Mayo Clinic Classification Research Articles

Renal Cell Carcinoma with Venous Tumor Thrombus: 15 Years of Experience in an Oncology Center.

Background: The purpose of this study is to report the experience of a single Portuguese oncology center in the management of patients with renal cell carcinoma (RCC) and venous tumor thrombus (VTT). Methods: This is a retrospective analysis of all patients with RCC and VTT surgically treated in our center between 2008 and 2023. Only patients with VTT up to level III (Mayo Clinic classification) were included. Patient, tumor characteristics and peri-operative outcome data were registered. Administration of systemic therapy was performed upon progression. Survival analysis was conducted with the collected data. Results: A total of 64 patients (n = 16 women) were included in this study. The mean age at diagnosis was 66.3 ± 10.7 years old. The VTT level was 0, I, II and III in 40 (62.5%), 12 (18.7%), 6 (9.4%) and 6 (9.4%) patients, respectively. Nine patients (14.1%) had distant metastasis at diagnosis. No peri-operative deaths occurred, and the major complication rate was 3.1%. Histology revealed 98.4% of clear cell RCC, with sarcomatoid differentiation present in 12.5% of the cases. A negative margin status was achieved in 54 (84.4%) patients. Systemic therapy was administered in 24 (37.5%) patients during follow-up. The median progression-free (PFS), cancer-specific (CSS) and overall (OS) survival were 23, 60 and 48 months, respectively. In multivariable analysis, significant predictors of CSS were tumor size, sarcomatoid differentiation and collecting system invasion. Conclusions: Radical nephrectomy with VTT excision up to level III is a feasible and safe procedure. Patients with large tumor size, sarcomatoid differentiation and collecting system invasion are at the highest risk and should be closely monitored.

PE075 Ten years experience of robot-assisted nephrectomy and venous tumor thrombectomy: Is it reasonable to reconsider Mayo Clinic Classification?

PE075 Ten years experience of robot-assisted nephrectomy and venous tumor thrombectomy: Is it reasonable to reconsider Mayo Clinic Classification?

MicroRNA and renal fibrosis in autosomal dominant polycystic kidney disease: a longitudinal study.

Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary kidney disorder that may progress to kidney failure, accounting for 5-10% of all patients with end-stage kidney disease (ESKD). Clinical data, as well as molecular genetics and advanced imaging techniques have provided surrogate prognostic biomarkers to predict rapid decline in kidney function, nonetheless enhanced tools for assessing prognosis for ADPKD are stillneeded. The aim of this study was to analyze specific microRNAs involved in the pathogenesis of ADPKD and in the development of renal fibrosis, evaluating their potential role as predictors of renal function loss. We evaluated kidney function by estimated glomerular filtration rate (eGFR) in 32 ADPKD patients in different stages of kidney disease at T0 and after a 24-month follow up (T1). Patients were divided into two groups: Rapid disease progression ([RP], n 15) and Non-rapid disease progression ([NRP], n 17), according to the Mayo Clinic classification criteria. At T0, ADPKD patients underwent plasma sampling for quantitative analysis of h-miR-17-5p, h-miR-21-5p and h-miR-199a-5p microRNA expression, using the quantitative reverse transcriptase-polymerase chain reaction (qRT-PCR) method and a 3T magnetic resonance imaging (MRI), using an advanced MRI imaging protocol, for the quantification of total kidney volume (TKV), total perfusion volume (TPV) and total fibrotic volume (TFV). The expression of h-miR17-5p was higher (p < 0.05) in ADPKD patients with rapid disease progression. h-miR-17-5p, h-miR-21-5p and h-mir-199-5p showed a positive and significant correlation with the eGFR slope (mL/min/1.73 m2/year) (p < 0.05) but not with the eGFR at both T0 and T1. Both total fibrotic volume (cm3) and height-adjusted total fibrotic volume (cm3/m) were positively and significantly correlated to h-miR 21-5p and h-miR 199-5p (p < 0.05), but not to total kidney volume (cm3) and height-adjusted total kidney volume (cm3/m). The microRNAs we studied were associated with fibrosis and renal damage, suggesting their possible role as biomarkers able to identify ADPKD patients at high risk of disease progression regardless of the degree of kidney function, and therefore suitable for medical therapy, and may help uncovering new molecular mechanisms underlying cystogenesis.

#2862 Genetics and electron microscopy: complementary tools for management of focal segmental glomerulosclerosis?

