Onset Of Renal Replacement Therapy Research Articles

#699 Diagnostic accuracy of the Modified Renal Angina Index (mRAI) for predicting acute kidney injury among critically ill Filipino adults

Abstract Background and Aims The proposed mRAI (modified renal angina index) (Fig. 1) predicts moderate to severe AKI within the first 7 days of ICU stay which outperforms serum creatinine and urine output. The use of the mRAI in AKI may be warranted to improve predicted performance of AKI biomarkers. The study aims to evaluate the diagnostic accuracy of modified renal angina index in predicting Acute Kidney Injury (AKI) among Critically ill Adult Filipino patient in a Tertiary Institution and its association with in hospital MAKE (Major Adverse Kidney Events). Method A retrospective cohort study including all adult critically ill patients, they were dichotomized into “low” (<10) or “high risk” (>10) based on the mRAI score upon the 1st ICU admission (Day 0-24 hr) during the hospital stay. Inclusion criteria: age >18 years old, ICU patients between 2019-2022. Exclusion criteria: Chronic Kidney Disease (Egfr <30 ml/min), Transplant recipients, HIV/Cancer, <48 hrs ICU stay, little changes in creatinine (<2x from baseline between Day 0-24 hr ICU admission). The composite outcome is achieved if at least one is met during the index of hospitalization: All-cause mortality prior to discharge or after ICU admission, New onset of renal replacement therapy (RRT), persistent renal dysfunction. The sensitivity, specificity, predictive values and area under the curve is used to determine diagnostic accuracy of the mRAI Scoring and its association with in Hospital MAKE. Simple and multiple logistic regression analysis is used to control the effect of confounders in determining the association of mRAI to AKI. SPSS version 20 will be used in the analysis. Results Using a cut off score of 10, the mRAI show 84.29% sensitivity, 76.06% specificity, 77.63%, PPV 83.08% NPV 80.14% and AUC 80.14% in predicting moderate to severe AKI (Fig. 2). A mRAI of >10 is associated with in hospital MAKE showing a 97.14% sensitivity, 67.61% specificity, 74.73% PPV, 96.0% NPV, and AUC 82.27% (Fig. 3). Among the high risk group there were 68 (97.1%) who had MAKE, 62 (88.6%) new onset RRT, 12% (17.1%) died within 7 days of ICU admission, 64 (91.4%) persistent renal dysfunction (Fig. 4). Conclusion The proposed score can be applied among critically ill adult Filipino patients which is an easy bedside tool for identification of moderate to severe AKI. mRAI constitutes a sensitive test with a high negative predictive, affirming its capability being a screening test, complementary to laboratory parameters and clinical judgement. We also found out that a mRAI score of >10 is associated with In-Hospital Major Adverse Kidney Events.

Comparison of balanced and unbalanced crystalloids as resuscitation fluid in patients treated for cardiogenic shock

BackgroundThe efficacy and safety of saline versus balanced crystalloid solutions in ICU-patients remains complicated by exceptionally heterogenous study population in past comparative studies. This study sought to compare saline and balanced crystalloids for fluid resuscitation in patients with cardiogenic shock with or without out-of-hospital cardiac arrest (OHCA).MethodsWe retrospectively analyzed 1032 propensity score matched patients with cardiogenic shock from the Munich University Hospital from 2010 to 2022. In 2018, default resuscitation fluid was changed from 0.9% saline to balanced crystalloids. The primary endpoint was defined as 30-day mortality rate.ResultsPatients in the saline group (n = 516) had a similar 30-day mortality rate as patients treated with balanced crystalloids (n = 516) (43.1% vs. 43.0%, p = 0.833), but a higher incidence of new onset renal replacement therapy (30.2% vs 22.7%, p = 0.007) and significantly higher doses of catecholamines. However, OHCA-patients with a lactate level higher than 7.4 mmol/L had a significantly lower 30-day mortality rate when treated with saline (58.6% vs. 79.3%, p = 0.013). In addition, use of balanced crystalloids was independently associated with a higher mortality in the multivariate cox regression analysis after OHCA (hazard ratio 1.43, confidence interval: 1.05–1.96, p = 0.024).ConclusionsIn patients with cardiogenic shock, use of balanced crystalloids was associated with a similar all-cause mortality at 30 days but a lower rate of new onset of renal replacement therapy. In the subgroup of patients after OHCA with severe shock, use of balanced crystalloids was associated with a higher mortality than saline.Trial registration: LMUshock registry (WHO International Clinical Trials Registry Platform Number DRKS00015860).

