scholarly journals The Association of Combined Total Kidney and Liver Volume with Pain and Gastrointestinal Symptoms in Patients with Later Stage Autosomal Dominant Polycystic Kidney Disease

2017 ◽  
Vol 46 (3) ◽  
pp. 239-248 ◽  
Author(s):  
Hedwig M.A. D'Agnolo ◽  
Niek F. Casteleijn ◽  
Tom J.G. Gevers ◽  
Hans de Fijter ◽  
Maartje D.A. van Gastel ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Liver Volume ◽  
Gastrointestinal Symptoms ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Total Liver Volume ◽  
Gi Symptoms ◽  
Autosomal Dominant Polycystic Kidney

Background: There is an ongoing debate if and how kidney and liver volume are associated with pain and gastrointestinal (GI) symptoms in autosomal dominant polycystic kidney disease (ADPKD) patients. Since both kidney and liver volume could interact, we investigated whether combined total kidney and liver volume had stronger associations with ADPKD-related pain and GI symptoms than the volumes of the organs separately. Methods: We used baseline data from the DIPAK-1 study, which included ADPKD patients with an estimated glomerular filtration rate (eGFR) between 30 and 60 mL/min/1.73 m2. MR imaging was performed to measure height-adjusted total kidney volume (hTKV), height-adjusted total liver volume (hTLV) and the combination of both (height-adjusted total kidney liver volume [hTKLV]). Results: Three hundred nine ADPKD patients were included with a mean age of 48 ± 7 years, 53% female, eGFR 50 ± 11 mL/min/1.73 m2 and median hTKV, hTLV and hTKLV of 1,095 (758-1,669), 1,173 (994-1,523) and 2,496 (1,972-3,352) mL/m, respectively. ADPKD-related pain and GI symptoms were present in, respectively, 27.5 and 61.2% of patients. Gender was no effect modifier in the association between kidney and/or liver volume, and symptom burden, indicating that all models could be tested in the overall study population. hTKLV and hTLV were significantly associated with pain and GI symptoms, whereas hTKV was not. Model testing revealed that the associations of pain and GI symptoms with hTKLV were significantly stronger than with hTKV (p = 0.04 and p = 0.04, respectively) but not when compared to hTLV (p = 0.2 and p = 0.5, respectively). Conclusions: This study indicates that combined kidney and liver volume was associated with the presence and severity of pain and GI symptoms in ADPKD, with a more prominent role for hTLV than for hTKV.

scholarly journals Automatic Measurement of Kidney and Liver Volumes from MR Images of Patients Affected by Autosomal Dominant Polycystic Kidney Disease

2019 ◽  
Vol 30 (8) ◽  
pp. 1514-1522 ◽  
Author(s):  
Maatje D.A. van Gastel ◽  
Marie E. Edwards ◽  
Vicente E. Torres ◽  
Bradley J. Erickson ◽  
Ron T. Gansevoort ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Liver Volume ◽  
Magnetic Resonance Images ◽  
Kidney Volume ◽  
Automated Segmentation ◽  
Segmentation Method ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney

BackgroundThe formation and growth of cysts in kidneys, and often liver, in autosomal dominant polycystic kidney disease (ADPKD) cause progressive increases in total kidney volume (TKV) and liver volume (TLV). Laborious and time-consuming manual tracing of kidneys and liver is the current gold standard. We developed a fully automated segmentation method for TKV and TLV measurement that uses a deep learning network optimized to perform semantic segmentation of kidneys and liver.MethodsWe used 80% of a set of 440 abdominal magnetic resonance images (T2-weighted HASTE coronal sequences) from patients with ADPKD to train the network and the remaining 20% for validation. Both kidneys and liver were also segmented manually. To evaluate the method’s performance, we used an additional test set of images from 100 patients, 45 of whom were also involved in longitudinal analyses.ResultsTKV and TLV measured by the automated approach correlated highly with manually traced TKV and TLV (intraclass correlation coefficients, 0.998 and 0.996, respectively), with low bias and high precision (<0.1%±2.7% for TKV and −1.6%±3.1% for TLV); this was comparable with inter-reader variability of manual tracing (<0.1%±3.5% for TKV and −1.5%±4.8% for TLV). For longitudinal analysis, bias and precision were <0.1%±3.2% for TKV and 1.4%±2.9% for TLV growth.ConclusionsThese findings demonstrate a fully automated segmentation method that measures TKV, TLV, and changes in these parameters as accurately as manual tracing. This technique may facilitate future studies in which automated and reproducible TKV and TLV measurements are needed to assess disease severity, disease progression, and treatment response.

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.

Autosomal Dominant Polycystic Kidney Disease: Clinical Assessment of Rapid Progression

2018 ◽  
Vol 48 (4) ◽  
pp. 308-317 ◽  
Author(s):  
Mónica Furlano ◽  
Irene Loscos ◽  
Teresa Martí ◽  
Gemma Bullich ◽  
Nadia Ayasreh ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Rapid Progression ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Decision Algorithm ◽  
Autosomal Dominant Polycystic Kidney ◽  
The Impact