Abstract Background and Aims Focal segmental glomerular sclerosis (FSGS) defines a histologic lesion that represents a pattern of injury associated to different pathologic conditions affecting both children and adults. It is classified into primary forms, presumably immune-mediated, secondary forms, determined by a maladaptive response to different conditions, genetic and undetermined forms. According to Mayo Clinic classification, primary and secondary forms can de differentiated using electron microscopy (EM), with a podocytes foot process effacement (FPE) ≥80% identifying primary forms. Better understanding the role of Genetics in FSGS and response to treatment in different forms is of vital importance in this rare and multifaceted condition. We analyzed a discrete cohort of patients with FSGS examined both with EM and genetic testing (GT). Main aims of our study were: 1) assessment of GT in primary and secondary forms; 2) analysis of response to therapy according to histologic and genetic classification. Method This is a retrospective observational study on patients that received a histological diagnosis of FSGS, including EM, and underwent NGS genetic testing from January 2020 to June 2023. Primary and secondary forms of FSGS were distinguished by degree of pedicle fusion at EM, according to Mayo Clinic classification. Clinical, laboratory, genetic data and used therapies were recorded. Complete response (CR) to therapy was defined as a reduction of proteinuria to 0.3 g/day, with normalization of albuminemia. Partial response (PR) was defined as halving proteinuria into a sub-nephrotic range and increasing albuminemia up to 3.5 g/dL. Absent response was defined as a persisting nephrotic range proteinuria. Results In the study period, 33 patients receiving a histologic diagnosis of FSGS with EM examination underwent NGS testing. Of these, 25 had available genetic results and were included. Ten patients (40%) were females, 15 (60%) were males; mean age was 40.8 years (SD ±14.5); 20 (80%) were Caucasian, 4 (16%) Black, 1 (4%) Asian. Based on EM, 11 (44%) were classified as primary forms, 14 (56%) as secondary forms. Twelve (48%) patients presented with nephrotic syndrome. Of these, 10 had a primary form. Seven (28%) had nephrotic-range proteinuria without hypoalbuminemia, 6 (24%) had subnephrotic range proteinuria. Six (54.5%) of the primary forms and 8 (57.1%) of the secondary forms had a positive genetic testing (p = 0.78). Among secondary forms, through GT, one diagnosis of Fabry disease and one of Tuberous Sclerosis were made in patients without clear systemic symptoms. Fabry disease was confirmed by presence of zebra bodies at EM. All primary forms received immunosuppressive therapies, all being rituximab-based regimens; all secondary forms received support therapy. Nine (81.1%) primary forms and 9 (64.3%) secondary forms had at least a PR to treatment (4 CR in primary group and 1 CR in secondary group) (p = 0.33). Among primary forms, 5 of 6 patients (83%) with a positive GT had at least a PR with a strong immunosuppressive therapy. In secondary forms group 4 of 8 patients (50%) had at least PR with offered support therapy. Fabry and Tuberous Sclerosis were offered specific treatments with benefit. Conclusion Percentage of positive GT did not differ in primary and secondary forms of FSGS as defined by Mayo Clinic classification. Thus a positive GT should not be considered a unique feature of secondary forms. GT was critical for an unexpected diagnosis of systemic hereditary diseases, thus enabling adequate target therapies. We believe GT should be offered to all patients with FSGS. In primary FSGS, defined according to EM, more than 80% of patients had at least a PR following immunosuppressive therapy, even in patients with positive GT. In conclusion, as clinical presentation and light microscopy are not sufficient for an accurate diagnosis, combining EM and GT is of utmost importance in proper classification and management of FSGS.

Scheimpflug Tomography as a Predictor of Corneal Edema After Phacoemulsification in Fuchs Endothelial Corneal Dystrophy.

To determine if Scheimpflug tomography pachymetry map and posterior elevation map patterns can predict the occurrence of corneal edema following uneventful phacoemulsification surgery in Fuchs endothelial corneal dystrophy (FECD). Observational prospective case-control study. Fifty FECD eyes (50 patients) with visually significant cataract: 25 with subclinical corneal edema (SCE) versus 25 without SCE. Preoperatively, FECD was clinically assessed, and only patients devoid of clinical corneal edema were enrolled. Utilizing the Mayo Clinic classification for subclinical corneal edema (SCE), eligible FECD eyes were stratified based on Scheimpflug imaging pachymetry map and posterior elevation map characteristics, including loss of regular isopachs, displacement of the cornea's thinnest point, and the presence of posterior surface depression, into two groups: Group A representing FECD with SCE, and Group B: FECD without SCE. One week postoperatively, clinical and tomographic evaluation was performed. Regression analysis was conducted to evaluate predictors of corneal edema after uneventful phacoemulsification surgery in both groups. All patients were successfully imaged before and 1 week after surgery. Visual acuity was significantly improved in both groups (P < 0.001). No postoperative clinical edema was observed in Group B, while 23 (92%) had mild edema and 2 (8%) had moderate edema in Group A. Both groups showed a significant increase in postoperative central corneal thickness (CCT) and thinnest corneal thickness (TCT) (both P < 0.001). Compared to Group B, Group A showed a significant central flattening of the anterior corneal surface (P = 0.007 and P = 0.04 for K1 and K2 respectively), and a significant increase in the postoperative posterior surface depression. Multivariate analysis showed that 94% of postoperative corneal edema could be predicted by the presence of preoperative posterior surface depression (P = 0.04, ARR = 5.8 (1.89-35.7)). Scheimpflug tomography pachymetry map and posterior elevation map patterns can predict corneal edema after uneventful phacoemulsification surgery in FECD with subclinical corneal edema.

A Decade of Robotic-Assisted Radical Nephrectomy with Inferior Vena Cava Thrombectomy: A Systematic Review and Meta-Analysis of Perioperative Outcomes.

