ACR logo

Appropriateness Criteria

Reference Study Type Patients/Events Study Objective(Purpose of Study) Study Results Study Quality
2. Caroli A, Kline TL. Abdominal Imaging in ADPKD: Beyond Total Kidney Volume. J Clin Med. 2023 Aug 05;12(15):5133. Review/Other-Dx N/A To provide an overview of ADPKD imaging biomarkers, focusing on the quantification methods, potential, and necessary steps toward a successful translation to clinical practice. No results stated in abstract. 4
3. Odedra D, Sabongui S, Khalili K, Schieda N, Pei Y, Krishna S. Autosomal Dominant Polycystic Kidney Disease: Role of Imaging in Diagnosis and Management. Radiographics. 2023 Jan;43(1):e220126. Review/Other-Dx N/A Role of imaging in diagnosis and management of ADPKD. No results stated in abstract. 4
4. Chapman AB, Bost JE, Torres VE, et al. Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 2012 Mar;7(3):479-86. Observational-Dx 241 patients To determine if height-adjusted TKV (htTKV) predicts the onset of renal insufficiency. After a mean follow-up of 7.9 years, stage 3 CKD was attained in 30.7% of the enrollees. Using baseline htTKV, negative correlations with GFR increased from -0.22 at baseline to -0.65 at year 8. In multivariable analysis, a baseline htTKV increase of 100 cc/m significantly predicted the development of CKD within 8 years with an odds ratio of 1.48 (95% confidence interval: 1.29, 1.70). In receiver operator characteristic curve analysis, baseline htTKV of 600 cc/m most accurately defined the risk of developing stage 3 CKD within 8 years with an area under the curve of 0.84 (95% confidence interval: 0.79, 0.90). htTKV was a better predictor than baseline age, serum creatinine, BUN, urinary albumin, or monocyte chemotactic protein-1 excretion (P<0.05). 2
5. Smith AD, Nikolaidis P, Khatri G, et al. ACR Appropriateness Criteria® Acute Pyelonephritis: 2022 Update. J Am Coll Radiol 2022;19:S224-S39. Review/Other-Dx N/A Evidence-based guidelines to assist referring physicians and other providers in making the most appropriate imaging or treatment decision for acute pyelonephritis. No results stated in abstract. 4
6. Gupta RT, Kalisz K, Khatri G, et al. ACR Appropriateness Criteria® Acute Onset Flank Pain-Suspicion of Stone Disease (Urolithiasis). J Am Coll Radiol 2023;20:S315-S28. Review/Other-Dx N/A Evidence-based guidelines to assist referring physicians and other providers in making the most appropriate imaging or treatment decision for acute onset flank pain-suspicion of stone disease (urolithiasis). No results stated in abstract. 4
8. Pei Y, Obaji J, Dupuis A, et al. Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2009 Jan;20(1):205-12. Review/Other-Dx 577 Individuals who are at risk for autosomal dominant polycystic kidney disease are often screened by ultrasound using diagnostic criteria derived from individuals with mutations in PKD1. 577 and 371 at-risk individuals from 58 PKD1 and 39 PKD2 families, respectively, were assessed by renal ultrasound and molecular genotyping. Using sensitivity data derived from genetically affected individuals and specificity data derived from genetically unaffected individuals, various diagnostic criteria were compared. In addition, data sets were created to simulate the PKD1 and PKD2 case mix expected in practice to evaluate the performance of diagnostic criteria for families of unknown genotype. The diagnostic criteria currently in use performed suboptimally for individuals with mutations in PKD2 as a result of reduced test sensitivity. In families of unknown genotype, the presence of three or more (unilateral or bilateral) renal cysts is sufficient for establishing the diagnosis in individuals aged 15 to 39 y, two or more cysts in each kidney is sufficient for individuals aged 40 to 59 y, and four or more cysts in each kidney is required for individuals > or = 60 yr. Conversely, fewer than two renal cysts in at-risk individuals aged > or = 40 yr is sufficient to exclude the disease. 4
9. Mai J, Lee VW, Lopez-Vargas P, Vladica P. KHA-CARI Autosomal Dominant Polycystic Kidney Disease Guideline: Imaging Approaches for Diagnosis. Semin Nephrol. 2015 Nov;35(6):S0270-9295(15)00170-9. Review/Other-Dx N/A To present the KHA-CARI autosomal dominant polycystic kidney disease guideline. No abstract available. 4
10. Ars E, Bernis C, Fraga G, et al. Consensus document on autosomal dominant polycystic kindey disease from the Spanish Working Group on Inherited Kindey Diseases. Review 2020. Nefrologia (Engl Ed). 42(4):367-389, 2022 Jul-Aug. Review/Other-Dx N/A Revised consensus statement from the previous 2014 version, presenting the recommendations of the Spanish Working Group on Inherited Kidney Diseases. No results stated in abstract. 4
11. Torres VE, Ahn C, Barten TRM, et al. KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD): executive summary. Kidney Int. 2025 Feb;107(2):S0085-2538(24)00481-2. Review/Other-Dx N/A Clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD) No results stated in abstract. 4