The Usefulness of Calcium/Magnesium Ratio in the Risk Stratification of Early Onset of Renal Replacement Therapy.

Background: A growing number of studies have reported a close relationship between high serum calcium (Ca)/low serum magnesium (Mg) and vascular calcification. Endothelial dysfunction and vascular inflammation seem plausible risk factors for the enhanced progression of kidney disease. The aim of this study was to evaluate the role of the Ca/Mg ratio as a predictor of the early onset of renal replacement therapy (RRT). Methods: This was a prospective study conducted in an outpatient low-clearance nephrology clinic, enrolling 693 patients with stages 4–5 of CKD. Patients were divided into two groups according to the start of renal replacement therapy (RRT). Results: The kidney’s survival at 120 months was 60% for a Ca–Mg ratio < 6 and 40% for a Ca–Mg ratio ≥ 6 (p = 0.000). Patients who started RRT had lower levels of Hb, Ca, Mg, albumin, and cholesterol and higher values of phosphorus, the Ca/Mg ratio, and PTH. High values of phosphorus and the Ca/Mg ratio and low levels of Mg and GFR were independent predictors of entry into RRT. A high Ca/Mg ratio, high phosphorus levels, and low levels of GFR were associated with a cumulative risk for initiation of RRT. Conclusions: In our population, the Ca/Mg ratio is an independent predictive factor for the initiation of a depurative technique.

MO336: Renal Recovery After Acute Kidney Injury Associated to COVID Requiring CRRT During ICU Hospitalization

