Bundgaardgates5614

yndrome.

Our patients showed clear evidence of biallelic LAMA5 truncating variants causing infantile nephrotic syndrome. We also discerned the clinical and pathologic characteristics observed in LAMA5-related nephropathy. LAMA5 variant screening should be performed in patients with congenital/infantile nephrotic syndrome.

Long-term arteriovenous fistula (AVF) survival has been shown to be adversely affected by the presence of previous tunneled vascular catheters (TVC). We analyzed the effect of previous TVCs and their location (ipsilateral versus contralateral) on the successful function of upper-limb AVFs in the first 12 months after creation.

We retrospectively reviewed clinical data on patients' first upper-limb AVFs, created between January 2013 and December 2017. We analyzed the rates of successful AVF function (successful cannulation using two needles for ≥50% sessions over a 2-week period) at 6 and 12 months after creation, time to AVF maturation, and rates of assisted maturation.

In total, 287 patients with first AVFs were identified, of which 142 patients had a previous TVC (102 contralateral, 40 ipsilateral) and 145 had no previous TVC. The no TVC group had higher rates of AVF function at both 6 months (69% versus 54%, OR, 1.84; 95% CI, 1.00 to 3.39,

=0.05) and 12 months (84% versus 64%, OR, 3.10; 95% CI, 1.5lateral TVC was associated with lower successful AVF use at 6 months, compared with contralateral TVC.

Primary nephrotic syndromes are rare diseases which can impede adequate sample size for observational patient-oriented research and clinical trial enrollment. A computable phenotype may be powerful in identifying patients with these diseases for research across multiple institutions.

A comprehensive algorithm of inclusion and exclusion ICD-9 and ICD-10 codes to identify patients with primary nephrotic syndrome was developed. The algorithm was executed against the PCORnet CDM at three institutions from January 1, 2009 to January 1, 2018, where a random selection of 50 cases and 50 noncases (individuals not meeting case criteria seen within the same calendar year and within 5 years of age of a case) were reviewed by a nephrologist, for a total of 150 cases and 150 noncases reviewed. The classification accuracy (sensitivity, specificity, positive and negative predictive value, F1 score) of the computable phenotype was determined.

The algorithm identified a total of 2708 patients with nephrotic syndrome frory nephrotic syndrome utilizing only ICD-9 and ICD-10 codes, which are available across institutions in the United States. This may facilitate future screening and enrollment for research studies and enable comparative effectiveness research. Further refinements to the algorithm including use of laboratory data or addition of natural language processing may help better distinguish primary and secondary causes of nephrotic syndrome.

Hemodialysis arteriovenous fistulas (AVFs) are the preferred vascular access for patients on hemodialysis. In the Hemodialysis Fistula Maturation Study, 44% of the patients achieved unassisted maturation of their fistula without needing an intervention. Venous neointimal hyperplasia (VNH) and subsequent venous stenosis are responsible for lack of maturation. There are no therapies that can prevent VNH/VS formation. The goal of this paper is to present the background, rationale, and trial design of an innovative phase 1/2 clinical study that is investigating the safety of autologous adipose-derived mesenchymal stem cells delivered locally to the adventitia of newly created upper extremity radiocephalic (RCF) or brachiocephalic fistula (BCF).

The rationale and preclinical studies used to obtain a physician-sponsored investigational new drug trial are discussed. The trial design and end points are discussed.

This is an ongoing trial that will complete this year.

This is a phase 1/2 single-center, randomized trial that will investigate the safety and efficacy of autologous AMSCs in promoting maturation in new upper-extremity AVFs.

NCT02808208.

This is a phase 1/2 single-center, randomized trial that will investigate the safety and efficacy of autologous AMSCs in promoting maturation in new upper-extremity AVFs.Clinical Trial registration number NCT02808208.

Accessibility to dialysis facilities plays a central role when deciding on a patient's long-term dialysis modality. Studies investigating the effect of distance to nearest dialysis-providing unit on modality choice have yielded conflicting results. We set out to investigate the association between patients' dialysis modality and both the driving and straight-line distances to the closest HD- and PD-providing units.

All patients with ESKD who initiated in-center HD and PD in 2017, were 18-90 years old, and were on dialysis for ≥30 days were included. Patients in residence zip codes in nonconterminous United States or lived >90 miles from the nearest HD-providing unit were excluded.

A total of 102,247 patients in the United States initiated in-center HD and PD in 2017. Compared with patients on HD, patients on PD had longer driving distances to their nearest PD unit (4.4 versus 3.4 miles;

<0.001). Patients who lived >30 miles from the nearest HD unit were more likely to be on PD if the nearest PD unit was a distance equal to/less than that of the HD unit. PD utilization increased with increasing distance from patients' homes to the nearest HD unit. No change in this association was found regardless of if the PD unit was farther from/closer than the nearest HD unit. This association was not seen with straight-line distance analysis.

With increasing distances from the nearest dialysis-providing units (HD or PD), PD utilization increased. Using driving distance rather than straight-line distance affects data analysis and outcomes. Increasing the number of PD units may have a limited effect on increasing PD utilization.

