Emergency start (ES) of dialysis has been associated with worse outcome, but remains poorly documented. This study aims to compare the profile and outcome of a large cohort of patients starting ...dialysis as an emergency or as a planned step in France.
Data on all patients aged 18 years or older who started dialysis in mainland France in 2012 or in 2006 were collected from the Renal Epidemiology and Information Network and compared, depending on the dialysis initiation condition: ES or Planned Start (PS). ES was defined as a first dialysis within 24 h after a nephrology visit due to a life-threatening event. Three-year survival were compared, and a multivariate model was performed after multiple imputation of missing data, to determine the parameters independently associated with three-year survival.
In 2012, 30.3% of all included patients (n = 8839) had ES. Comorbidities were more frequent in the ES than PS group (≥ 2 cardiovascular diseases: 39.2% vs 28.8%, p < 0.001). ES was independently associated with worse three-year survival (57% vs. 68.2%, p = 0.029, HR 1.10, 95% CI 1.01-1.19) in multivariate analysis. Among ES group, a large part had a consistent previous follow-up: 36.4% of them had ≥3 nephrology consultations in the previous year. This subgroup of patients had a particularly high comorbidity burden. ES rate was stable between 2006 and 2012, but some proactive regions succeeded in reducing markedly the ES rate.
ES remains frequent and is independently associated with worse three-year survival, demonstrating that ES deleterious impact is never overcome. This study shows that a large part of patients with ES had a previous follow-up, but high comorbidity burden that could favor acute decompensation with life-threatening conditions before uremic symptoms appearance. This suggests the need of closer end-stage renal disease follow-up or early dialysis initiation in these high-risk patients.
Etiology and Outcomes of Thrombotic Microangiopathies Bayer, Guillaume; von Tokarski, Florent; Thoreau, Benjamin ...
Clinical journal of the American Society of Nephrology,
04/2019, Letnik:
14, Številka:
4
Journal Article
Recenzirano
Odprti dostop
Thrombotic microangiopathies constitute a diagnostic and therapeutic challenge. Secondary thrombotic microangiopathies are less characterized than primary thrombotic microangiopathies (thrombotic ...thrombocytopenic purpura and atypical hemolytic and uremic syndrome). The relative frequencies and outcomes of secondary and primary thrombotic microangiopathies are unknown.
We conducted a retrospective study in a four-hospital institution in 564 consecutive patients with adjudicated thrombotic microangiopathies during the 2009-2016 period. We estimated the incidence of primary and secondary thrombotic microangiopathies, thrombotic microangiopathy causes, and major outcomes during hospitalization (death, dialysis, major cardiovascular events acute coronary syndrome and/or acute heart failure, and neurologic complications stroke, cognitive impairment, or epilepsy).
We identified primary thrombotic microangiopathies in 33 of 564 patients (6%; thrombotic thrombocytopenic purpura: 18 of 564 3%; atypical hemolytic and uremic syndrome: 18 of 564 3%). Secondary thrombotic microangiopathies were found in 531 of 564 patients (94%). A cause was identified in 500 of 564 (94%): pregnancy (35%; 11 of 1000 pregnancies), malignancies (19%), infections (33%), drugs (26%), transplantations (17%), autoimmune diseases (9%), shiga toxin due to
(6%), and malignant hypertension (4%). In the 31 of 531 patients (6%) with other secondary thrombotic microangiopathies, 23% of patients had sickle cell disease, 10% had glucose-6-phosphate dehydrogenase deficiency, and 44% had folate deficiency. Multiple causes of thrombotic microangiopathies were more frequent in secondary than primary thrombotic microangiopathies (57% versus 19%;
<0.001), and they were mostly infections, drugs, transplantation, and malignancies. Significant differences in clinical and biologic differences were observed among thrombotic microangiopathy causes. During the hospitalization, 84 of 564 patients (15%) were treated with dialysis, 64 of 564 patients (11%) experienced major cardiovascular events, and 25 of 564 patients (4%) had neurologic complications; 58 of 564 patients (10%) died, but the rates of complications and death varied widely by the cause of thrombotic microangiopathies.
