Identification and Functional Characterization of an N-terminal Oligomerization Domain for Polycystin-2
  159. Activation of TRPP2 through mDia1-dependent voltage gating
  160. Chronic Renal Disease
  161. Autosomal Dominant Polycystic Kidney Disease: Recent Advances in Pathogenesis and Treatment
  162. Hyperproliferation of PKD1 cystic cells is induced by insulin-like growth factor-1 activation of the Ras/Raf signalling system
  163. Involvement of Hypoxia-Inducible Transcription Factors in Polycystic Kidney Disease
  164. Endothelin B Receptor Blockade Accelerates Disease Progression in a Murine Model of Autosomal Dominant Polycystic Kidney Disease
  165. Contributors
  166. Identification of an N-terminal glycogen synthase kinase 3 phosphorylation site which regulates the functional localization of polycystin-2 in vivo and in vitro
  167. Detection of Proximal Tubular Motile Cilia in a Patient With Renal Sarcoidosis Associated With Hypercalcemia
  168. Molecular pathogenesis of ADPKD: The polycystin complex gets complex
  169. Expression and Cellular Localisation of Renal Endothelin-1 and Endothelin Receptor Subtypes in Autosomal-Dominant Polycystic Kidney Disease
  170. Aberrant Polycystin-1 Expression Results in Modification of Activator Protein-1 Activity, whereas Wnt Signaling Remains Unaffected
  171. Functional Analysis of PKD1 Transgenic Lines Reveals a Direct Role for Polycystin-1 in Mediating Cell-Cell Adhesion
  172. Association of mutation position in polycystic kidney disease 1 (PKD1) gene and development of a vascular phenotype
  173. Genetic Renal Abnormalities
  174. Polycystic kidney disease—the ciliary connection
  175. Polycystin-2 expression is increased following experimental ischaemic renal injury
  176. Identification, Characterization, and Localization of a Novel Kidney Polycystin-1-Polycystin-2 Complex
  177. Polycystin Expression in the Kidney and Other Tissues: Complexity, Consensus and Controversy
  178. Endothelin antagonists in renal disease
  179. Polycystin-1 expression in PKD1, early-onset PKD1, and TSC2/PKD1 cystic tissue
  180. Coordinate Expression of the Autosomal Dominant Polycystic Kidney Disease Proteins, Polycystin-2 And Polycystin-1, in Normal and Cystic Tissue
  181. Cyst formation in ADPKD: new insights from natural and targeted mutants
  182. Regional Variations in Endothelin-1 and its Receptor Subtypes in Human Coronary Vasculature: Pathophysiological Implications in Coronary Disease
  183. Molecular basis of renal cyst formation—one hit or two?
  184. A stable, nonsense mutation associated with a case of infantile onset polycystic kidney disease 1 (PKD1)
  185. Polycystin, the polycystic kidney disease 1 protein, is expressed by epithelial cells in fetal, adult, and polycystic kidney.
  186. Candidate 56 and 58 kDa protein(s) responsible for mediating the renal defects in oncogenic hypophosphatemic osteomalacia
  187. Tubulointerstitial actions of endothelins in the kidney: roles in health and disease
  188. Tubulointerstitial actions of endothelins in the kidney: roles in health and disease
  189. Oncogenous hypophosphataemic osteomalacia: effects on phosphate transport and vitamin D metabolism in cultured human kidney cells
  190. Human high density lipoproteins stimulate endothelin-1 release by cultured human renal proximal tubular cells
  191. Tubular lipidosis: Epiphenomenon or pathogenetic lesion in human renal disease?
  192. Loss of glomerular function and tubulointerstitial fibrosis:Cause or effect?
  193. Tubular-Derived Growth Factors and Cytokines in the Pathogenesis of Tubulointerstitial Fibrosis: Implications for Human Renal Disease Progression
  194. Tubular-derived cytokines and human renal disease progression
  195. Mechanisms of tubulo‐interstitiaI injury in progressive renal diseases
  196. Effect of cyclosporin A on endothelin synthesis by cultured human renal cortical epithelial cells
  197. Sinus arrest and asystole due to severe lithium intoxication
  198. The pathogenesis of the ovarian hyperstimulation syndrome (OHS): a possible role for ovarian renin
  199. Atrial natriuretic peptide release responds to atrial stretch and not to atrial pressure: observations during pericardiocentesis in a young woman
  200. The Use of Lithium Clearance in Bartter's Syndrome
  201. Intermittent absorption of warfarin caused by an unrecognized pharyngeal pouch
  202. Effects of noradrenaline infusion on platelet catecholamine levels and platelet aggregation
  203. Hypersensitivity vasculitis complicating intravenous streptokinase therapy in acute myocardial infarction