BACKGROUND AND AIMSRenal recovery (RR) after AKI is a determinant outcome of future comorbidity and mortality in critical care patients. Related predictive factors remain uncertain.METHODWe retrospectively analyzed patients admitted to ICU between January 2020 and February 2021 from our critical nephrology database.We analyzed adult patients with diagnosis of AKI (KDIGO criteria) treated with renal replacement therapy (RRT) during ICU hospitalization. We excluded patients with dialysis support previous to the admission.The main outcomes we evaluated were(1) RR (successful suspension of RRT without hyperkalemia, increase in serum creatinine (SCr), hypervolemia or acidemia after 1 week without RRT, with urine volume > 500 mL/d without diuretic treatment or > 2000 mL/d with diuretics).(2) Mortality during hospitalization.RESULTSWe found 1442 patients were admitted to ICU, 418 presented AKI (29.8%), of them, 178 patients (64% male) required RRT (AKI-RRTd) in ICU during follow-up, with mean age of 66 year old (52.8% >65 year). Main comorbidity and demographic data are in Table 1. Mean time in ICU was 19 days (RIC 11–35). The most frequent admission cause was non-surgical pathologies (93%), 53% of admitted patients had COVID-19 as main diagnosis (95 patients). There was need of vasoactive support in 73.6%, ventilatory support (82.6) and 67.2% of patients had fluid overload.The indication of dialysis was determined by a nephrologist: mainly oliguria, acidosis, hyperkalemia, fluid overload and increase SCr. Mean SCR at admission was 2.5 mg/dL. There were missing data in 48% of basal SCr (known SCr between 1 and 12 months prior to admission). Total mortality in AKI-RRTd was 70.8% (126 patients). In COVID patients, was 77.9% (74 patients).We found renal recovery in 63.4% of total survivors (33/52 patients). When analyzing COVID, there were 21 survivors, and we found renal recovery in 80.9% of patients.Patients who did not achieved renal recovery had longer ICU stay (median: 20 days, RIC: 4–26) and inhospitalization (median: 41 days, RIC: 29–58). Those patients were older, and had higher morbidity (diabetes), higher SCr at ICU admission and lower urine output. Their fluid balance was higher at 48 h after CRRT initiation (OR 3.05, 95% CI 1.39–6.65, P <.01). In COVID population without renal recovery, there were more urgent dialysis onset (OR 8.33, 95% confidence interval (95% CI) 1.04–66.2; P = .04), age > 65 year (OR 6.48, 95% CI 1.94–21.6; P < .01), positive fluid balance at 48 h after RRT (OR 3.25; 95% CI 1.09–9.69; P = .03).The risk factors for mortality, were age > 65 year (OR 4.14, 95% CI 2.05–8.35; P < .01), mechanical ventilation (OR 3.28, 95% CI 1.48–7.30; P < .01), haemodynamic support (OR 4.37, 95% CI 2.14–8.92; P < .01). Otherwise, lower SCr at admission (OR 0.82, 95% CI 0.71–0.93; P < .01) and at instauration of RRT (OR 0.75, 95% CI 0.065–0.88; P < .01) were associated to lower mortality.In COVID patients, fluid overload at RRT initiation (OR 10.83, 95% CI 1.37–85.36; P = .02), age > 65 year old (OR 8.85, 95% CI 2.68–29.1; P < .01) and FiO2 > 50% at RRT start (OR 2.77, 95% CI 1.02–7.50; P = .04) were associated to higher mortality.CONCLUSIONIn ICU patients with AKI-RRT dependence, negative fluid balance at 48 h after RRT onset and in COVID patients, age < 65 year old, negative fluid balance at 48 h after RRT onset and non-urgent onset of RRT were related with renal recovery.

MO451: The Usefulness of Calcium/Magnesium Ratio in the Risk Stratification of Early Onset of Renal Replacement Therapy

Abstract BACKGROUND AND AIMS Recently, a growing number of studies have reported a close relationship between high serum calcium and low serum magnesium with vascular calcification. Endothelial dysfunction and vascular inflammation seem plausible risk factors for enhanced progression of kidney disease nevertheless, the knowledge remains scarce. The aim of this study is to evaluate the role of the calcium/magnesium ratio as a risk factor in CKD progression. METHOD Observational, prospective study involving 693 patients (f = 371, m = 322) with stage 4 and 5 CKD. Patients were divided into two groups, according to the development of end-stage renal disease (ESRD): G1 (n = 541), who did not undergo renal replacement therapy and G2 (n = 152), who had started renal replacement therapy (RRT). Several laboratory parameters were measured: haemoglobin, eGFR (MDRD), albumin, cholesterol, magnesium and mineral metabolism markers (PTH, calcium and phosphorus). Baseline characteristics were analysed and compared using Student%u2019s T-test for continuous variables and chi-squared test for categorical variables. Multivariate Cox regression analysis was used to identify independent factors associated with RRT initiation. A modified Poisson regression with robust error variance was used to estimate the cumulative relative risk for RRT initiation. RESULTS The mean age and estimated glomerular filtration rate (eGFR) of the study population was 70.09 ± 12.51 years and 19.91 ± 8.11 mL/min, respectively. Comparing the two groups, G2 had a significantly lower serum levels of Hb (11.75 versus 10.95 g/dL, P = 0.000), calcium (9.34 versus 8.95 mg/dL, P = 0.000), magnesium (1.92 versus 1.40 mg/dL, P = 0.0001), albumin (4.00 versus 3.88 g/dL, P = 0.03) and cholesterol (183.17 versus 172.39 mg/dL, P = 0.01) and a higher serum levels of phosphorus (3.88 versus 4.69 mg/dL, P = 0.0001), calcium/magnesium ratio (5.73 versus 7.56, P = 0.0001) and PTH (209.71 versus 338.84 pg/mL, P = 0.0001). In univariate Cox regression analysis, age, haemoglobin, eGFR, calcium, magnesium, phosphorus, calcium-magnesium ratio and PTH correlate with onset of RRT, which were further tested using a multivariate COX regression model. The results showed a clear relationship between high levels of phosphorus (HRa = 1.638, P = 0.001) and calcium-magnesium ratio (HRa = 1.292; P = 0.002), and low levels of magnesium (HRa = 0.761, P = 0.005) and eGFR (HRa = 0.934; P = 0.0001) were independent risk factors to start depurative techniques. Additionally, Poisson regression analysis showed that high calcium-magnesium ratios (aPR = 1.986; 95% CI 1.026–3.051; P = 0.002), high phosphorus levels (aPR = 1.607; 95% CI 1.324–1.950; P &amp;lt; 0.0001) and low levels eGFR (aPR = 0.927; 95% CI 0.891–0.964; P &amp;lt; 0.0001) were associated with a cumulative risk for initiation of RRT. CONCLUSION Our results suggest that the calcium/magnesium ratio is an independent predictive factor for the initiation of renal replacement therapy. Further studies are required to validate the use of this novel marker as a predictor of CKD progression.