Secondary thrombotic microangiopathies represent the majority of thrombotic microangiopathies. Multiple thrombotic microangiopathies causes are present in one half of secondary thrombotic microangiopathies. The risks of dialysis, neurologic and cardiac complications, and death vary by the cause of thrombotic microangiopathies.
Autosomal-dominant polycystic kidney disease (ADPKD) is a common, progressive, adult-onset disease that is an important cause of end-stage renal disease (ESRD), which requires transplantation or ...dialysis. Mutations in PKD1 or PKD2 (∼85% and ∼15% of resolved cases, respectively) are the known causes of ADPKD. Extrarenal manifestations include an increased level of intracranial aneurysms and polycystic liver disease (PLD), which can be severe and associated with significant morbidity. Autosomal-dominant PLD (ADPLD) with no or very few renal cysts is a separate disorder caused by PRKCSH, SEC63, or LRP5 mutations. After screening, 7%–10% of ADPKD-affected and ∼50% of ADPLD-affected families were genetically unresolved (GUR), suggesting further genetic heterogeneity of both disorders. Whole-exome sequencing of six GUR ADPKD-affected families identified one with a missense mutation in GANAB, encoding glucosidase II subunit α (GIIα). Because PRKCSH encodes GIIβ, GANAB is a strong ADPKD and ADPLD candidate gene. Sanger screening of 321 additional GUR families identified eight further likely mutations (six truncating), and a total of 20 affected individuals were identified in seven ADPKD- and two ADPLD-affected families. The phenotype was mild PKD and variable, including severe, PLD. Analysis of GANAB-null cells showed an absolute requirement of GIIα for maturation and surface and ciliary localization of the ADPKD proteins (PC1 and PC2), and reduced mature PC1 was seen in GANAB+/− cells. PC1 surface localization in GANAB−/− cells was rescued by wild-type, but not mutant, GIIα. Overall, we show that GANAB mutations cause ADPKD and ADPLD and that the cystogenesis is most likely driven by defects in PC1 maturation.
Autosomal-dominant polycystic kidney disease (ADPKD) is a common, progressive, adult-onset disease that is an important cause of end-stage renal disease (ESRD), which requires transplantation or ...dialysis. Mutations in PKD1 or PKD2 (~85% and ~15% of resolved cases, respectively) are the known causes of ADPKD. Extrarenal manifestations include an increased level of intracranial aneurysms and polycystic liver disease (PLD), which can be severe and associated with significant morbidity. Autosomal-dominant PLD (ADPLD) with no or very few renal cysts is a separate disorder caused by PRKCSH, SEC63, or LRP5 mutations. After screening, 7%-10% of ADPKD-affected and ~50% of ADPLD-affected families were genetically unresolved (GUR), suggesting further genetic heterogeneity of both disorders. Whole-exome sequencing of six GUR ADPKD-affected families identified one with a missense mutation in GANAB, encoding glucosidase II subunit a (GIIa). Because PRKCSH encodes GIIβ, GANAB is a strong ADPKD and ADPLD candidate gene. Sanger screening of 321 additional GUR families identified eight further likely mutations (six truncating), and a total of 20 affected individuals were identified in seven ADPKD- and two ADPLD-affected families. The phenotype was mild PKD and variable, including severe, PLD. Analysis of GANAB-null cells showed an absolute requirement of GIIa for maturation and surface and ciliary localization of the ADPKD proteins (PC1 and PC2), and reduced mature PC1 was seen in GANAB+/- cells. PC1 surface localization in GANAB-/- cells was rescued by wild-type, but not mutant, GIIa. Overall, we show that GANAB mutations cause ADPKD and ADPLD and that the cystogenesis is most likely driven by defects in PC1 maturation.