12. Pei Y, Hwang YH, Conklin J, et al. Imaging-based diagnosis of autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 2015 Mar;26(3):746-53. Observational-Dx 126 The clinical use of conventional ultrasonography (US) in autosomal dominant polycystic kidney disease (ADPKD) is currently limited by reduced diagnostic sensitivity, especially in at-risk subjects younger than 30 years of age. We analyzed 110 at-risk subjects whose disease status was unequivocally defined by molecular testing and 45 unaffected healthy control subjects. Using a total of >10 cysts as a test criterion in subjects younger than 30 years of age, we found that MRI provided both a sensitivity and specificity of 100%. Comparison of our results from HR US with those from a previous study of conventional US using the test criterion of a total of three or more cysts found a higher diagnostic sensitivity (approximately 97% versus approximately 82%) with a slightly decreased specificity (approximately 98% versus 100%) in this study. Similar results were obtained in test subjects between the ages of 30 and 40 years old. 3
13. Pei Y, Watnick T. Diagnosis and screening of autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis. 2010 Mar;17(2):140-52. Review/Other-Dx N/A To review the clinical utilities and limitations of both imaging- and molecular-based diagnostic tests and outline our approach for the evaluation of individuals suspected to have ADPKD. No results stated in abstract. 4
16. Sharma K, Rupprecht C, Caroli A, et al. Automatic Segmentation of Kidneys using Deep Learning for Total Kidney Volume Quantification in Autosomal Dominant Polycystic Kidney Disease. Sci Rep. 2017 May 17;7(1):2049. Review/Other-Dx 165 An automated segmentation method based on deep learning has been proposed for TKV computation on computed tomography (CT) dataset of ADPKD patients exhibiting mild to moderate or severe renal insufficiency. The proposed method has been trained (n = 165) and tested (n = 79) on a wide range of TKV (321.2-14,670.7 mL) achieving an overall mean Dice Similarity Coefficient of 0.86 ± 0.07 (mean ± SD) between automated and manual segmentations from clinical experts and a mean correlation coefficient (?) of 0.98 (p < 0.001) for segmented kidney volume measurements in the entire test set. 4
17. Kline TL, Edwards ME, Korfiatis P, Akkus Z, Torres VE, Erickson BJ. Semiautomated Segmentation of Polycystic Kidneys in T2-Weighted MR Images. AJR Am J Roentgenol. 207(3):605-13, 2016 Sep. Review/Other-Dx 40 patients To develop and validate a fast, accurate, and reproducible method that will increase and improve institutional measurement of total kidney volume and thereby avoid the higher costs, increased operator processing time, and inherent subjectivity associated with manual contour tracing. The MIROS approach required less than 5 minutes of user interaction in all cases. When compared with the ground-truth reference standard, MIROS showed no significant bias and had low variability (mean ± 2 SD, 0.19% ± 6.96%). 4
18. Chapman AB, Guay-Woodford LM, Grantham JJ, et al. Renal structure in early autosomal-dominant polycystic kidney disease (ADPKD): The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) cohort. Kidney Int. 2003 Sep;64(3):1035-45. Observational-Dx 241 ADPKD individuals Autosomal-dominant polycystic kidney disease (ADPKD) is characterized by gradual renal enlargement and cyst growth prior to loss of renal function. Reliability coefficients for MR renal and cyst volume measurements in phantoms were 99.9% and 89.2%, respectively. In the full-scale protocol, 241 ADPKD individuals (145 women and 96 men) were enrolled. Total renal, cyst, and % cyst volume were significantly greater in each decade group. Hypertensive individuals demonstrated greater renal, cyst, and % cyst volume than normotensive subjects. Age-adjusted renal (r = -0.31, P < 0.0001), cyst (r = -0.36, P < 0.0001), and % cyst volume (r = -0.35, P < 0.0001) were inversely related to glomerular filtration rate (GFR). Age-adjusted renal volume (r = 0.42, P < 0.0001), cystic (r = 0.39, P < 0.0001, and % cyst volume (r = 0.41, P < 0.0001) were related with urinary albumin excretion. 3