Open radical nephrectomy with inferior vena cava thrombectomy (O-CT) is standard management for renal cell carcinoma with inferior vena cava thrombus. First reported a decade ago, robotic-assisted radical nephrectomy with inferior vena cava thrombectomy (R-CT) is a minimally invasive option for this disease. We aimed to perform a systematic review to assess the safety and feasibility of R-CT in terms of perioperative outcomes and compare the outcomes between R-CT and O-CT. The PubMed®, Scopus®, Cochrane Central Register of Controlled Trials and Web of ScienceTM databases were searched using the free-text and MeSH terms "renal cell carcinoma," "inferior vena cava," "thrombosis" or "thrombus," "robot" and "thrombectomy." Studies reporting perioperative outcomes of R-CT and studies comparing R-CT with O-CT were included. The review was done in accordance with PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. The search retrieved 28 articles describing R-CT, including 7 comparative studies. This systematic review included 1,375 patients, out of which 329 patients were in single-arm studies and 1,046 patients were in comparative studies. Of the 329 patients who underwent R-CT, 14.7% were level I, 60.9% level II, 20.4% level III and 2.5% level IV thrombus. Operative time ranged from 150 to 530 minutes; blood transfusion was administered in 38.2% (126). The overall complication rate was 30.3% (99). R-CT, in comparison to O-CT, was associated with a lower blood transfusion rate (18.4% vs 64.3%, p=0.002) and a lower complication rate (14.5% vs 36.7%, p=0.005). Major complication and 30-day mortality rates were similar in both groups. R-CT has acceptable perioperative outcomes in carefully selected patients. Compared with O-CT, R-CT is associated with a lower blood transfusion rate and fewer overall complications. In experienced hands with carefully selected patients, R-CT is feasible and safe, with acceptable outcomes; however, selection bias limits definitive inference of these results, and optimal patient selection criteria remain to be described.

MO027: Clinical efficacy of treatment with tolvaptan in patients with autosomal dominant polycystic kidney disease

Abstract BACKGROUND AND AIMS Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. The only treatment approved today to slow its progression is tolvaptan (vasopressin antagonist). However, there is no clinical efficacy parameter for follow-up during treatment. To assess the clinical efficacy of treatment based on analytical (Fg) and radiological parameters: total kidney volume adjusted to height (TKV h). METHOD Retrospective observational study was undertaken of patients with ADPKD on treatment with tolvaptan after its approval in Spain in 2017. RESULTS Of the 182 ADPKD, 18% (n = 33) started treatment. The mean follow-up time of patients was 39.85 ± 10.04 months. Of the patients included; 60.6% (n = 20) were male, age 47.64 ± 8.64 years, a baseline glomerular filtration rate of 65.24 ± 20.14 mL/min and a height-adjusted total baseline renal volume of 1137.45 ± 667.58 mL. Baseline Mayo Clinic classification: 1C (72.7%), 1D (15.2%) and 1E (12.1%). Baseline CKD stages were as follows: 1: 15.2% (n = 5), 2:48.5% (n = 16), 3a:18.2% (n = 6), a 12.1% (n = 4) and 6.1% stage 4 (n = 2). The variation of Fg (mL/min), with respect to baseline was as follows: 1, 3, 6, 12, 24 and 36 months: (−4.26 ± 5.65; −3.54 ± 4.52; −1.68 ± 5.71, −10.89 ± 21.65, −10.54 ± 12.64, −8.08 ± 7.5). Fg decreased at months 1, 3, 6, 12, 12, 24 and 36 by 72.7%, 67.7%, 44.8%, 70.8%, 70.8%, 85% and 85.7%, the latter follow-up period being reached in 14 patients of the sample studied. Despite the decrease in Fg, 100% of the patients at 36 months had a higher Fg than expected according to the Mayo Clinic Fg prediction model, in accordance with their TKV at baseline. A mean decrease in TKVh was observed in annual control periods, the mean being as follows: −80.8 ± 290, −14.7 ± 187 and −82.8 ± 415.8 mL. In 100% of the patients with control at the third year (17 patients of the total sample), the percentage increase in TKV was lower than expected according to the Mayo Clinic model. CONCLUSION Although there is no efficacy parameter in the follow-up of treatment with tolvaptan in patients with ADPKD, our study shows an improvement in the expected value of both Fg and TKV; therefore, it could be considered an effective drug.

Epidemiological, Lesional, Therapeutic and Evolutionary Aspects of Olecranon Fractures

Introduction: Olecranon fractures are the most frequent articular fractures of the elbow. Objective: The aim of our work is to study the epidemiological, lesional, therapeutic and evolutionary aspects of olecranon fractures in the Orthopaedic-Traumatology Department of Aristide Le Dantec Hospital in Dakar. Patients and methods: This was a retrospective study over a period of 4 years, from January 1, 2014 to December 31, 2017, including 32 patients operated for a recent olecranon fracture using the pinning-hooking technique. Results: The series included 21 patients, 18 males and 3 females with an average age of 34 years (extremes 21 and 62 years). Road and traffic accidents were the leading cause with 09cases ie 43 percent. According to the Mayo Clinic classification, we observed 04 type I fractures (03 type IA and 01 type IB), 17 type II fractures (13 type IIA and 4 type IIB). The associated lesions were: skin openings with 03 cases (type I of Cauchoix and Duparc); section of the ulnar nerve; osteochondral and tendon fractures and lesions involving the elbow, forearm, radius and humerus. The average delay to consultation surgery was 13 days. Evaluation according to the modified Mayo Clinic criteria showed 80% good and excellent results and 20% fair results. The average follow-up was 3 years. Complications included 1 case of infection, 3 cases of secondary displacement, 2 cases of stiffness and 1 case of mal union. Conclusion: The treatment of olecranon fractures remains surgical and the technique of tension band wiring is the gold standard.

Prognostic significance of extent of venous tumor thrombus in patients with non-metastatic renal cell carcinoma: Results from a Canadian multi-institutional collaborative.