The Usefulness of Calcium/Magnesium Ratio in the Risk Stratification of Early Onset of Renal Replacement Therapy

The Usefulness of Calcium/Magnesium Ratio in the Risk Stratification of Early Onset of Renal Replacement Therapy

Effect of comorbidities on survival in patients >80 years of age at onset of renal replacement therapy: data from the ERA-EDTA Registry.

The number of elderly patients on renal replacement therapy (RRT) is increasing. The survival and quality of life of these patients may be lower if they have multiple comorbidities at the onset of RRT. The aim of this study was to explore whether the effect of comorbidities on survival is similar in elderly RRT patients compared with younger ones. Included were 9333 patients ≥80 years of age and 48 352 patients 20-79 years of age starting RRT between 2010 and 2015 from 15 national or regional registries submitting data to the European Renal Association-European Dialysis and Transplantation Association Registry. Patients were followed until death or the end of 2016. Survival was assessed by Kaplan-Meier curves and the relative risk of death associated with comorbidities was assessed by Cox regression analysis. Patients ≥80 years of age had a greater comorbidity burden than younger patients. However, relative risks of death associated with all studied comorbidities (diabetes, ischaemic heart disease, chronic heart failure, cerebrovascular disease, peripheral vascular disease and malignancy) were significantly lower in elderly patients compared with younger patients. Also, the increase in absolute mortality rates associated with an increasing number of comorbidities was smaller in elderly patients. Comorbidities are common in elderly patients who enter RRT, but the risk of death associated with comorbidities is less than in younger patients. This should be taken into account when assessing the prognosis of elderly RRT patients.

Low protein diets for non-diabetic adults with chronic kidney disease.

Low protein diets for non-diabetic adults with chronic kidney disease.

New mutation associated with autosomal dominant polycystic kidney disease with founder effect located in the Alpujarra region of Granada

