Correlations between renal function and the total kidney volume measured on imaging for autosomal dominant polycystic kidney disease: A systematic review and meta-analysis

2017 ◽  
Vol 95 ◽  
pp. 56-65 ◽  
Author(s):  
Woo Ri Jo ◽  
Seong Hee Kim ◽  
Kyung Won Kim ◽  
Chong Hyun Suh ◽  
Jeong Kon Kim ◽  
...  
Keyword(s):  
Systematic Review ◽  
Renal Function ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Meta Analysis ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Total Kidney Volume ◽  
Autosomal Dominant Polycystic Kidney

scholarly journals Preservation of kidney function irrelevant of total kidney volume growth rate with tolvaptan treatment in patients with autosomal dominant polycystic kidney disease

2021 ◽  
Author(s):  
Shigeo Horie ◽  
Satoru Muto ◽  
Haruna Kawano ◽  
Tadashi Okada ◽  
Yoshiyuki Shibasaki ◽  
...  
Keyword(s):  
Renal Function ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Japanese Patients ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Total Kidney Volume ◽  
Autosomal Dominant Polycystic Kidney ◽  
Egfr Decline

Abstract Background Tolvaptan slowed the rates of total kidney volume (TKV) growth and renal function decline over a 3-year period in patients with autosomal dominant polycystic kidney disease (ADPKD) enrolled in the Tolvaptan Efficacy and Safety in Management of Autosomal Dominant Polycystic Kidney Disease and Its Outcomes (TEMPO) 3:4 trial (NCT00428948). In this post hoc analysis of Japanese patients from TEMPO 3:4, we evaluated whether the effects of tolvaptan on TKV and on renal function are interrelated. Methods One hundred and forty-seven Japanese patients from TEMPO 3:4 were included in this analysis (placebo, n = 55; tolvaptan, n = 92). Tolvaptan-treated patients were stratified into the responder group (n = 37), defined as tolvaptan-treated patients with a net decrease in TKV from baseline to year 3, and the non-responder group (n = 55), defined as tolvaptan-treated patients with a net increase in TKV. Results Mean changes during follow-up in the placebo, responder, and non-responder groups were 16.99%, − 8.33%, and 13.95%, respectively, for TKV and − 12.61, − 8.47, and − 8.58 mL/min/1.73 m2, respectively, for estimated glomerular filtration rate (eGFR). Compared with the placebo group, eGFR decline was significantly slowed in both the responder and non-responder groups (P < 0.05). Conclusion Tolvaptan was effective in slowing eGFR decline, regardless of TKV response, over 3 years in patients with ADPKD in Japan. Treatment with tolvaptan may have beneficial effects on slowing of renal function decline even in patients who have not experienced a reduction in the rate of TKV growth by treatment with tolvaptan.

scholarly journals Hypertension in autosomal dominant polycystic kidney disease: a meta-analysis

2016 ◽  
Vol 101 (12) ◽  
pp. 1142-1147 ◽  
Author(s):  
Matko Marlais ◽  
Oliver Cuthell ◽  
Dean Langan ◽  
Jan Dudley ◽  
Manish D Sinha ◽  
...  
Keyword(s):  
Systematic Review ◽  
Renal Function ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Meta Analysis ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney ◽  
Hypertension In Children ◽  
Reduced Renal Function

ContextAutosomal dominant polycystic kidney disease (ADPKD) is a common disorder that can cause hypertension during childhood, but the true prevalence of hypertension during childhood is not known.ObjectiveWe undertook a systematic review and meta-analysis to determine the prevalence of hypertension in children with ADPKD.Data sourcesSystematic review of articles published between 1980 and 2015 in MEDLINE and EMBASE.Study selectionStudies selected by two authors independently if reporting data on prevalence of hypertension in children and young persons aged <21 years with a diagnosis of ADPKD. Observational series were included with study populations of >15 children. Articles were excluded if inadequate diagnostic criteria for hypertension were used. Studies with selection bias were included but analysed separately.Data extractionData extracted on prevalence of hypertension, proteinuria and reduced renal function using standardised form. Meta-analysis was performed to calculate weighted mean prevalence.Results903 articles were retrieved from our search; 14 studies met the inclusion criteria: 1 prospective randomised controlled trial; 8 prospective observational studies; and 5 retrospective cross-sectional studies. From 928 children with clinically confirmed ADPKD, 20% (95% CI 15% to 27%) were hypertensive. The estimated prevalence of proteinuria in children with ADPKD is 20% (8 studies; 95% CI 9% to 40%) while reduced renal function occurred in 8% (5 studies; 95% CI 2% to 26%).LimitationsStudies showed a high degree of methodological heterogeneity (I2=73.4%, τ2=0.3408, p<0.0001). Most studies did not use ambulatory blood pressure (BP) monitoring to diagnose hypertension.ConclusionsIn this meta-analysis we estimate 20% of children with ADPKD have hypertension. In the population, many children with ADPKD are not under regular follow-up and remain undiagnosed. We recommend that all children at risk of ADPKD have regular BP measurement.