The prognostic significance of level of venous tumor thrombus (VTT) extension in patients with non-metastatic renal cell carcinoma (RCC) has been controversial. The aim of this study was to examine the prognostic significance of VTT extent in patients who underwent surgery for non-metastatic RCC. The Canadian Kidney Cancer information system database was used to identify patients who underwent surgery for non-metastatic RCC and VTT from January 2011 to December 2019. Association between VTT level and recurrence-free survival (RFS), cancer-specific survival (CSS) and overall survival (OS) was examined. Univariable and multivariable analyses were performed to estimate predictors of survival. Out of 6,340 patients during the study period, 228 patients (3.6%) had VTT. VTT was level 0 in 84 (37%), level I to II in 112 (49%), and level III to IV in 33 (14%) patients as per the Mayo Clinic classification. Median age was 65.4 years (interquartile range [IQR] 57.6-72.2) and 169 (74.1%) were male. After a median follow-up of 21.2 months, VTT level did not significantly impact the RFS, CSS, or OS. For VTT level 0, I to II, and III to IV, there was no significant difference in estimated 5-year RFS (31%, 23%, and 30.5%; P > 0.05), CSS (70%, 69%, and 55%; P > 0.05) and OS (64%, 66%, and 50%; P > 0.05). Adjusting for known prognostic factors, thrombus level was not associated with risk of recurrence or death. In a large, multi-institutional cohort of patients undergoing surgery for non-metastatic RCC with tumor thrombus, thrombus extent was not independently associated with recurrence or death.

P-120: Improving the risk stratification of multiple myeloma with a nucleotide editor/inflammation-based scoring system

<h3>Background</h3> Current clinical prognostication of newly diagnosed MM (NDMM) patients relies on clinical parameters and/or recurrent genetic changes, which mainly reflect early events in the development of MM. However, recent insights into the pathogenesis of MM highlighted genome/transcriptome editing through APOBEC genes as well as inflammation as drivers for the onset and progression of MM. <h3>Aims</h3> To build a superior nucleotide editor/inflammation-based risk scoring system reflecting biological processes that drive the progression of MM. <h3>Method</h3> We hypothesized that a prognostic score reflecting biological processes as well as clinical features is superior to the currently used classification systems for MM patients, such as ISS, R-ISS and the Mayo clinic classification. The Multiple Myeloma Research Foundation CoMMpass study genomics dataset, combining mRNA Seq and clinical data from more than 700 patients, allowed us to evaluate the prognostic value of demographic and clinical parameters, cytogenetics, and gene expression levels of APOBEC family as well as inflammation-modulating cytokines of MM patients. We calculated hazard ratios and Kaplan-Meier survival estimates for all extracted features. Combining clinical variables that were significantly associated with PFS and OS, we then applied machine learning approaches to identify the most accurate classification model to define a new risk score that is easy to compute and able to stratify NDMM patients more accurately than cytogenetics-based classifiers. <h3>Result</h3> Based on our machine learning models, we defined a weighted OS/PFS risk score (Editor-Inflammation score) based on expression of APOBEC2, APOBEC3B, IL11, TGFB1, TGFB3, as well as β-2-microglobulin and LDH serum levels, that achieved the best classification outcome and showed superior performance compared to ISS and R-ISS. Of note, cytogenetic abnormalities did not proof relevant have therefore not been included in the EI score. Besides superior overall risk stratification, the EI score further allowed to identify subgroups of MM patients with very good prognosis who do not significantly benefit from bone marrow transplantation maintenance therapy. <h3>Conclusion</h3> Our findings support the adoption of molecular biomarkers, reflecting dynamic biological processes rather than cytogenetics for a more accurate risk classification of MM. Considering that mRNA-seq is as cost effective as FISH and is being increasingly adopted by diagnostic centers, the EI score is a unique approach to shed light on underlying molecular mechanisms that drive disease progression and the development of true risk-adapted treatment strategies.

Cerebral Microbleeds May Be Less Detectable by Susceptibility Weighted Imaging MRI From 24 to 72 Hours After Traumatic Brain Injury.

Purpose: A former rodent study showed that cerebral traumatic microbleeds (TMBs) may temporarily become invisible shortly after injury when detected by susceptibility weighted imaging (SWI). The present study aims to validate this phenomenon in human SWI.Methods: In this retrospective study, 46 traumatic brain injury (TBI) patients in various forms of severity were included and willingly complied with our strict selection criteria. Clinical parameters potentially affecting TMB count, Rotterdam and Marshall CT score, Mayo Clinic Classification, contusion number, and total volume were registered. The precise time between trauma and MRI [5 h 19 min to 141 h 54 min, including SWI and fluid-attenuated inversion recovery (FLAIR)] was individually recorded; TMB and FLAIR lesion counts were assessed. Four groups were created based on elapsed time between the trauma and MRI: 0–24, 24–48, 48–72, and >72 h. Kruskal–Wallis, ANOVA, Chi-square, and Fisher’s exact tests were used to reveal differences among the groups within clinical and imaging parameters; statistical power was calculated retrospectively for each comparison.Results: The Kruskal–Wallis ANOVA with Conover post hoc analysis showed significant (p = 0.01; 1−β > 0.9) median TMB number differences in the subacute period: 0–24 h = 4.00 (n = 11); 24–48 h = 1 (n = 14); 48–72 h = 1 (n = 11); and 72 h ≤ 7.5 (n = 10). Neither clinical parameters nor FLAIR lesions depicted significant differences among the groups.Conclusion: Our results demonstrate that TMBs on SWI MRI may temporarily become less detectable at 24–72 h following TBI.