ObjectiveTo demonstrate that the variant not described in PKD1 gene c.7292T> A, identified in four families from the Alpujarra in Granada, is the cause of autosomal dominant polycystic kidney disease (ADPKD). This variant consists of a transversion of thymine (T) by adenine (A) that at the level of the Polycystin 1 protein produces a change of leucine (Leu/L) by Glutamine (Gln/Q) in position 2431 (p.Leu2431Gln). MethodSociodemographic and clinical variables were registered using clinical histories, genealogical trees, ultrasounds and genetic analysis to ADPKD and healthy individuals belonging to these families in the context of segregation study. ResultsAll PKD individuals carried the c.7292T> A variant in heterozygosis, whereas healthy ones did not. Among all ADPKD patients, 62.9% were women. ADPKD diagnosis was made at 29.3 ± 15.82 years, after having the first child in 64.8%. The main reasons for diagnosis were family history and hematuria episodes. The onset of renal replacement therapy (RRT) occurred at 55.8 ± 7.62 years (range 44–67), and death at 63 ± 92.2 years (range 48–76), being the cause unknown, cardiovascular and insufficiency kidney the most frequent; the median of renal survival was established at 58.5 ± 0.77 years and the median survival of patients at 67.2 ± 3.54 years. No differences in kidney and patient survivals were observed according to sex. Among deceased patients, 52.2% required RRT and 94.4% suffered from renal failure. ConclusionsThe variant c.7292T> A in PKD1 gene is responsible for the disease, and its distribution in the Alpujarra region of Granada suggests a founder effect. In ADPKD it is necessary to perform segregation studies that help us to reclassify genetic variants, in this case from indeterminate to pathogenic.

The ERA-EDTA Registry Annual Report 2017: a summary.

BackgroundThis article presents a summary of the 2017 Annual Report of the European Renal Association–European Dialysis and Transplant Association (ERA-EDTA) Registry and describes the epidemiology of renal replacement therapy (RRT) for end-stage renal disease (ESRD) in 37 countries.MethodsThe ERA-EDTA Registry received individual patient data on patients undergoing RRT for ESRD in 2017 from 32 national or regional renal registries and aggregated data from 21 registries. The incidence and prevalence of RRT, kidney transplantation activity and survival probabilities of these patients were calculated.ResultsIn 2017, the ERA-EDTA Registry covered a general population of 694 million people. The incidence of RRT for ESRD was 127 per million population (pmp), ranging from 37 pmp in Ukraine to 252 pmp in Greece. A total of 62% of patients were men, 52% were ≥65 years of age and 23% had diabetes mellitus as the primary renal disease. The treatment modality at the onset of RRT was haemodialysis for 85% of patients. On 31 December 2017, the prevalence of RRT was 854 pmp, ranging from 210 pmp in Ukraine to 1965 pmp in Portugal. The transplant rate in 2017 was 33 pmp, ranging from 3 pmp in Ukraine to 103 pmp in the Spanish region of Catalonia. For patients commencing RRT during 2008–12, the unadjusted 5-year patient survival probability for all RRT modalities combined was 50.8%.

Using machine learning models to predict the initiation of renal replacement therapy among chronic kidney disease patients.

Starting renal replacement therapy (RRT) for patients with chronic kidney disease (CKD) at an optimal time, either with hemodialysis or kidney transplantation, is crucial for patient’s well-being and for successful management of the condition. In this paper, we explore the possibilities of creating forecasting models to predict the onset of RRT 3, 6, and 12 months from the time of the patient’s first diagnosis with CKD, using only the comorbidities data from National Health Insurance from Taiwan. The goal of this study was to see whether a limited amount of data (including comorbidities but not considering laboratory values which are expensive to obtain in low- and medium-income countries) can provide a good basis for such predictive models. On the other hand, in developed countries, such models could allow policy-makers better planning and allocation of resources for treatment. Using data from 8,492 patients, we obtained the area under the receiver operating characteristic curve (AUC) of 0.773 for predicting RRT within 12 months from the time of CKD diagnosis. The results also show that there is no additional advantage in focusing only on patients with diabetes in terms of prediction performance. Although these results are not as such suitable for adoption into clinical practice, the study provides a strong basis and a variety of approaches for future studies of forecasting models in healthcare.