19. Irazabal MV, Rangel LJ, Bergstralh EJ, et al. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol. 26(1):160-72, 2015 Jan. Review/Other-Dx 590 The rate of renal disease progression varies widely among patients with autosomal dominant polycystic kidney disease (ADPKD). Patients from the Mayo Clinic Translational PKD Center with ADPKD (n=590) with computed tomography/magnetic resonance images and three or more eGFR measurements over =6 months were classified radiologically as typical (n=538) or atypical (n=52). Total kidney volume (TKV) was measured using stereology (TKVs) and ellipsoid equation (TKVe). Typical patients were randomly partitioned into development and internal validation sets and subclassified according to height-adjusted TKV (HtTKV) ranges for age (1A-1E, in increasing order). Consortium for Radiologic Imaging Study of PKD (CRISP) participants (n=173) were used for external validation. TKVe correlated strongly with TKVs, without systematic underestimation or overestimation. A longitudinal mixed regression model to predict eGFR decline showed that log2HtTKV and age significantly interacted with time in typical patients, but not in atypical patients. When 1A-1E classifications were used instead of log2HtTKV, eGFR slopes were significantly different among subclasses and, except for 1A, different from those in healthy kidney donors. The equation derived from the development set predicted eGFR in both validation sets. The frequency of ESRD at 10 years increased from subclass 1A (2.4%) to 1E (66.9%) in the Mayo cohort and from 1C (2.2%) to 1E (22.3%) in the younger CRISP cohort. Class and subclass designations were stable. 4
20. Bevilacqua MU, Hague CJ, Romann A, et al. CT of Kidney Volume in Autosomal Dominant Polycystic Kidney Disease: Accuracy, Reproducibility, and Radiation Dose. Radiology. 2019 Jun;291(3):660-667. Observational-Dx 30 patients 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. Thirty participants (mean age, 41 years; age range, 24-67 years) had a mean TKV of 1368.9 mL ± 1146.13 (standard deviation). The ULD and LD CT protocols had median dose-length product of 58.8 and 115.5 mGy · cm, respectively (P = .01), and CT dose index of 1.2 and 3.9 mGy, respectively (P < .001). All imaging modalities and measurement equations had excellent correlation with the reference standard (r2 > 0.98). RMSE ranged from 80.5 to 157.3 mL (5.9%-11.5% of mean TKV). Intraclass correlation coefficients were greater than 0.98 for all methods. Mean measurement times for the ellipsoid method ranged from 4.6 to 5.2 minutes compared with a mean of 27.7 minutes (range, 14-60 minutes) for manual planimetry. 3
21. Gundogdu E, Cihan C, Yazici C. Total kidney volume in autosomal dominant polycystic kidney disease: intraobserver and interobserver agreement of two methods with MRI. TURK. J. MED. SCI.. 54(3):537-544, 2024. Review/Other-Dx 55 patients To evaluate intra- and interobserver agreement of the ellipsoid formula (EF) and manual boundary tracing method (MBTM) used in TKV measurement of ADPKD patients. The ICC (95% CI) indicated excellent agreement between the observers for both methods (among all observers, p < 0.001). Furthermore, excellent intraobserver agreement was found between all observer measurements either EF or MBTM based on ICC (95% CI) (p < 0.001). The results of the linear regression analysis demonstrated high correlations between the two methods in all three observers (r = 0.992, p < 0.001 for the first observer; r = 0.975, p < 0.001 for the second observer; r = 0.989, p < 0.001 for the third observer). 4
22. Kline TL, Korfiatis P, Edwards ME, et al. Performance of an Artificial Multi-observer Deep Neural Network for Fully Automated Segmentation of Polycystic Kidneys. J Digit Imaging. 30(4):442-448, 2017 Aug. Review/Other-Dx 2000 cases To describe a fully automated approach for segmenting PKD kidneys within MR images that simulates a multi-observer approach in order to create an accurate and robust method for the task of segmentation and computation of TKV for PKD patients. No results stated in abstract. 4
23. Simms RJ, Doshi T, Metherall P, et al. A rapid high-performance semi-automated tool to measure total kidney volume from MRI in autosomal dominant polycystic kidney disease. Eur Radiol. 29(8):4188-4197, 2019 Aug. Review/Other-Dx 61 patients To develop a high-performance, rapid semi-automated method (Sheffield TKV Tool) for measuring total kidney volume (TKV) from magnetic resonance images (MRI) in patients with autosomal dominant polycystic kidney disease (ADPKD). Sixty-one patients (mean age 45 ± 14 years, baseline eGFR 76 ± 32 ml/min/1.73 m2) with ADPKD had a wide range of TKV (258-3680 ml) measured manually. The Sheffield TKV Tool was highly accurate (mean volume error 0.5 ± 5.3% for right kidney, - 0.7 ± 5.5% for left kidney), reproducible (intra-operator variability - 0.2 ± 1.3%; inter-operator variability 1.1 ± 2.9%) and outperformed published methods. It took less than 6 min to execute and performed consistently with high accuracy in an external MRI dataset of T2-weighted sequences with TKV acquired using three different scanners and measured using a different segmentation methodology (mean volume error was 3.45 ± 3.96%, n = 65). 4