Predicting Future Renal Function Decline in Patients with Autosomal Dominant Polycystic Kidney Disease Using Mayo Clinic Classification

Introduction: Mayo clinic classification (MCC) has been proposed in patients with autosomal dominant polycystic kidney disease (ADPKD) to identify who may experience a rapid decline of renal function. Our aim was to validate this predictive model in a population from southern Spain. Methods: ADPKD patients with measurements of height-adjusted total kidney volume (HtTKV) and baseline estimated glomerular filtration rate (eGFR) >30 mL/min/1.73 m² were selected. Last eGFR was estimated with Mayo Clinic (MC) equation and bias and accuracy were studied. We also analyzed predictive capacity of MCC classes using survival analysis and Cox regression models. Results: We included 134 patients with a mean follow-up of 82 months. While baseline eGFR was not different between classes, last eGFR decreased significantly with them. eGFR variation rate was different according to the MCC class with a more rapid decline in 1C, 1D, and 1E classes. Final eGFR predicted was not significantly different from the real one, with an absolute bias of 0.6 ± 17.0 mL/min/1.73 m². P10 accuracy was low ranging from 37.5 to 59.5% in classes 1C, 1D, and 1E. Using MC equation, the rate of eGFR decline was underestimated in 1C, 1D, and 1E classes. Cox regression analysis showed that MCC class is a predictor of renal survival after adjusting with baseline eGFR, age, sex, and HtTKV, with 1D and 1E classes having the worst prognosis. Conclusion: MCC classification is able to identify patients who will undergo a more rapid decline of renal function in a Spanish population. Prediction of future eGFR with MC equation is acceptable as a group, although it shows a loss of accuracy considering individual values. The rate of eGFR decline calculated using MC equation can underestimate the real rate presented by patients of 1C, 1D, and 1E classes.

V11-05 MONOCENTRIC EXPERIENCE OF ROBOT-ASSISTED RADICAL NEPHRECTOMY WITH VENA CAVA THROMBECTOMY: TECHNIQUE AND RESULTS

You have accessJournal of UrologyRenal Oncology II (V11)1 Sep 2021V11-05 MONOCENTRIC EXPERIENCE OF ROBOT-ASSISTED RADICAL NEPHRECTOMY WITH VENA CAVA THROMBECTOMY: TECHNIQUE AND RESULTS Louis Gobinet, Lorenzo Tesi, Nam Son Vuong, Melanie Percot, Laure Dupitout, Helene Simeon, Eric Alezra, Franck Bladou, Gregoire Robert, Marine Gross Goupil, and Jean Christophe Bernhard Louis GobinetLouis Gobinet More articles by this author , Lorenzo TesiLorenzo Tesi More articles by this author , Nam Son VuongNam Son Vuong More articles by this author , Melanie PercotMelanie Percot More articles by this author , Laure DupitoutLaure Dupitout More articles by this author , Helene SimeonHelene Simeon More articles by this author , Eric AlezraEric Alezra More articles by this author , Franck BladouFranck Bladou More articles by this author , Gregoire RobertGregoire Robert More articles by this author , Marine Gross GoupilMarine Gross Goupil More articles by this author , and Jean Christophe BernhardJean Christophe Bernhard More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002073.05AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Open surgery remains the gold standard treatment for renal tumours with thrombus extension of the inferior vena cava (IVC), whereas the robotic approach has been developed by some expert teams. Two comparative studies are available in the literature. This video’s objective was to present the technique and results of robot-assisted radical nephrectomy with IVC thrombectomy. METHODS: We described the surgical technique of the robotic approach, carried out with the Da-Vinci SI (Intuitive Surgical) surgical robot and intra-operative ultrasound (Hitachi). We present the results of the comparative monocentric retrospective study, which included patients undergoing a radical nephrectomy with IVC thrombus from level 1 to 3 (Mayo clinic classification), between December 2015 and December 2019. Clinical data, collected after consent, come from the prospective French kidney cancer data base UroCCR (CNIL DR 2013-206, NCT03293563). Patients were divided into 2 groups, according to the surgical approach, open or robotic. We present univariate and multivariate analysis of perioperative morbidity and oncological findings. RESULTS: The patient, an 81-year-old man, presented an 8cm tumour of the upper and mid-pole of the right kidney, associated with a thrombus of the IVC. The CT-scan for extension work-up was negative. Operative time (OT) was 320 minutes, including 24 minutes of IVC clamping, with 300cc of blood loss. The length of hospital stay (LOS) was 4 days. Pathology report showed a Clear cell renal carcinoma, Fuhrman grade 4, pT3cN0M0.The open-approach group included 30 patients and the robotic group 10 patients. OT was significantly longer in the robotic group (350 vs 208 min (p<0.01) while blood loss (500 vs 1250 mL (p=0.02)) and LOS (7 Vs 10 days (p<0.01) were reduced. There was no significant difference in terms of postoperative complications or oncological results. CONCLUSIONS: The robotic approach for radical nephrectomy with IVC thrombectomy appears to be feasible and safe, for selected patients in expert centers. It leads to longer operative times, but reduced blood loss and shorter length of stay. Source of Funding: none © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e859-e859 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Louis Gobinet More articles by this author Lorenzo Tesi More articles by this author Nam Son Vuong More articles by this author Melanie Percot More articles by this author Laure Dupitout More articles by this author Helene Simeon More articles by this author Eric Alezra More articles by this author Franck Bladou More articles by this author Gregoire Robert More articles by this author Marine Gross Goupil More articles by this author Jean Christophe Bernhard More articles by this author Expand All Advertisement Loading ...