Nueva mutación asociada a poliquistosis renal autosómica dominante con efecto fundador localizada en la Alpujarra de Granada

ObjectiveTo demonstrate that the variant not described in PKD1 gene c.7292T> A, identified in four families from the Alpujarra in Granada, is the cause of autosomal dominant polycystic kidney disease (ADPKD). This variant consists of a transversion of thymine (T) by adenine (A) that at the level of the Polycystin 1 protein produces a change of leucine (Leu / L) by Glutamine (Gln / Q) in position 2431 (p.Leu2431Gln). MethodSociodemographic and clinical variables were registered using clinical histories, genealogical trees, ultrasounds and genetic analysis to ADPKD and healthy individuals belonging to these families in the context of segregation study. ResultsAll PKD individuals carried the c.7292T>A variant in heterozygosis, whereas healthy ones did not. Among all ADPKD patients, 62.9% were women. ADPKD diagnosis was made at 29.3 ± 15.82 years, after having the first child in 64.8%. The main reasons for diagnosis were family history and hematuria episodes. The onset of renal replacement therapy (RRT) occurred at 55.8 ± 7.62 years (range 44-67), and death at 63 ± 92.2 years (range 48-76), being the cause unknown, cardiovascular and insufficiency kidney the most frequent; the median of renal survival was established at 58.5 ± 0.77 years and the median survival of patients at 67.2 ± 3.54 years. No differences in kidney and patient survivals were observed according to sex. Among deceased patients, 52.2% required RRT and 94.4% suffered from renal failure. ConclusionsThe variant c.7292T>A in PKD1 gene is responsible for the disease, and its distribution in the Alpujarra region of Granada suggests a founder effect. In ADPKD it is necessary to perform segregation studies that help us to reclassify genetic variants, in this case from indeterminate to pathogenic.

Sex Disparities in Risk of Mortality Among Children With ESRD

In the general population, girls have lower mortality risk compared with boys. However, few studies have focused on sex differences in survival and in access to kidney transplantation among children with end-stage kidney disease. Retrospective cohort study. Children aged 2 to 19 years registered in the US Renal Data System who started renal replacement therapy (RRT) between 1995 and2011. Study participant sex. Time to death and time to kidney transplantation. We used adjusted Cox models to examine the association between sex and all-cause mortality. We used Fine-Gray models to examine the association between sex and kidney transplantation accounting for the competing risk for death. We included 14,024 children, of whom 1,880 died during a median 7.1 years of follow-up. Inadjusted analyses, the HR for death was higher for girls (HR, 1.36; 95% CI, 1.25-1.50) than boys. When we further adjusted our survival models for transplantation as a time-dependent covariate, the hazard rate of death in girls was partially attenuated but remained statistically significantly higher than that for boys (HR, 1.28; 95% CI, 1.17-1.41). Girls were also less likely to receive a kidney transplant than boys (adjusted subdistribution HR, 0.91; 95% CI, 0.88-0.95) in analyses treating death as a competing risk. Lack of data for disease course before the onset of RRT and observational study data. The mortality rate was substantially higher for girls than for boys treated with RRT. Access to transplantation was lower for girls than boys, but differences in transplantation access accounted for only a small proportion of the survival differences by sex.

Low protein diets for non-diabetic adults with chronic kidney disease.

Low protein diets for non-diabetic adults with chronic kidney disease.

Factors associating with differences in the incidence of renal replacement therapy among elderly: data from the ERA-EDTA Registry.

The incidence of renal replacement therapy (RRT) in the general population ≥75years of age varies considerably between countries and regions in Europe. Our aim was to study characteristics and survival of elderly RRT patients and to find explanations for differences in RRT incidence. Patients ≥75years of age at the onset of RRT in 2010-2013 from 29 national or regional registries providing data to the European Renal Association-European Dialysis and Transplant Association Registry were included. Chi-square and Mann-Whitney U tests were used to assess variation in patient characteristics and linear regression was used to study the association between RRT incidence and various factors. Kaplan-Meier curves and Cox regression were employed for survival analyses. The mean annual incidence of RRT in the age group ≥75 years of age ranged from 157 to 924 per million age-related population. The median age at the start of RRT was higher and comorbidities were less common in areas with higher RRT incidence, but overall the association between patient characteristics and RRT incidence was weak. The unadjusted survival was lower in high-incidence areas due to an older age at onset of RRT, but the adjusted survival was similar [relative risk 1.00 (95% confidence interval, 0.97-1.03)] in patients from low- and high-incidence areas. Variation in the incidence of RRT among the elderly across European countries and regions is remarkable and could not be explained by the available data. However, the survival of patients in low- and high-incidence areas was remarkably similar.