The course of autosomal dominant polycystic kidney disease (ADPKD) varies among individuals, with some reaching ESRD before 40 years of age and others never requiring RRT. In this study, we developed ...a prognostic model to predict renal outcomes in patients with ADPKD on the basis of genetic and clinical data. We conducted a cross-sectional study of 1341 patients from the Genkyst cohort and evaluated the influence of clinical and genetic factors on renal survival. Multivariate survival analysis identified four variables that were significantly associated with age at ESRD onset, and a scoring system from 0 to 9 was developed as follows: being male: 1 point; hypertension before 35 years of age: 2 points; first urologic event before 35 years of age: 2 points; PKD2 mutation: 0 points; nontruncating PKD1 mutation: 2 points; and truncating PKD1 mutation: 4 points. Three risk categories were subsequently defined as low risk (0-3 points), intermediate risk (4-6 points), and high risk (7-9 points) of progression to ESRD, with corresponding median ages for ESRD onset of 70.6, 56.9, and 49 years, respectively. Whereas a score ≤3 eliminates evolution to ESRD before 60 years of age with a negative predictive value of 81.4%, a score >6 forecasts ESRD onset before 60 years of age with a positive predictive value of 90.9%. This new prognostic score accurately predicts renal outcomes in patients with ADPKD and may enable the personalization of therapeutic management of ADPKD.
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.
Background
The pre-dialysis care trajectory impact on post-dialysis outcomes is poorly known. This study assessed survival, access to kidney transplant waiting list and to transplantation after ...dialysis initiation by taking into account the patients’ pre-dialysis care consumption (inpatient and outpatient) and the conditions of dialysis start: initiation context (emergency or planned) and vascular access type (catheter or fistula).
Methods
Adults who started dialysis in France in 2015 were included. Clinical data came from the French REIN registry and data on the care trajectory from the French National Health Data system (SNDS). The Cox model was used to assess survival and access to kidney transplantation.
Results
We included 8856 patients with a mean age of 68 years. Survival was shorter in patients with emergency or planned dialysis initiation with a catheter compared to patients with planned dialysis with a fistula. The risk of death was lower in patients who were seen by a nephrologist more than once in the 6 months before dialysis than in those who were seen only once. The rate of kidney transplant at 1 year post-dialysis was lower for patients with emergency or planned dialysis initiation with a catheter (respectively, HR = 0.5 0.4; 0.8 and HR = 0.7 0.5; 0.9) compared to patients with planned dialysis start with a fistula. Patients who were seen by a nephrologist more than three times between 0 and 6 months before dialysis start were more likely to access the waiting list 1 and 3 years after dialysis start (respectively, HR = 1.3 1.1; 1.5 and HR = 1.2 1.1; 1.4).
Conclusions
Nephrological follow-up in the year before dialysis initiation is associated with better survival and higher probability of access to kidney transplantation. These results emphasize the importance of early patient referral to nephrologists by general practitioners.
Graphical abstract
Background Recent randomized trials report that mortality is lower with high-convection-volume hemodiafiltration (HDF) than with hemodialysis (HD). Study Design We used data from the French national ...Renal Epidemiology and Information Network (REIN) registry to investigate trends in HDF use and its relationship with mortality in the total population of incident dialysis patients. Setting & Participants The study included those who initiated HD therapy from January 1, 2008, through December 31, 2011, and were dialyzed for more than 3 months; follow-up extended to the end of 2012. Factor HDF use at the patient and facility level. Outcomes All-cause and cardiovascular mortality, using Cox models to estimate HRs of HDF as time-dependent covariate at the patient level, with age as time scale and fully adjusted for comorbid conditions and laboratory data at baseline, catheter use, and facility type as time-dependent covariates. Analyses completed by Cox models for HRs of the facility-level exposure to HDF updated yearly. Results Of 28,407 HD patients, 5,526 used HDF for a median of 1.2 (IQR, 0.9-1.9) years; 2,254 of them used HDF exclusively. HRs for all-cause and cardiovascular mortality associated with HDF use were 0.84 (95% CI, 0.77-0.91) and 0.73 (95% CI, 0.61-0.88), respectively. In patients treated exclusively with HDF, these HRs were 0.77 (95% CI, 0.67-0.87) and 0.66 (95% CI, 0.50-0.86). At the facility level, increasing the percentage of patients using HDF from 0% to 100% was associated with HRs for all-cause and cardiovascular mortality of 0.87 (95% CI, 0.77-0.99) and 0.72 (95% CI, 0.54-0.96), respectively. Limitations Observational study. Conclusions Whether analyzed as a patient- or facility-level predictor, HDF treatment was associated with better survival.