PKD2 gene variants in Chinese patients with autosomal dominant polycystic kidney disease.

PKD2 gene variants account for 4.5% to 20% of patients with autosomal dominant polycystic kidney disease (ADPKD). Little is known about the clinical characteristics of PKD2 variants in Chinese patients with ADPKD. Herein, we performed a comprehensive search for variants of PKD2 gene in 44 Chinese ADPKD pedigrees and a total of 37 variants were identified. Of these 37 variants, 18 were nonsense variants, 10 frameshift variants, 4 missense variants, and 5 splice site variants. 11/37 variants were detected for the first time. The median age at diagnosis was 30.5 years, and positive family history was detected in 77.27% patients, liver cysts in 68.18%, hypertension in 45.45%, nephrolithiasis in 31.82%, macro-hematuria in 22.73%, and proteinuria in 13.63%. The level of estimated glomerular filtration rate in 8/39 patients were blow 60 ml/min/1.73m2 . 11/17 patients were classified as rapid progression by Mayo Clinic classification. The end stage renal disease (ESRD) events were reported in 9/22 pedigrees, and the presence of nephrolithiasis and macro-hematuria were significantly associated with ESRD in the pedigrees with PKD2 variants. The identified variants and clinical features will facilitate the early diagnosis and prognosis prediction in Chinese ADPKD patients with PKD2 variants.

MO449VALIDATION OF MAYO CLINIC CLASSIFICATION AS A PREDICTOR OF FUTURE RENAL FUNCTION IMPAIRMENT IN PATIENTS WITH AUTOSOMAL POLYCYSTIC KIDNEY DISEASE (ADPKD) IN A POPULATION IN THE SOUTH OF SPAIN

Abstract Background and Aims The Mayo Clinic classification (MCC) is used in patients with ADPKD to identify those who may experience a more rapid deterioration of glomerular filtration rate (GFR). It has been developed in the American population but has not been validated in other populations. Our objective was to analyze whether the CCM predictive model is valid in an ADPKD population from southern Spain and whether it can identify populations with different renal survival. Method We selected patients with ADPKD with measurements of height-adjusted total renal volume (HtTKV) performed with CT or MR and with GFR CKD-EPI&amp;gt; 15 mL / min / 1.73 m2. We estimated the GFR at the end of the follow-up using the Irazábal equation and the rate of GFR deterioration, bias and precision were calculated. We analyzed the predictive power of BC using survival analysis using the Kaplan-Meier technique and using Cox regression models. Results We included 128 patients, aged 44 ± 13 years and a follow-up time of 79 ± 45 months (median 88), at the end of which 22 (17.2%) patients had a GFR &amp;lt;10 mL / min / 1.73 m2 or were included in renal replacement therapy. The distribution of patients according to the CM classification was: 1A 4.7%, 1B 28.1%, 1C 33.6%, 1D 22.7%, 1E 10.9%. Age decreased progressively: 1A 58 ± 11, 1B 48 ± 14, 1C 46 ± 13, 1D 40 ± 8, 1E 30 ± 7 (p &amp;lt;0.001). In contrast, HtTKV increased significantly: 1A 275 ± 52, 1B 486 ± 191, 1C 887 ± 410, 1D 1222 ± 510, 1E 1324 ± 800 mL / m (p &amp;lt;0.001). While the initial GFR was not different between classes, the GFR at the end of the follow-up decreased significantly: 1A 59 ± 36, 1B 63 ± 29, 1C54 ± 35, 1D 48 ± 26, 1E 44 ± 33 mL / min / 1.73 m2 (p &amp;lt;0.001). The GFR variation rate was significantly different according to the MCC classes: 1A 1.31 ± 6.80, 1B -2.48 ± 3.12, 1C -4.13 ± 4.33, 1D -4.70 ± 2.66, 1E -6.18 ± 3.03 mL / min / 1.73 m2 / year (p = 0.008). The final GFR predicted with the Irazábal equation was not significantly different from the real one. The absolute bias of the final GFR estimated with the MC equation was 2.6 ± 16.0 mL / min / 1.73 m2 and the relative bias was 38.7 ± 110, and it was not significantly different in the MCC classes. The P10 precision was low, with values of 65.1%, 51.7% and 50% for classes 1C, 1C and 1E respectively. The rate of deterioration of the GFR was underestimated in classes 1C, 1D and 1E. In the renal survival analysis with the Cox regression analysis, we found that the MCC classification is a predictor of survival, with classes 1D and 1E having the worst prognosis. Conclusion The MCC classification is capable of identifying populations that will suffer a more rapid deterioration of the GFR and constitutes a marker of renal survival in a Spanish population of patients with ADPKD. The prediction of future GFR with the Irazábal equation is acceptable as a group, although it shows a loss of precision at the individual level, especially in patients with higher GFR at baseline.