scholarly journals Effect of tolvaptan in Japanese patients with autosomal dominant polycystic kidney disease: a post hoc analysis of TEMPO 3:4 and TEMPO Extension Japan

2021 ◽  
Author(s):  
Satoru Muto ◽  
Tadashi Okada ◽  
Yoshiyuki Shibasaki ◽  
Tatsuki Ibuki ◽  
Shigeo Horie
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Efficacy Evaluation ◽  
Total Kidney Volume ◽  
Post Hoc Analysis ◽  
Autosomal Dominant Polycystic Kidney ◽  
Post Hoc

Abstract Background Autosomal dominant polycystic kidney disease (ADPKD) is a progressive condition that eventually leads to end-stage renal disease. A phase 3 trial of tolvaptan (TEMPO 3:4; NCT00428948) and its open-label extension (TEMPO Extension Japan: TEMPO-EXTJ; NCT01280721) were conducted in patients with ADPKD. In this post hoc analysis, effects on renal function and the safety profile of tolvaptan were assessed over a long-term period that included the 3-year TEMPO 3:4 and the approximately 3-year TEMPO-EXTJ trials. Methods Patients from Japanese trial sites who completed TEMPO 3:4 were offered participation in TEMPO-EXTJ. Patients whose efficacy parameters were measured at year 2 in TEMPO-EXTJ for efficacy evaluation were included. The annual slope of the estimated glomerular filtration rate (eGFR) and growth in total kidney volume (TKV) were analyzed. Results In patients who received tolvaptan in TEMPO 3:4 and TEMPO-EXTJ, the annual slope of eGFR (mL/min/1.73 m2) was − 3.480 in TEMPO 3:4 and − 3.417 in TEMPO-EXTJ, with no apparent effect of an approximately 3.6-month off-treatment interval between the two trials. In patients who received a placebo in TEMPO 3:4 before initiating tolvaptan in TEMPO-EXTJ, the slope of eGFR was significantly less steep from TEMPO 3:4 (− 4.287) to TEMPO-EXTJ (− 3.364), a difference of 0.923 (P = 0.0441). Conclusion The TEMPO-EXTJ trial supports a sustained beneficial effect of tolvaptan on eGFR. In patients who received a placebo in TEMPO 3:4, initiation of tolvaptan in TEMPO-EXTJ was associated with a significant slowing of eGFR decline.

scholarly journals Effect of Statins on Renal Function and Total Kidney Volume in Autosomal Dominant Polycystic Kidney Disease

2020 ◽  
Vol 6 (6) ◽  
pp. 407-413
Author(s):  
Cheng Xue ◽  
Li-Ming Zhang ◽  
Chenchen Zhou ◽  
Chang-Lin Mei ◽  
Sheng-Qiang Yu
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Lipid Lowering ◽  
Control Group ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Total Kidney Volume ◽  
Yearly Change ◽  
Autosomal Dominant Polycystic Kidney