24. van Gastel MDA, Edwards ME, Torres VE, Erickson BJ, Gansevoort RT, Kline TL. Automatic Measurement of Kidney and Liver Volumes from MR Images of Patients Affected by Autosomal Dominant Polycystic Kidney Disease. J Am Soc Nephrol. 30(8):1514-1522, 2019 08. Observational-Dx 440 MRIs To develope 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. TKV 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. 2
25. Gregory AV, Anaam DA, Vercnocke AJ, et al. Semantic Instance Segmentation of Kidney Cysts in MR Images: A Fully Automated 3D Approach Developed Through Active Learning. J Digit Imaging. 34(4):773-787, 2021 08. Review/Other-Dx N/A To develope the first 3D semantic instance cyst segmentation algorithm for kidneys in MR images. No results stated in abstract. 4
26. Taylor J, Thomas R, Metherall P, et al. An Artificial Intelligence Generated Automated Algorithm to Measure Total Kidney Volume in ADPKD. KI Rep. 9(2):249-256, 2024 Feb. Review/Other-Dx 227 scans To report an artificial intelligence (AI)-generated method for routinely measuring total kidney volume (TKV). The training or internal validation cohort was younger (mean age 44.0 vs. 51.5 years) and the female-to-male ratio higher (1.2 vs. 0.94) compared to the clinical validation cohort. The majority of CYSTic patients had PKD1 mutations (79%) and typical disease (Mayo Imaging class 1, 86%). The median DICE score on the clinical validation data set between the algorithm and human analysts was 0.96 for left and right kidneys with a median TKV error of -1.8%. The time taken to manually segment kidneys in the CYSTic data set was 56 (±28) minutes, whereas manual corrections of the algorithm output took 8.5 (±9.2) minutes per scan. 4
27. Sore R, Cathier P, Vlachomitrou AS, et al. Deep learning-based segmentation of kidneys and renal cysts on T2-weighted MRI from patients with autosomal dominant polycystic kidney disease. Eur Radiol Exp. 8(1):122, 2024 Oct 30. Review/Other-Dx 164 patients To train and test a deep learning-based algorithm for automatically segmenting kidneys and renal cysts in patients with autosomal dominant polycystic kidney disease (ADPKD). We included 164 ADPKD patients. Dice similarity coefficients ranged from 85.9% to 87.4% between the algorithms and the raters' segmentations and from 84.2% to 86.2% across raters' segmentations. For TCV assessment, the biases ± standard deviations (SD) were 3-19 ± 137-151 mL between the algorithm and the raters, and 22-45 ± 49-57 mL across raters. The algorithm underestimated TKV and TCV in two outliers with TCV > 2800 mL. For cystic index assessment, the biases ± SD were 2.5-6.9% ± 6.7-8.3% between the algorithm and the raters, and 2.1-9.4 ± 7.4-11.6% across raters. 4
28. Iijima H, Tada T, Hashimoto M, et al. Utility of ultrasonography for predicting indications for tolvaptan in patients with autosomal dominant polycystic kidney disease. J Med Ultrason (2001). 50(1):81-87, 2023 Jan. Observational-Dx 46 patients To investigate whether ultrasonography can be used for TKV assessment in patients with ADPKD. TKV determined by CT was 796.8 (508.8-1,560.3) mL. The median length, anteroposterior distance, and mediolateral distance determined using ultrasonography were 15.7 cm, 7.6 cm, and 7.6 cm in the left kidney, and 13.4 cm, 6.9 cm, and 7.2 cm in the right kidney, respectively. Multivariate regression analysis showed that total kidney length (left and right) [variance inflation factor (VIF), 9.349] and total mediolateral distance (left and right) (VIF, 3.988) were independently associated with TKV. The correlation (r) between the logarithm of TKV determined by CT and total mediolateral distance determined using ultrasonography was 0.915 (p < 0.001). The linear regression equation was log (total kidney volume) = 1.833 + 0.075 × total mediolateral distance (left and right) based on ultrasonography. The area under the receiver operating characteristic curve for total mediolateral distance determined using ultrasonography to predict TKV of 750 mL or more was 0.989. Using the total mediolateral distance cut-off value of 14.2 cm, the sensitivity and specificity were 96.0% and 100.0%, respectively. 4
29. Xie Y, Xu M, Chen Y, Zhu X, Ju S, Li Y. The predictive value of renal parenchymal information for renal function impairment in patients with ADPKD: a multicenter prospective study. Abdom Radiol. 47(8):2845-2857, 2022 08. Review/Other-Dx 340 patients To demonstrate that renal parenchymal information, combined with parenchymal volume and radiomics features, may have more valuable clinical guiding significance. Compared with TKV, decreased RPV presented a closer relationship with renal function impairment, namely, with the impairment rate (RPV: 82.3% vs. TVK: 67.1%) and eGFR (RPV: r = 0.614, p < 0.001 vs. TKV: r = -0.452, p < 0.001), and showed higher predictive power (RPV: AUC = 0.752 [95%CI 0.692-0.805], p < 0.001 vs. TKV: AUC = 0.711 [95%CI 0.649-0.768], p < 0.001). Correspondingly, the radiomics analysis that was derived from the renal parenchyma also showed a satisfactory result (AUC = 0.849 [95%Cl 0.797-0.892], p < 0.001). Importantly, the predictive power for renal function impairment was further improved in the combined model of RPV and radiomics features (AUC = 0.902 [95%Cl 0.857-0.937], p < 0.001). 4