The epidemiology of renal replacement therapy in two different parts of the world: the Latin American Dialysis and Transplant Registry versus the European Renal Association-European Dialysis and Transplant Association Registry.

ABSTRACTObjectiveTo compare the epidemiology of renal replacement therapy (RRT) for end-stage renal disease (ESRD) in Latin America and Europe, as well as to study differences in macroeconomic indicators, demographic and clinical patient characteristics, mortality rates, and causes of death between these two populations.MethodsWe used data from 20 Latin American and 49 European national and subnational renal registries that had provided data to the Latin American Dialysis and Renal Transplant Registry (RLADTR) and the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry, respectively. The incidence and prevalence of RRT in 2013 were calculated per million population (pmp), overall and by subcategories of age, sex, primary renal disease, and treatment modality. The correlation between gross domestic product and the prevalence of RRT was analyzed using linear regression. Trends in the prevalence of RRT between 2004 and 2013 were assessed using Joinpoint regression analysis.ResultsIn 2013, the overall incidence at day 91 after the onset of RRT was 181 pmp for Latin American countries and 130 pmp for European countries. The overall prevalence was 660 pmp for Latin America and 782 pmp for Europe. In the Latin American countries, the annual increase in the prevalence averaged 4.0% (95% confidence interval (CI): 2.5%-5.6%) from 2004 to 2013, while the European countries showed an average annual increase of 2.2% (95% CI: 2.0%-2.4%) for the same time period. The crude mortality rate was higher in Latin America than in Europe (112 versus 100 deaths per 1 000 patient-years), and cardiovascular disease was the main cause of death in both of those regions.ConclusionsThere are considerable differences between Latin America and Europe in the epidemiology of RRT for ESRD. Further research is needed to explore the reasons for these differences.

Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry

BackgroundAs outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry data.MethodsData were available for 88 male PBS patients aged <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD).ResultsMedian age at onset of RRT in PBS was lower [7.0; interquartile range (IQR) 0.9–12.2 years] than in COU (9.6; IQR: 3.0–14.1 years) and RHD (9.4; IQR: 2.7–14.2 years). Unadjusted 10-year patient survival was 85% for PBS, 94% for COU, and 91% for RHD. After adjustment for country, period, and age, PBS mortality was similar to that of RHD but higher compared with COU [hazard ratio (HR) 1.96, 95% confidence interval (CI) 1.03–3.74]. Seventy-four PBS patients (84%) received a first kidney transplant after a median time on dialysis of 8.4 (IQR 0.0–21.1) months. Outcomes with respect to time on dialysis before transplantation, chance of receiving a first transplant within 2 years after commencing RRT, and death-censored, adjusted risk of graft loss were similar for all groups.ConclusionsThis study in the largest cohort of male patients with PBS receiving RRT to date demonstrates that outcomes are comparable with other congenital anomalies of the kidney and urinary tract, except for a slightly higher mortality risk compared with patients with COU.

Prolonged renal survival in light chain amyloidosis: speed and magnitude of light chain reduction is the crucial factor