<b><i>Background:</i></b> Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary nephropathy with few treatments to slow renal progression. The evidence on the effect of lipid-lowering agents (statins) on ADPKD progression remains inconclusive. <b><i>Methods:</i></b> We performed a systematic review and meta-analysis by searching the PubMed, Embase, Web of Science, and Cochrane databases (up to November 2019). Changes in estimated glomerular filtration rate (eGFR) and total kidney volume (TKV) were the primary outcomes. Mean differences (MDs) for continuous outcomes and 95% confidence intervals (CIs) were calculated by a random-effects model. <b><i>Results:</i></b> Five clinical studies with 648 participants were included. Statins did not show significant benefits in the yearly change in eGFR (4 studies, MD = −0.13 mL/min/m<sup>2</sup>, 95% CI: −0.78 to 0.52, <i>p</i> = 0.70) and the yearly change in TKV (3 studies, MD = −1.17%, 95% CI: −3.40 to 1.05, <i>p</i> = 0.30) compared with the control group. However, statins significantly decreased urinary protein excretion (−0.10 g/day, 95% CI: −0.16 to -0.03, <i>p</i> = 0.004) and serum low-density lipoprotein level (−0.34 mmol/L, 95% CI: −0.58 to −0.10, <i>p</i> = 0.006). <b><i>Conclusion:</i></b> Despite these proteinuria and lipid-lowering benefits, the effect of statins on ADPKD progression was uncertain.

scholarly journals Genetic Characteristics of Korean Patients with Autosomal Dominant Polycystic Kidney Disease by Targeted Exome Sequencing

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Hyunsuk Kim ◽  
Hayne Cho Park ◽  
Hyunjin Ryu ◽  
Hyunho Kim ◽  
Hyun-Seob Lee ◽  
...  
Keyword(s):  
Renal Function ◽  
Kidney Disease ◽  
Exome Sequencing ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Genetic Characteristics ◽  
Targeted Exome Sequencing ◽  
Autosomal Dominant Polycystic Kidney

AbstractAutosomal dominant polycystic kidney disease (ADPKD) is one of the main causes of end-stage renal disease (ESRD). Genetic information is of the utmost importance in understanding pathogenesis of ADPKD. Therefore, this study aimed to demonstrate the genetic characteristics of ADPKD and their effects on renal function in 749 Korean ADPKD subjects from 524 unrelated families. Genetic studies of PKD1/2 were performed using targeted exome sequencing combined with Sanger sequencing in exon 1 of the PKD1 gene and a multiple ligation probe assay. The mutation detection rate was 80.7% (423/524 families, 331 mutations) and 70.7% was novel. PKD1 protein-truncating (PKD1-PT) genotype was associated with younger age at diagnosis, larger kidney volume, lower renal function compared to PKD1 non-truncating and PKD2 genotypes. The PKD1 genotype showed earlier onset of ESRD compared to PKD2 genotype (64.9 vs. 72.9 years old, P < 0.001). In frailty model controlled for age, gender, and familial clustering effect, PKD2 genotype had 0.2 times lower risk for reaching ESRD than PKD1-PT genotype (p = 0.037). In conclusion, our results suggest that genotyping can contribute to selecting rapid progressors for new emerging therapeutic interventions among Koreans.

scholarly journals Recent Advances in the Management of Autosomal Dominant Polycystic Kidney Disease

2018 ◽  
Vol 13 (11) ◽  
pp. 1765-1776 ◽  
Author(s):  
Fouad T. Chebib ◽  
Vicente E. Torres
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Somatostatin Analogs ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Total Kidney Volume ◽  
Disease Modifying ◽  
Autosomal Dominant Polycystic Kidney ◽  
Disease Stratification

Autosomal dominant polycystic kidney disease (ADPKD), the most common monogenic cause of ESKD, is characterized by relentless development of kidney cysts, hypertension, and destruction of the kidney parenchyma. Over the past few years, major advancements in diagnosing, prognosticating, and understanding the pathogenesis and natural course of the disease have been made. Currently, no kidney disease is more suitable for nephron-protective strategies. Early nephrology referral and implementation of these strategies may have a substantial effect. Total kidney volume is a good prognostication marker and allows stratification of patients into slow or rapid progressing disease, with implications for their management. Measurement of total kidney volume, disease stratification, and prognostication are possible using readily available tools. Although some patients require only monitoring and basic optimized kidney protective measures, such as rigorous BP control and various lifestyle and dietary changes, others will benefit from disease-modifying treatments. Vasopressin V2 receptor antagonists, a likely disease-modifying treatment, has been approved in several countries and recently by the US Food and Drug Administration; other therapies, such as somatostatin analogs and other novel agents, are currently in clinical trials. The purpose of this article is to present our views on the optimal management to delay kidney disease progression in ADPKD.

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