30. Sise C, Kusaka M, Wetzel LH, et al. Volumetric determination of progression in autosomal dominant polycystic kidney disease by computed tomography. Kidney Int. 2000 Dec;58(6):2492-501. Review/Other-Dx 10 patients To determine the rate of kidney enlargement in patients with ADPKD. The mean initial Vt, Vp, and Vc values (+/- SEM) were 561 +/- 66, 243 +/- 19, and 317 +/- 57 mL per kidney, respectively. In 10 patients, after a mean of 5.7 years (range 3.3 to 11.9), Vt increased 323 +/- 79 mL (P < 0.01, range -25 to 1182 mL); the rate of volume increase was 53.9 +/- 10.4 mL/year/kidney (P < 0.001). In eight patients with repeat contrast-enhanced scans, Vt, Vp, and Vc increased 211 +/- 58 mL (P < 0.005), 26 +/- 11 mL (P > 0.05), and 185 +/- 52 mL (P < 0.01), respectively. In 19 individual spherical cysts selected in six patients, the mean initial volume was 15.0 +/- 7.2 mL (range 1.1 to 137 mL), and the average rate of volume increase was 0.52 +/- 0.21 mL/month (P < 0.025, range 0.02 to 4.15 mL/month). In five patients who eventually required dialysis, 11.2 years after the initial CT scan, the initial cyst/kidney volume ratio (combined for both kidneys) exceeded 0.43; four patients with lower cyst/kidney volume ratios had serum creatinine levels <1.5 mg/dL, 8.7 years after the initial CT scan. 4
31. Hammoud S, Tissier AM, Elie C, et al. Ultrasonographic renal volume measurements in early autosomal dominant polycystic disease: comparison with CT-scan renal volume calculations. Diagn Interv Imaging. 96(1):65-71, 2015 Jan. Observational-Dx 24 patients To investigate the correlation and concordance between the ellipsoid volume calculated by ultrasonography measurements (Vol3DUS) and the reference kidney volume measured by CT (VolTDM) in early autosomal dominant polycystic kidney disease (ADPKD). The US volume was strongly correlated with the CT volume by using the maximum width in a transverse section (r=0.83) with a mean Vol3DUS=692±348ml [180; 2069]. The most reproducible ultrasonography measurement was the height. When the kidney volume exceeded 800ml, US underestimated the volume. However, the median error was -57.5ml [-1090; 183] and 85% of the Vol3DUS calculated differed by more than 5% from the reference measurement. 3
32. Moriyama T, Nakayama Y, Soejima M, et al. Effect of tolvaptan on renal involvement in patients with autosomal dominant polycystic kidney disease according to different gene mutations. Clin Exp Nephrol. 25(3):251-260, 2021 Mar. Review/Other-Dx 135 patients explore the different effects of tolvaptan on the annual changes in total kidney volume (%TKV) and estimated glomerular filtration rate (eGFR) according to the gene mutation type in ADPKD patients. Patients (age: 52.3 ± 11.2 years) were administered tolvaptan at a dose of 45 or 60 mg. No variation was observed in the annual changes in eGFR (%eGFR) (before: - 10.5% ± 13.9%, after: - 14.4% ± 8.1%, P = 0.139), whereas %TKV was significantly improved after the tolvaptan treatment (before: 14.9% ± 8.0%, after: - 5.4% ± 7.6%, P < 0.001). Unlike %eGFR, tolvaptan treatment significantly improved %TKV, regardless of the type of gene mutation. 4
33. O'Neill WC, Robbin ML, Bae KT, et al. Sonographic assessment of the severity and progression of autosomal dominant polycystic kidney disease: the Consortium of Renal Imaging Studies in Polycystic Kidney Disease (CRISP). Am J Kidney Dis. 2005 Dec;46(6):1058-64. Review/Other-Dx 230 patients The accuracy and precision of ultrasonography (US) in assessing the severity of autosomal dominant polycystic kidney disease (ADPKD) is unknown. Variability between different sonographers ranged from 18% to 42%. Correlations between US and MRI volume were 0.88 and 0.89. The SD of the discrepancy from MRI ranged from 21% to 33% and was unrelated to kidney size or body mass. Kidney length was the most reproducible measurement, and its correlation with MRI volume was 0.84. All patients with an US volume less than 700 cm3 had an MRI volume less than 1,000 cm3, and all patients with an US volume greater than 1,700 cm3 had an MRI volume greater than 1,000 cm3. Increases in volume after 1 year were 12% +/- 36% for the ellipsoid method, 6% +/- 29% for the direct method, and 4.2% +/- 7.2% for MRI. Correlation between US and MRI measurement of fractional cyst volume was 0.80. 4