With increasing distances from the nearest dialysis-providing units (HD or PD), PD utilization increased. Using driving distance rather than straight-line distance affects data analysis and outcomes. Increasing the number of PD units may have a limited effect on increasing PD utilization.

Variation in dose and duration of corticosteroids for childhood-onset steroid-sensitive nephrotic syndrome occurs worldwide, likely reflecting the evolving evidence on optimal dosing and variable severity of the disease observed between patients. We conducted a study to determine the associations between site, physician, and patient factors, and average daily corticosteroid dose and duration of therapy.

Data were derived from the Canadian Childhood Nephrotic Syndrome (CHILDNEPH) Project, an observational longitudinal study from 2013 to 2019 of children with nephrotic syndrome involving pediatric nephrologists in 11 sites across Canada. The primary outcome was average daily corticosteroid dose prescribed per episode of proteinuria, reported as mg/m

prednisone equivalents. Secondary outcome was duration of treatment for each episode of proteinuria in days. Exposure variables were categorized into site-, physician-, and patient-level variables.

In total, 328 children, median age at enrollment of 4.3 yearof therapy.

We found practice variation with respect to corticosteroid prescriptions across 11 Canadian sites, and that variation is mostly explained at the site level. Age and ethnicity are important factors to be considered, because they are significantly associated with the average corticosteroid dose and duration of therapy.

Historically, kidney transplantation has been considered inappropriate for most patients with AL amyloidosis-associated kidney failure because of concerns about recurrent disease in the allograft and poor long-term survival. With improvements in rates and durability of hematologic responses and survival that have accompanied treatment advances, a greater proportion of patients with AL amyloidosis may be suitable for kidney transplantation. However, there are no widely accepted criteria for kidney transplant eligibility for this patient population.

We administered surveys electronically to transplant nephrologists and amyloidosis experts at a geographically diverse set of academic medical centers in the United States. Questions were designed to elucidate views about suitability and timing of kidney transplantation for patients with AL amyloidosis-associated kidney failure.

The survey was completed by 20 (65%) of invited amyloidosis experts and 20 (29%) of invited transplant physicians. Respondents indicaociated kidney failure differed between amyloidosis and transplant nephrology program respondents, with amyloidosis specialists generally favoring a less-restrictive approach to transplant eligibility. The findings suggest a need for consensus building across specialties.

Views about criteria for kidney transplantation for patients with AL amyloidosis-associated kidney failure differed between amyloidosis and transplant nephrology program respondents, with amyloidosis specialists generally favoring a less-restrictive approach to transplant eligibility. The findings suggest a need for consensus building across specialties.Atmospheric aerosols have significant effects on the climate and on human health. New particle formation (NPF) is globally an important source of aerosols but its relevance especially towards aerosol mass loadings in highly polluted regions is still controversial. In addition, uncertainties remain regarding the processes leading to severe pollution episodes, concerning e.g. the role of atmospheric transport. In this study, we utilize air mass history analysis in combination with different fields related to the intensity of anthropogenic emissions in order to calculate air mass exposure to anthropogenic emissions (AME) prior to their arrival at Beijing, China. The AME is used as a semi-quantitative metric for describing the effect of air mass history on the potential for aerosol formation. We show that NPF events occur in clean air masses, described by low AME. However, increasing AME seems to be required for substantial growth of nucleation mode (diameter less then 30 nm) particles, originating either from NPF or direct emissions, into larger mass-relevant sizes. This finding assists in establishing and understanding the connection between small nucleation mode particles, secondary aerosol formation and the development of pollution episodes. We further use the AME, in combination with basic meteorological variables, for developing a simple and easy-to-apply regression model to predict aerosol volume and mass concentrations. Since the model directly only accounts for changes in meteorological conditions, it can also be used to estimate the influence of emission changes on pollution levels. We apply the developed model to briefly investigate the effects of the COVID-19 lockdown on PM2.5 concentrations in Beijing. While no clear influence directly attributable to the lockdown measures is found, the results are in line with other studies utilizing more widely applied approaches.Organic hydroperoxides (ROOHs) play key roles in the atmosphere as a reactive intermediate species. Due to the low volatility and high hydrophilicity, ROOHs are expected to reside in atmospheric condensed phases such as aerosols, fogs, and cloud droplets. The decomposition mechanisms of ROOHs in the liquid phase are, however, still poorly understood. Here we report a temperature-dependent kinetics and theoretical calculation study of the aqueous-phase decompositions of C12 or C13 α-alkoxyalkyl-hydroperoxides (α-AHs) derived from ozonolysis of α-terpineol in the presence of 1-propanol, 2-propanol, and ethanol. HOIPIN-8 purchase We found that the temporal profiles of α-AH signals, detected as chloride-adducts by negative ion electrospray mass spectrometry, showed single-exponential decay, and the derived first-order rate coefficient k for α-AH decomposition increased as temperature increased, e.g., k(288 K) = (5.3 ± 0.2) × 10-4 s-1, k(298 K) = (1.2 ± 0.3) × 10-3 s-1, k(308 K) = (2.1 ± 1.4) × 10-3 s-1 for C13 α-AHs derived from the reaction of α-terpineol Criegee intermediates with 1-propanol in the solution at pH 4.