In patients with tuberous sclerosis complex (TSC), renal complications are not limited to bleeding angiomyolipoma (AML); although rare, end-stage renal disease (ESRD) may occur. New treatments (e.g. mammalian target of rapamycin (m-Tor) inhibitors) for AML might influence the epidemiology of ESRD in patients with TSC. In France, 99 patients with TSC from the Renal Epidemiology and Information Network (REIN) registry and having undergone renal replacement therapy (RRT) between 2002 and 2016 were included in the present study. Additional data were collected from the patients' medical charts. The mean ± standard deviation age at RRT initiation was 48.4 ± 16.4 and 73.8% had a neurologic impairment. Fifty-four patients underwent kidney transplantation after an average of 23 ± 12.3 months on dialysis. Among the 61 patients with additional data the most common renal lesion was AML: 26.2% of the patients had isolated AML, and 26.2% had AML and renal cysts, 65.6% of patients had undergone nephrectomy, and 16.4% had undergone at least one embolization. None of the patients had been treated with an m-Tor inhibitor before dialysis. The graft survival rate was 92.5% at 5 years and 70.2% at 10 years. The present cohort study is the first to have assessed TSC patients on RRT from a national registry. Nephrectomy or embolization due to AML was the leading cause of ESRD in our cohort. By reducing the size of the AML, m-tor inhibitors might lower the risk of complications and thus reduce the number of patients with TSC requiring RRT.
Kidney transplantation is the best renal replacement therapy (medically and economically) for eligible patients with end-stage kidney disease. Studies in some French regions and in other countries suggest a lower access to the kidney transplant waiting listing and also to kidney transplantation, once waitlisted, for women. Using a mixed methods approach, this study aims to precisely understand these potential sex disparities and their causes. The quantitative study will explore the geographic disparities, compare the determinants of access to the waiting list and to kidney transplantation, and compare the reasons and duration of inactive status on the waiting list in women and men at different scales (national, regional, departmental, and census-block). The qualitative study will allow describing and comparing women’s and men’s views about their disease and transplantation, as well as nephrologists’ practices relative to the French national guidelines on waiting list registration. This type of study is important in the current societal context in which the reduction of sex/gender-based inequalities is a major social expectation.
Funding Acknowledgements Financial support: French society of cardiovascular imaging. Background The respective strength of the different mechanisms that could lead to significant secondary TR occurrence remains debated. Purpose The main objective of our study was to characterize the determinants of tricuspid regurgitation (TR) severity in stable patients with preserved left ventricle ejection fractionand without significant left valvular heart disease. Patients were classified into 5 groups according to echocardiographic TR severity assessment: mild / moderate / severe / massive / torrential. Secondary objectives were to describe the evolution of the dimensions of the right cavities and the right ventricle (RV) function parameters according to the severity of the TR. Methods This is a prospective observational and multicentric study. Criteria for inclusion were: age ≥ 18 years; at least moderate TR; euvolemic status. We excluded patients with organic TR. All patients underwent standard trans-thoracic echocardiographyat distance from initial diagnosis and after stable optimized medical treatment. All exams were analysed in a Core Laboratory. Results 100 patients (12 presented mild TR, 31 moderate, 18 severe, 17 massive and 22 torrential) were enrolled and we used effective regurgitant orifice area (EROA) to quantify the severity of TR. To explain TR severity in multivariate analysis, right atrium (RA) indexed volume and tethering area were statistically significant (p < 0.001). For an increase of 10 mL/m2of RA volume, EROA increases by 4.2 mm2and for an increase of 0.1 cm2of tethering area, EROA increases by 2.2 mm2. The right heart dilation and the degree of restriction of tricuspid apparatus change significantly according to the severity of the TR (p < 0.001). RV function parameters did not differ significantly according to the degree of TR. Conclusion Early TA enlargement is present in secondary isolated TR. Then, increase in tethering area and RA indexed volume are associated with increase in TR severity. All right heart cavities dilate progressively without clear impact on RV-function parameters when TR increase. Factor determining increase in EROA coefficient SD p value RA volume (mL/m2) 0.42 0.09 < 0.001 Tethering area (cm2) 22.1 3.9 < 0.001 Multivariate linear regression model. EROA: Effective Regurgitant Orifice Area; SD: Standard Deviation; RA: Right Atrium. Abstract P1277 Figure. RV-EDA: probability of TR class severity
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