34. Jagtap JM, Gregory AV, Homes HL, et al. Automated measurement of total kidney volume from 3D ultrasound images of patients affected by polycystic kidney disease and comparison to MR measurements. Abdom Radiol. 47(7):2408-2419, 2022 07. Review/Other-Dx 22 patients To investigate a deep learning-based approach for automated segmentation of TKV from 3D US in ADPKD patients. Our method automatically segmented polycystic kidneys in 3D US images obtaining an average Dice coefficient of 0.80 on the test dataset. The kidney volume measurement compared with linear regression coefficient and bias from human tracing were R2 = 0.81, and - 4.42%, and between AI and reference standard were R2 = 0.93, and - 4.12%, respectively. MRI and US measured kidney volumes had R2 = 0.84 and a bias of 7.47%. 4
35. Balbo BE, Sapienza MT, Ono CR, et al. Cyst infection in hospital-admitted autosomal dominant polycystic kidney disease patients is predominantly multifocal and associated with kidney and liver volume. Braz J Med Biol Res. 47(7):584-93, 2014 Jul. Observational-Dx 18 patients To evaluate clinical and imaging factors associated with CI in kidney (KCI) and liver (LCI) in ADPKD. Total kidney (TKV) and liver (TLV) volumes were measured by CT-derived multiplanar reconstruction. CI was detected in 18 patients who experienced 24 episodes during an interval of 30 months (LCI in 12, KCI in 10 and concomitant infection in 2). Sensitivities of CT, magnetic resonance imaging and PET/CT were 25.0, 71.4, and 95.0%. Dysuria (P<0.05), positive urine culture (P<0.01), and previous hematuria (P<0.05) were associated with KCI. Weight loss (P<0.01) and increased C-reactive protein levels (P<0.05) were associated with LCI. PET/CT revealed that three or more infected cysts were present in 70% of the episodes. TKV was higher in kidney-affected than in LCI patients (AUC=0.91, P<0.05), with a cut-off of 2502 mL (72.7% sensitivity, 100.0% specificity). TLV was higher in liver-affected than in KCI patients (AUC=0.89, P<0.01) with a cut-off of 2815 mL (80.0% sensitivity, 87.5% specificity). A greater need for invasive procedures was observed in LCI (P<0.01), and the overall mortality was 20.8%. 2
36. Bobot M, Ghez C, Gondouin B, et al. Diagnostic performance of [(18)F]fluorodeoxyglucose positron emission tomography-computed tomography in cyst infection in patients with autosomal dominant polycystic kidney disease. Clin Microbiol Infect. 22(1):71-77, 2016 Jan. Review/Other-Dx 24 patients To evaluate the diagnostic performance of [(18)F]fluorodeoxyglucose positron emission tomography-computed tomography (18-FDG PET-CT) for the diagnosis of cyst infections among ADPKD patients, in comparison with computed tomography (CT) and magnetic resonance imaging (MRI). Thirty-two 18-FDG PET-CT scans were performed in 24 ADPKD patients with suspected cyst infection. A diagnosis of cyst infection was retained in 18 of 32 cases: 14 with positive 18-FDG PET-CT findings, and four false negatives. There were no false positives and no hypermetabolism of cyst walls in nine ADPKD control patients. 18-FDG PET-CT had a sensitivity of 77%, a specificity of 100%, and a negative predictive value of 77%. 18-FDG PET-CT allowed a differential diagnosis in three patients. In contrast, CT had a sensitivity of 7% and a negative predictive value of 35% (p <0.001 vs. 18-FDG PET-CT). Only eight MRI scans were performed. 4
37. Suwabe T. Cyst infection in autosomal dominant polycystic kidney disease: our experience at Toranomon Hospital and future issues. [Review]. Clin Exp Nephrol. 24(9):748-761, 2020 Sep. Review/Other-Dx N/A Cyst infection is a frequent and serious complication of autosomal dominant polycystic kidney disease (ADPKD) that is often difficult to treat and can be fatal. No results stated in abstract. 4
38. Suwabe T, Ubara Y, Ueno T, et al. Intracystic magnetic resonance imaging in patients with autosomal dominant polycystic kidney disease: features of severe cyst infection in a case-control study. BMC Nephrol. 17(1):170, 2016 11 09. Review/Other-Dx 76 patients To investigate the usefulness of intracystic MRI features for detection of severe cyst infection that is usually refractory to antibiotic therapy alone in patients with autosomal dominant polycystic kidney disease. At least one of four intracystic MRI features (high signal intensity (SI) on diffusion-weighted images (DWI), fluid-fluid level, wall thickening, or gas) was found in all of the cases, but such findings were also detected in some controls. Intracystic gas was specific for cyst infection, but its sensitivity was only 1.1 %. A high intracystic SI on DWI showed a sensitivity of 86.4 %, but its specificity was lower at 33.3 %. Both the specificity and sensitivity of a fluid-fluid level or wall thickening were about 80 %. However, the specificity of these MRI features decreased as total liver and kidney volume (TLKV) increased, falling to 65.8 % in patients with organomegaly (TLKV > 8500 cm3). A cyst diameter > 5 cm was useful for detecting severely infected cysts that needed drainage, and specificity was increased by combining the other four MRI findings with a cyst diameter > 5 cm. 4