MO025TREATMENT WITH TOLVAPTAN IN ADPKD PATIENTS: RESULTS FROM AN OBSERVATIONAL, MULTICENTRIC STUDY

Abstract Background and Aims In recent years, treatment of ADPKD (Autosomal Dominant Polycystic Kidney of the Adult) has been based on the antisecretive and antiproliferative effect of several drugs. Tolvaptan, a vasopressin receptor antagonist, has been shown to slow disease progression in the face of side effects such as aquaresis and liver damage. Currently, the drug's reimbursability is allowed in patients aged up to 55 years, CKD 2-4 (GFR ≥ 25 ml/min) with evidence of rapid disease progression. Method We report the experience with Tolvaptan in 62 patients with ADPKD who were recruited from 11 Nephrology Units in Lazio (Italy). Baseline characteristics of patients are reported in Table 1. Renal volume was evaluated by MRI (26 patients), CT scan (3 patients), or ultrasonography (28 patients). Rapid progression’s criteria were the following: Mayo Clinic classification, PRO-PKD score and the annual loss of GFR. Mean duration of Tolvaptan’s treatment was 522 days (range 56-867). The Tolvaptan dosage used at the beginning of therapy was 60 mg/day in 95% of patients; at the end of follow-up, 37% were still taking 60 mg/day, while 38.7% were taking 90 mg/day and 16.1% were taking 120 mg/day. Results Median diuresis was 5-6 liters over 24 hours. A mean reduction in GFR of 12.5% (which was maintained for the next 12 months) was observed after initiation of therapy as a likely effect of tubulo-glomerular feedback. Monthly blood tests were performed to monitor side effects (hyper/hyponatremia, increased bilirubin and/or transaminases, etc.). Therapy was withdrawn by 2 patients (3.2%) due to aquaresis, while it was discontinued in 3 patients (4.8%) according to clinicians’ decision due to hepatic damage (3-fold increase in transaminases and/or bilirubin normal values). No significant alteration in natremia was observed in any patient. An increase in CK was observed in one patient, leading to momentary discontinuation of therapy. One patient withdraw therapy because of CKD’s progression to stage 5 after 2 years of therapy. Conclusion Results of this observational study show that Tolvaptan therapy was well tolerated and the incidence of liver damage was superimposed on that described in the literature. It will be necessary to continue clinical observation over the time and apply Tolvaptan’s therapy to a larger population in order to assess its effects on disease progression.

Clinical outcomes of radical surgery in patients with renal carcinoma and associated venous thrombosis: Single-centre experience in a tertiary care institution.

Renal carcinoma and associated venous thrombosis cause specific perioperative and postoperative challenges. We aimed to evaluate the factors affecting clinical outcomes in patients undergoing radical surgery because of renal carcinoma and associated venous thrombosis. Hospital records were retrospectively reviewed to identify patients with renal carcinoma and associated venous thrombosis treated with radical surgery between 2006 and 2019. Preoperative, perioperative, and postoperative findings were analysed to determine the associations between clinical and survival outcomes. Overall and disease-free survival were analysed by the Kaplan-Meier method. Other associated prognostic variables were assessed using univariate and multivariate Cox regression analyses. Thirty-three patients with renal carcinoma and associated venous thrombosis were enrolled for this study. There were 15 (45.4%) patients with level I, five (15.2%) with level II, eight (24.2%) with level III, and five (15.2%) with level IV venous thrombosis according to the Mayo Clinic classification system. The median follow-up was 35.6months. In the univariate analysis, increased tumour size was associated with poor overall and disease-free survival. Preoperative clinic M1 disease was associated with poor overall survival. A high Mayo Clinic thrombus level was associated with poor disease-free survival. In the multivariate analysis, only tumour size and clinic M1 disease were independently correlated with poor overall survival. No independent statistically significant association was detected between thrombus level and survival outcomes. Although the thrombus level was not associated with overall and disease-free survival, tumour size and clinic M1 disease were found to have an independent prognostic impact on overall survival.

Randall-type monoclonal immunoglobulin deposition disease: description of cardiac involvement

Abstract Background Randall-type monoclonal immunoglobulin disease (MIDD) is a rare complication of a monoclonal plasma cell clone. MIDD differs from AL amyloidosis by the presence of Congo red negative non-organized immunoglobulin (Ig) deposits, most commonly light chains (LCDD) along basement membranes and sometimes heavy chains (HCDD) or light and heavy chains (LHCDD). As AL amyloidosis MIDD is a multi-systemic disease, and affects the heart. To date no study has focused on the clinical characteristics of heart disease in MIDD. Purpose The aim of this study was to describe the cardiologic features of patients with biopsy-proven MIDD and suspected cardiac involvement. Methods This multi-center, nation-wide retrospective study extracted from the database of the French reference center for AL amyloidosis and other Ig deposition diseases between 2012 to 2019. Diagnosis of cardiac involvement was assessed according to the International Society of Amyloidosis criteria for amyloid heart disease, as follows: left ventricular hypertrophy with a diastolic septum thickness ≥12mm, NTproBNP serum level ≥332 ng / L, histological evidence on cardiac or extra cardiac biopsy of typical linear non-organized Ig deposits along basement membranes. Severity was defined according to the Mayo Clinic classification for AL amyloidosis. Results Among 20 patients included (mean age was 70±9 years), 11 (55%) were males; 13 (65%) were LCDD, 3 (15%) HCDD and 4 (20%) LHCDD. At diagnosis, 19 (95%) had a history of hypertension, 3 (16%) had atrial fibrillation, 3 (15%) had NYHA grade 3 or 4 dyspnea. Mayo Clinic score was stage 3a in 4 patients (20%) and stage 3b in 6 patients (30%). The most frequent ECG changes were microvoltage (40%) and pseudo Q wave (40%); 64% of patients had altered sinus variability on 24-hour Holter monitoring, one patient had a high-grade conduction disorder and another had ventricular tachycardia. On echocardiography, all showed diastolic dysfunction; mean diastolic septum thickness was 13.5mm; only one patient had LVEF impairment but 38% had global longitudinal strain impairment. 10 patients had cardiac MRI, none showed contrast enhancement after gadolinium injection. After median follow-up of 28 months, 4 patients were hospitalized for heart failure, including 2 with cardiogenic shock. Seven (35%) patients died within a median of 10 months from diagnosis. Among patients with Mayo clinic stage 3 (a or b), 67% died within a median of 8 months from the diagnosis. Conclusions To our knowledge, we present the first case series dedicated to the description of cardiac parameters in MIDD patients with cardiac involvement. Except for MRI appearance of cardiac infiltration, these patients showed features close to that of AL amyloid heart disease. Overall prognosis appears seemingly poor in MIDD patients with Mayo Clinic stage 3 cardiac disease. Funding Acknowledgement Type of funding source: None