Background:
Effective collaboration between general practitioners (GP) and nephrologists is crucial in CKD care. We aimed to analyse GPs’ and nephrologists’ presence and involvement in CKD care and ...assess how they intertwine to shape patients’ trajectories.
Methods:
We conducted a mixed-methods study that included all patients with CKD who started dialysis in France in 2015 (the REIN registry) and a sample of nephrologists and GPs. We quantified professionals’ presence through patients’ reimbursed healthcare from the French National Health Data System, 2 years before and 1 year after dialysis start. Involvement in CKD care was derived from the nephrologists’ and GPs’ interviews.
Results:
Among 8856 patients included, nephrologists’ presence progressively increased from 29% to 67% of patients with a contact during the 2 years before dialysis start. However, this was partly dependent on the GPs’ referral practices. Interviews revealed that GPs initially controlled the therapeutic strategy on their own. Although unease grew with CKD’s management complexity, reducing their involvement in favour of nephrologists, GPs’ presence remained frequent throughout the pre-dialysis period. Upon dialysis start, nephrologists’ presence and involvement became total, while GPs’ greatly decreased (48% of patients with a contact at month 12 after dialysis start). Collaboration was smooth when GPs maintained contact with patients and could contribute to their care through aspects of their specialty they valued.
Conclusions:
This mixed-methods study shows presences and forms of involvement of GPs and nephrologists in CKD care adjusting along the course of CKD and unveils the mechanisms at play in their collaboration.
Pneumococcal immunization is recommended in dialysis patients. We aimed to estimate pneumococcal vaccination coverage among patients who initiate dialysis in France, and its association with ...mortality.
Data were extracted from two prospective national databases, merged using a deterministic linkage method: renal epidemiology and information network (REIN) registry, which includes all patients on dialysis and kidney transplants recipients in France, and the national health insurance information system (SNIIRAM) which collects individual data on health expenditure reimbursement, including vaccines. We enrolled all patients who initiated chronic dialysis in 2015. Data on health status at dialysis initiation, dialysis modalities, and pneumococcal vaccine prescribed from 2 years before to 1 year after dialysis start were collected. Univariate and multivariate Cox proportional hazard models were used to assess one-year all-cause mortality.
Among the 8,294 incident patients included, 1,849 (22.3 %) received at least one pneumococcal vaccine before (n = 542, 6.5 %), or after (n = 1,307, 15.8 %) dialysis start, as follows: 13-valent pneumococcal conjugated vaccine (PCV13) followed by 23-valent pneumococcal polysaccharide vaccine (PPSV23), n = 938 (50.7 %); only PPSV23, n = 650 (35.1 %); or only PCV13, n = 261 (14.1 %). Vaccinated patients were younger (mean, 66.5 ± 14.8 years vs. 69.0 ± 14.9 years, P ≤ 0.001), more likely to suffer from glomerulonephritis (17.0 % vs. 11.0 %, P ≤ 0.001), and less likely to start dialysis in emergency (27.2 % vs. 31.1 %, P = 0.001). On multivariate analysis, patients who received PCV13 and PPSV23, or only PCV13 were less likely to die (respectively, HR = 0.37; 95 %CI 0.28–0.51, and HR = 0.35; 95 %CI 0.19–0.65).
Pneumococcal immunization with PCV13 followed by PPSV23, or with PCV13 alone, but not with PPSV23 alone, is independently associated with decreased one year-mortality in patients who start dialysis.
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