39. Riyahi S, Dev H, Blumenfeld JD, et al. Hemorrhagic Cysts and Other MR Biomarkers for Predicting Renal Dysfunction Progression in Autosomal Dominant Polycystic Kidney Disease. J Magn Reson Imaging. 53(2):564-576, 2021 02. Review/Other-Dx 186 patients To investigate the utility of other MR features of ADPKD to predict progression. Hemorrhagic cysts, fraction of renal and hepatic cysts, height-adjusted liver and spleen volumes were significant independent predictors of future eGFR (final prediction model R2 = 0.88 P < 0.05). The number of hemorrhagic cysts significantly improved the prediction compared to ht-TKV in predicting future eGFR (area under the curve [AUC] = 0.94, 95% confidence interval [CI]: 0.9-0.94 vs. R2 = 0.9, 95% CI: 0.85-0.9, P = 0.045). For baseline eGFR =60 ml/min/1.73m2 , sensitivity for predicting eGFR<45 ml/min/1.73m2 by ht-TKV alone was 29%. Sensitivity increased to 72% with all MRI variables in the model (P < 0.05 = 0.019), whereas specificity was unchanged, 100% vs. 99%. 4
40. Schumacher K, Prince MR, Blumenfeld JD, et al. Quantitative susceptibility mapping for detection of kidney stones, hemorrhage differentiation, and cyst classification in ADPKD. Abdom Radiol. 49(7):2285-2295, 2024 Jul. Review/Other-Dx 33 patients To demonstrate feasibility of quantitative susceptibility mapping (QSM) in autosomal dominant polycystic kidney disease (ADPKD) patients and to compare imaging findings with traditional T1/T2w magnetic resonance imaging (MRI). QSM visualized two renal calcifications measuring 9 and 10 mm and three pelvic phleboliths measuring 2 mm but missed 24 calcifications measuring 1 mm or less and 1 larger calcification at the edge of the field of view. A total of 121 complex T1 hyperintense/T2 hypointense renal cysts were detected. 52 (43%) Cysts appeared hyperintense on QSM consistent with hemorrhage; 60 (49%) cysts were isointense with respect to simple cysts and normal kidney parenchyma, while the remaining 9 (7%) were hypointense. The presentation of the latter two complex cyst subtypes is likely indicative of proteinaceous composition without hemorrhage. 4
41. Neuville M, Hustinx R, Jacques J, Krzesinski JM, Jouret F. Diagnostic Algorithm in the Management of Acute Febrile Abdomen in Patients with Autosomal Dominant Polycystic Kidney Disease. PLoS ONE. 11(8):e0161277, 2016. Review/Other-Dx 173 patients Acute febrile abdomen represents a diagnostic challenge in patients with autosomal dominant polycystic kidney disease (ADPKD). Among a cohort of 173 ADPKD patients, 101 presented with 205 episodes of abdominal pain (n = 172) and/or fever (n = 33). 20 patients experienced 30 CyH, whereas 16 presented 23 episodes of definite (n = 11) or probable (n = 12) CyI. 35 IUO were observed in 31 patients. Clinically, fever was observed in 7% vs. 100% vs. 66% of CyH, CyI and IUO, respectively. Biologically, CRP cut-off at 70 mg/dl showed 92% sensitivity and 81% specificity in CyI diagnosis. Urine or blood cultures remained sterile in >90% of CyH, but were contributive in 53.4% of CyI and IUO, with a 74.2% prevalence for E. coli. Radiologically, ultrasounds, CT and magnetic resonance diagnosed CyI in 2.6%, 20% and 16.7% of cases, respectively. 18F-FDG positron-emission tomography (PET)/CT was done within a median period of 7 days post antibiotics, and significantly changed patient management in 71.4%. 4
42. Oh J, Shin CI, Kim SY. Infected cyst in patients with autosomal dominant polycystic kidney disease: Analysis of computed tomographic and ultrasonographic imaging features. PLoS ONE. 13(12):e0207880, 2018. Review/Other-Dx 51 To investigate the imaging features of cyst infection in autosomal dominant polycystic kidney disease (ADPKD) patients using computed tomography (CT) and ultrasonography (US). On CT scans, the median size of infected cysts was 5.5 cm (range: 2.3-18.8 cm) and 46 of 51 (90.2%) infected cysts were located in the subcapsular region. Most (48 of 51, 94.1%) infected cysts showed lobulated, focal bulging or irregular shape. Discernible wall thickening (84.1%) was the most frequently found imaging feature of infected cysts followed by relatively higher intracystic attenuation compared to normal cysts (79.1%) and pericystic fat infiltration (52.9%). Fluid/fluid level was found in 3 of 51 (5.9%) infected cysts and intracystic gas was found in 3 of 51 (5.9%) infected cysts, respectively. For hepatic cysts, 11 of 14 (78.6%) infected cysts showed pericystic hyperemia. Intracystic attenuation was significantly higher in infected cysts (median; 19.0 HU) than in presumed normal cysts (median; 8.5 HU) (P<0.001), and exceeded 25 HU in 18 (35.3%) of 51 infected cysts. Among the 41 infected cysts for which US images were available, 35 (85.1%) showed heterogeneous echogenicity. 4