Background: Autosomal dominant polycystic kidney disease (ADPKD) causes the development of renal cysts and leads to a decline in renal function. Limited guidance exists in clinical practice on the use of tolvaptan. A decision algorithm from the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Working Groups of Inherited Kidney Disorders and European Renal Best Practice (WGIKD/ERBP) has been proposed to identify candidates for tolvaptan treatment; however, this algorithm has not been assessed in clinical practice. Methods: Eighteen-month cross-sectional, unicenter, observational study assessing 305 consecutive ADPKD patients. The ERA-EDTA WGIKD/ERBP algorithm with a stepwise approach was used to assess rapid progression (RP). Subsequently, expanded criteria based on the REPRISE trial were applied to evaluate the ­impact of extended age (≤55 years) and estimated glomerular filtration rate (eGFR; ≥25 mL/min/1.73 m2). Results: Historical eGFR decline, indicative of RP, was fulfilled in 26% of 73 patients who were candidates for RP assessment, mostly aged 31–55 years. Further tests including ultrasound and MRI measurements of kidney volume plus genetic testing enabled the evaluation of the remaining patients. Overall, 15.7% of patients met the criteria for rapid or likely RP using the algorithm, and the percentage increased to 27% when extending age and eGFR. Conclusions: The ERA-EDTA WGIKD/ERBP algorithm provides a valuable means of identifying in routine clinical practice patients who may be eligible for treatment with tolvaptan. The impact of a new threshold for age and eGFR may increase the percentage of patients to be treated.

scholarly journals A Review of the Imaging Techniques for Measuring Kidney and Cyst Volume in Establishing Autosomal Dominant Polycystic Kidney Disease Progression

2018 ◽  
Vol 48 (1) ◽  
pp. 67-78 ◽  
Author(s):  
Riccardo Magistroni ◽  
Cristiana Corsi ◽  
Teresa Martí ◽  
Roser Torra
Keyword(s):  
Kidney Disease ◽  
Disease Progression ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Imaging Techniques ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Renal Disorder ◽  
Renal Imaging ◽  
Autosomal Dominant Polycystic Kidney

Background: Autosomal dominant polycystic kidney disease (ADPKD) is the commonest inherited renal disorder; it is defined by progressive renal cyst formation and subsequent renal enlargement that leads to end-stage renal disease. Until recently, only symptomatic treatments for ADPKD existed. However, therapies that address the underlying pathophysiology of ADPKD are now available and accurate identification of the rate of disease progression is essential. Summary: Published data on the different imaging modalities for measuring kidney and cyst volumes in ADPKD are reviewed. The advantages and drawbacks of the different techniques for calculating kidney volume from renal imaging are also examined, including the use of manual planimetry, stereology, and the ellipsoid equation, as well as the prospect of semi- and fully automatic techniques. The translation of these approaches into clinical practice and their role in informing treatment decisions is discussed. Key Messages: These new therapies require the accurate monitoring of disease progression, which along with diagnosis and prognosis, relies on the effective use of renal imaging techniques. There is growing support for the use of total kidney volume as a measure of cyst burden and as a prognostic predictor of renal function in ADPKD, showing promise as a marker of disease progression.

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 Somatostatin in renal physiology and autosomal dominant polycystic kidney disease

2019 ◽  
Vol 35 (8) ◽  
pp. 1306-1316 ◽  
Author(s):  
A Lianne Messchendorp ◽  
Niek F Casteleijn ◽  
Esther Meijer ◽  
Ron T Gansevoort
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Unmet Need ◽  
Cyst Formation ◽  
Renal Physiology ◽  
Intracellular Camp ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney

Abstract Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive cyst formation, leading to growth in kidney volume and renal function decline. Although therapies have emerged, there is still an important unmet need for slowing the rate of disease progression in ADPKD. High intracellular levels of adenosine 3′,5′-cyclic monophosphate (cAMP) are involved in cell proliferation and fluid secretion, resulting in cyst formation. Somatostatin (SST), a hormone that is involved in many cell processes, has the ability to inhibit intracellular cAMP production. However, SST itself has limited therapeutic potential since it is rapidly eliminated in vivo. Therefore analogues have been synthesized, which have a longer half-life and may be promising agents in the treatment of ADPKD. This review provides an overview of the complex physiological effects of SST, in particular renal, and the potential therapeutic role of SST analogues in ADPKD.

Comparison of Different Selection Strategies for Tolvaptan Eligibility among Autosomal Dominant Polycystic Kidney Disease Patients

2019 ◽  
Vol 50 (4) ◽  
pp. 281-290 ◽  
Author(s):  
Vera C. Wulfmeyer ◽  
Bernd Auber ◽  
Hermann Haller ◽  
Roland Schmitt
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Cohort Analysis ◽  
Rapid Progression ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Total Study Population ◽  
Autosomal Dominant Polycystic Kidney ◽  
Assessment Strategies

Background: Tolvaptan can slow down renal function decline in autosomal dominant polycystic kidney disease (­ADPKD). While there is consensus across international recommendations that the drug should only be used in patients with high risk of rapid progression, identification criteria for rapid progression vary. Here, we investigated different assessment strategies using a real-life ADPKD cohort. Methods: Observational retrospective cohort analysis. The study included 131 ADPKD patients aged 19–78 years who were referred to the Hannover Medical School outpatient clinic for evaluation of tolvaptan treatment. Six different assessment strategies for tolvaptan eligibility were tested for each patient. Comparative analysis for different assessments was performed in the total study population, the subpopulation with available computed tomography/magnetic resonance imaging data, and the genotyped subpopulation. Results: Comparing 6 assessment strategies revealed strong variations in the individual selection processes resulting in treatment recommendations for 14.5–64.9% of patients. The highest patient number was selected by the Scottish and the lowest by the Japanese approach. Few patients had positive recommendations by all 6 systems, but strong congruency was observed between the Scottish, U.K. and Canadian patient selection. The lowest number of overlapping patients was found between the Japanese and the ERA-EDTA selection. Important discrepancies were also found between the ERA-EDTA and the U.S. system due to different emphases on parameters of kidney function versus kidney volume. Limitations of the study included the restricted sample size, heterogeneity in parameter availability and lack of outcome data. Conclusions: The study draws attention to important discrepancies between different decision algorithms for tolvaptan eligibility in ADPKD patients.

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