Renal involvement causing progressive chronic kidney disease (CKD) is present in 70% of patients with systemic Iglight-chain (AL) amyloidosis at diagnosis. Chemotherapy thatsubstantially suppresses free light chain production is associated with improved patient survival, but its benefit in delaying the onset of renal replacement therapy among patients who present with established advanced CKD has notbeen studied. To evaluate this, we studied 1000 patients enrolled in the prospective UK AL amyloidosis chemotherapy study (ALchemy). Of these, 84 patients had advanced amyloid-related CKD defined by an estimated glomerular filtration rate (eGFR) under 20 ml/min/1.73 m2. We determined outcomes among these 84 patients, who had a median eGFR of 10 ml/min/1.73 m2, in relation to response to chemotherapy evaluated at three, six, and 12 months from baseline. Patients who achieved suppression of 90% or more in their amyloidogenic free light chain (dFLC) within three months of baseline had significantly better overall survival, prolonged time to dialysis, and prolonged time to the composite endpoint of 'death or dialysis' compared to those who achieved lesser degrees of clonal response at the same time point. Even when this target of greater than 90% dFLC response was achieved but was delayed beyond 3 months, it was associated with worse outcomes. Cox regression analyses confirmed that a 90% or better dFLC response within 3 months was the only significant independent predictor of all three of these outcome measures. Thus, renal survival among patients with systemic immunologic light chain amyloidosis who present with advanced CKD is strongly dependent upon the magnitude and speed with which the underlying hematologic disorder is suppressed by chemotherapy.

Long-term consequences of renal insufficiency in children: lessons learned from the Dutch LERIC study.

Few data exist on the prospects in adulthood for children on chronic renal replacement therapy (RRT). This article summarizes the results of a comprehensive Dutch long-term follow-up study performed in 2000 and 2010 of patients with RRT onset at age <15 years between 1972 and 1992. After a median of 25.5 RRT years, patients had stayed 23% of RRT time on dialysis. We observed a 30 times greater mortality risk compared with age-matched peers with cardiovascular disease (CVD) as the main cause of death during 1972-2000 and infections during 2000-10. The observed shift towards infections was associated with more RRT time with a graft and receiving a stricter CVD protective treatment. For patients >40 years of age, motor disabilities affecting routine activities, skin cancer and severe fatigue were the most disabling sequelae. After 30 years of transplantation, 41% of the survivors had developed cancer, a life-threatening form of squamous cell skin carcinoma being most prevalent. Important delays in autonomy development and educational attainment and a relatively high level of unemployment were observed. Transplanted patients reported a good mental and physical quality of life, but the latter tended to decrease over time. A long period of dialysis was associated with all adverse somatic and psychosocial outcomes. Paediatric nephrologists should aim for transplantation at the earliest possible time and focus on autonomy and educational attainment. Nephrologists should focus on strict CVD prevention, adjustment of immunosuppression to the lowest possible dose and surveillance of malignancy-associated viral infections in patients with childhood end-stage renal disease.

Progression of chronic renal disease in a reference hospital of social security of Peru 2012-2015

To describe the characteristics of the population with chronic kidney disease (CKD) stage 3 and 4, to determine the factors associated with CKD progression and admission to renal replacement therapy (RRT), as well as renal survival. Longitudinal retrospective study of patients referred between January 2012 and December 2015 to the Renal Health Unit of the Hospital Nacional Edgardo Rebagliati Martins (HNERM), who were evaluated and followed by a multidisciplinary team. The clinical and laboratory data for each query were recorded in a software created specifically for the program. A multivariate logistic regression analysis was performed to assess the factors associated with the progression of CKD, a Cox regression model to predict the risk of entering RRT and the Kaplan-Meier method for renal survival analysis. We assessed 1248 patients in stage 3A: 248 (20%), stage 3B: 548 (44%) and stage 4: 452 (36%). 352 (28%) progressed, being proteinuria the most important progression factor (OR: 3.2; CI 95%: 2,2-4.6). Proteinuria increases the risk of admission to RRT in four times and having a glomerular filtration rate < 30% in 3.6 times. Median follow-up was 12 months (RIC 5-27 months). 92 patients (7%) required to initiate RRT. Renal survival at 12 months of follow-up was 96% and at 24 months was 90%. Our study shows that in a specialized center a significant proportion of patients with CKD does not progress in their disease and that the factor that is most associated with progression of disease and at the onset of RRT is proteinuria.