43. Mei CL, Xue C, Yu SQ, et al. Executive Summary: Clinical Practice Guideline for Autosomal Dominant Polycystic Kidney Disease in China. Kidney Dis. 6(3):144-149, 2020 May. Review/Other-Dx N/A The main recommendations and suggestions of the ADPKD guidelines in this summary. No results stated in abstract. 4
44. Kwon HW, Lee HY, Hwang YH, Park HC, Ahn C, Kang KW. Diagnostic performance of 18F-FDG-labeled white blood cell PET/CT for cyst infection in patients with autosomal dominant polycystic kidney disease: a prospective study. Nucl Med Commun. 37(5):493-8, 2016 May. Observational-Dx 17 patients To evaluate the diagnostic performance of fluorine-18 fluorodeoxyglucose-labeled white blood cell (WBC) PET/computed tomography (CT) for detection of infected cysts in patients with ADPKD. Seven patients were classified as having renal CI (definite 6, probable 1). In this group, WBC PET/CT showed six positive findings and one equivocal finding. Seven patients were diagnosed with possible infection. In this group, WBC PET/CT showed six negative findings and one indeterminate finding. The diagnostic performance of WBC PET/CT showed advantages over CT or MRI scans (sensitivity 85.7%, specificity 87.5%, positive predictive value 85.7%, negative predictive value 87.5%). 2
45. Pijl JP, Glaudemans AWJM, Slart RHJA, Kwee TC. 18F-FDG PET/CT in Autosomal Dominant Polycystic Kidney Disease Patients with Suspected Cyst Infection. J Nucl Med. 59(11):1734-1741, 2018 11. Review/Other-Dx 30 patients To determine the value of 18F-FDG PET/CT for diagnosing renal or hepatic cyst infection in patients with autosomal dominant polycystic kidney disease (ADPKD). Thirty 18F-FDG PET/CT scans of 30 individual patients were included; 19 of them had positive results for cyst infection. According to a previously established clinical and biochemical reference standard, 18F-FDG PET/CT achieved a sensitivity of 88.9%, a specificity of 75.0%, a positive predictive value of 84.2%, and a negative predictive value of 81.8% for the diagnosis of cyst infection. In 5 cases, 18F-FDG PET/CT suggested that the symptoms could be explained by a different pathologic process, including pneumonia (n = 1), generalized peritonitis (n = 1), pancreatitis (n = 1), colitis (n = 1), and cholangitis (n = 1). The total duration of the hospital stay and the duration between the 18F-FDG PET/CT scan and hospital discharge for patients with 18F-FDG PET/CT scan results that were positive for cyst infection were significantly longer than those for patients with negative scan results (P = 0.005 and P = 0.009, respectively). Creatinine levels were significantly higher in patients with 18F-FDG PET/CT scan results that were positive for cyst infection than in patients with negative scan results (P = 0.015). Other comparisons of clinical parameters (age, sex, presence of fever [>38.5°C] for more than 3 d, abdominal pain, history of solid-organ transplantation and nephrectomy, and immune status), laboratory values (C-reactive protein level, leukocyte count, and estimated glomerular filtration rate), and microbiologic test results (blood and urine cultures) were not significantly different (P = 0.13-1.00) in patients with positive and negative 18F-FDG PET/CT scan results. 4
46. Neuville MF, Lovinfosse P, Jadoul A, et al. The use of a visual 4-point scoring scale improves the yield of 18F-FDG PET-CT imaging in the diagnosis of renal and hepatic cyst infection in patients with autosomal dominant polycystic kidney disease. Eur J Nucl Med Mol Imaging. 48(1):254-259, 2021 01. Review/Other-Dx 38 patients To characterize the [18F]FDG uptake in CyI in order to infer a visual 4-point diagnostic scale. Sixty [18F]FDG PET/CT scans were performed for suspected CyI in 38 ADPKD patients. Twenty-nine episodes met the gold-standard criteria for CyI. The visual assessment of PET/CT images reached a sensitivity of 73.1% and a specificity of 70.6%. Using the 4-point scale, an [18F]FDG score = 3 (i.e., cyst uptake > liver) improved the specificity to 85.3%. 4
47. Weinreb JC, Rodby RA, Yee J, et al. Use of Intravenous Gadolinium-based Contrast Media in Patients with Kidney Disease: Consensus Statements from the American College of Radiology and the National Kidney Foundation. Radiology. 298(1):28-35, 2021 01. Review/Other-Dx N/A To improve and standardize the care of patients with kidney disease who have indication(s) to receive ACR designated group II or group III intravenous gadolinium-based contrast media (GBCM). No results in abstract. 4
48. Davenport MS, Perazella MA, Yee J, et al. Use of Intravenous Iodinated Contrast Media in Patients with Kidney Disease: Consensus Statements from the American College of Radiology and the National Kidney Foundation. Radiology. 294(3):660-668, 2020 Mar. Review/Other-Dx N/A To discuss the risk of acute kidney injury (AKI) developing in patients with reduced kidney function following exposure to intravenous iodinated contrast media. No results in abstract 4
No of Rows: 44