A case report of IgG4-related disease: an insidious path to the diagnosis through kidney, heart and brain

BackgroundIgG4-related disease, described around the years 2000 as a form of autoimmune pancreatitis, is now increasingly accepted as a systemic syndrome. The diagnosis is based on both comprehensive and organ-specific criteria. For the kidney, Mayo clinic classification and the guidelines of the Japanese Nephrology Society are used. Ultimately, together with parameters that characterize every organ or apparatus involved, the key element is the confirmation of growing levels of IgG4 in blood or in tissues.Case presentationWe describe a male patient with chronic renal failure associated to hypertension without proteinuria. IgG4-related disease was diagnosed through renal biopsy. After an initial positive response to steroids, he presented tinnitus, and histological assessment showed cerebral and subsequently cardiac damage, both IgG4-related. This case appears unique for the type of histologically documented cardiac and neurological parenchymal involvement, and at the same time, exemplifies the subtle and pernicious course of the disease. Frequently, blurred and non-specific signs prevail. Here, kidney damage was associated with minimal urinary findings, slowly progressive renal dysfunction and other factors that can be equivocated in the differential diagnosis. Neurological involvement was represented by tinnitus alone, while cardiac alterations were completely asymptomatic.ConclusionsThis report is representative of the neurological and cardiac changes described in the literature for IgG4-related disease, which may be correlated or not with the renal form and highlights the need, in some cases, of targeted therapeutic approaches. In addition to glucocorticoids, as in this case, rituximab may be necessary.

P2733Prognostic value of cardiac dysautonomia in AL amyloidosis

Abstract Introduction Cardiac involvement is the major prognostic factor in patients with light chain amyloidosis (AL). Cardiac dysautonomia can occur early in amyloidosis and can be assess by Iodine-123-metaiodobenzylguanidine (123I-MIBG) scintigraphy. Its prognostic value has been shown in TTR amyloidosis but is unknown in AL amyloidosis. We aimed to evaluate the prognosis impact of cardiac dysautonomia in patients with AL amyloidosis. Methods We carried out a prospective study in consecutive patients with biopsy-proven AL amyloidosis. All patients underwent clinical examination, EKG, echocardiography, cardiac MRI and biological tests. The 2012 Mayo clinic prognostic classification was calculated by using blood levels of NT-proBNP, cardiac T troponin and the differential of free light chains as recommended. The sympathetic cardiac innervation was assessed by using 123I-MIBGscintigraphy and measurement of the heart-to-mediastinum uptake ratio (late H/M) in the anterior view of the chest. A cardiac denervation was defined by late H/M &lt;1.8 4h after injection of 3 MBq/kg of 123I-MIBG. The primary end-point was all-cause mortality during follow-up. Results Fifty consecutive patients with AL amyloidosis were included. The median age was 68 years old [58–73]. By using both echocardiography and MRI, cardiac involvement was diagnosed in 33 patients (66%) and thirteen of these patients were NYHA class III or IV. By using Mayo clinic classification, patients were I, II, III and IV classes in 9 (18%), 14 (28%), 16 (32%) and 11 (22%) cases respectively. According to echocardiographic data, the median wall thickness of left ventricle was 13 mm [12–15]. The late H/M was 1.51 [1.33–1.67]. Cardiac denervation was found in 44 patients (88%). The 6 patients (12%) with a normal late H/M had no cardiac amyloidosis involvement. During a median follow-up of 24 months, 9 patients (18%) died. The area under the ROC curve of late H/M for predicting death was 0.74 (CI 95% 0.58–0.86). According to this curve, the best threshold was 1.44 and 7 of the 9 patients who died had late H/M ≤1.44. The figure shows the 2 year-survival according to late H/M. Late H/M ≤1.44 predicted all-cause death irrespective of the Mayo clinic classification: HR 8.0 (CI 95% 2.1–63) after adjustment on the Mayo clinic score (p=0.005). In addition, unplanned hospitalization for heart failure occurred in 8 patients with late H/M ≤1.44 versus 3 patients with late H/M &gt;1.44 (p=0.03). Survival according to late H/M Conclusion Late H/M ≤1.44 is predictive of adverse outcomes in patients with AL amyloidosis, independently of the Mayo Clinic prognostic classification.