Population-based identification and temporal trend of children with primary nephrotic syndrome: The Kaiser Permanente nephrotic syndrome study.
Ontology highlight
ABSTRACT: Introduction
Limited population-based data exist about children with primary nephrotic syndrome (NS).Methods
We identified a cohort of children with primary NS receiving care in Kaiser Permanente Northern California, an integrated healthcare delivery system caring for >750,000 children. We identified all children <18 years between 1996 and 2012 who had nephrotic range proteinuria (urine ACR>3500 mg/g, urine PCR>3.5 mg/mg, 24-hour urine protein>3500 mg or urine dipstick>300 mg/dL) in laboratory databases or a diagnosis of NS in electronic health records. Nephrologists reviewed health records for clinical presentation and laboratory and biopsy results to confirm primary NS.Results
Among 365 cases of confirmed NS, 179 had confirmed primary NS attributed to presumed minimal change disease (MCD) (72%), focal segmental glomerulosclerosis (FSGS) (23%) or membranous nephropathy (MN) (5%). The overall incidence of primary NS was 1.47 (95% Confidence Interval:1.27-1.70) per 100,000 person-years. Biopsy data were available in 40% of cases. Median age for patients with primary NS was 6.9 (interquartile range:3.7 to 12.9) years, 43% were female and 26% were white, 13% black, 17% Asian/Pacific Islander, and 32% Hispanic.Conclusion
This population-based identification of children with primary NS leveraging electronic health records can provide a unique approach and platform for describing the natural history of NS and identifying determinants of outcomes in children with primary NS.
SUBMITTER: Parikh RV
PROVIDER: S-EPMC8516311 | biostudies-literature |
REPOSITORIES: biostudies-literature
Similar Datasets
Project description:BackgroundThe Kaiser Permanente model of integrated health delivery is highly regarded for high-quality and efficient health care. Efforts to reproduce Kaiser's success have mostly failed. One factor that has received little attention and that could explain Kaiser's advantage is its commitment to and investment in nursing as a key component of organizational culture and patient-centered care.PurposeThe aim of this study was to investigate the role of Kaiser's nursing organization in promoting quality of care.MethodologyThis was a cross-sectional analysis of linked secondary data from multiple sources, including a detailed survey of nurses, for 564 adult, general acute care hospitals from California, Florida, Pennsylvania, and New Jersey in 2006-2007. We used logistic regression models to examine whether patient (mortality and failure-to-rescue) and nurse (burnout, job satisfaction, and intent-to-leave) outcomes in Kaiser hospitals were better than in non-Kaiser hospitals. We then assessed whether differences in nursing explained outcomes differences between Kaiser and other hospitals. Finally, we examined whether Kaiser hospitals compared favorably with hospitals known for having excellent nurse work environments-Magnet hospitals.FindingsPatient and nurse outcomes in Kaiser hospitals were significantly better compared with non-Magnet hospitals. Kaiser hospitals had significantly better nurse work environments, staffing levels, and more nurses with bachelor's degrees. Differences in nursing explained a significant proportion of the Kaiser outcomes advantage. Kaiser hospital outcomes were comparable with Magnet hospitals, where better outcomes have been largely explained by differences in nursing.ImplicationsAn important element in Kaiser's success is its investment in professional nursing, which may not be evident to systems seeking to achieve Kaiser's advantage. Our results suggest that a possible strategy for achieving outcomes like Kaiser may be for hospitals to consider Magnet designation, a proven and cost-effective strategy to improve process of care through investments in nursing.
| S-EPMC4892964 | biostudies-literature
Project description:Health systems and physicians nationwide aspire to consistently and reliably apply genetic and genomic information to guide disease prevention, management, and treatment. However, clinical information, including genetics/genomics data from within and outside of the care delivery system, is expanding rapidly. Between November 2017 and April 2018, we surveyed 1502 Permanente Medical Group primary care and specialist physicians to assess the degree to which direct-to-consumer genetic test results were being presented to physicians and identify genetics educational needs among physicians (response rate 15%). Adjusted logistic regression (according to respondent characteristics) was used to calculate adjusted odds ratios (ORs) and 95% confidence intervals (CIs) comparing responses within groups. Results showed 35% and 12% of respondents reported receiving at least one direct-to-consumer health risk genetic result (DTC-health risk) or direct-to-consumer pharmacogenomic test result (DTC-PGx), respectively, from a patient in the past year. Of those receiving at least one test result, 40% (DTC-health risk) and 39% (DTC-PGx) of physicians reported 1+ referral(s); 78% (DTC-health risk) and 42% (DTC-PGx) of referrals were to clinical genetics. In total, 85% of physicians would spend ?2 h/year on genetics/genomics education.
| S-EPMC6963876 | biostudies-literature
Project description:BackgroundTo inform Danish health care reform efforts, we compared health care system inputs and performance and assessed the usefulness of these comparisons for informing policy.MethodsRetrospective analysis of secondary data in the Danish Health Care System (DHS) with 5.3 million citizens and the Kaiser Permanente integrated delivery system (KP) with 6.1 million members in California. We used secondary data to compare population characteristics, professional staff, delivery structure, utilisation and quality measures, and direct costs. We adjusted the cost data to increase comparability.ResultsA higher percentage of KP patients had chronic conditions than did patients in the DHS: 6.3% vs. 2.8% (diabetes) and 19% vs. 8.5% (hypertension), respectively. KP had fewer total physicians and staff compared to DHS, with 134 physicians/100,000 individuals versus 311 physicians/100,000 individuals. KP physicians are salaried employees; in contrast, DHS primary care physicians own and run their practices, remunerated by a mixture of capitation and fee-for-service payments, while most specialists are employed at largely public hospitals. Hospitalisation rates and lengths of stay (LOS) were lower in KP, with mean acute admission LOS of 3.9 days versus 6.0 days in the DHS, and, for stroke admissions, 4.2 days versus 23 days. Screening rates also differed: 93% of KP members with diabetes received retinal screening; only 46% of patients in the DHS with diabetes did. Per capita operating expenditures were PPP$1,951 (KP) and PPP $1,845 (DHS).ConclusionCompared to the DHS, KP had a population with more documented disease and higher operating costs, while employing fewer physicians and resources like hospital beds. Observed quality measures also appear higher in KP. However, simple comparisons between health care systems may have limited value without detailed information on mechanisms underlying differences or identifying translatable care improvement strategies. We suggest items for more in-depth analyses that could improve the interpretability of findings and help identify lessons that can be transferred.
| S-EPMC2630928 | biostudies-literature
Project description:AIMS:Nephrotic syndrome (NS) is the most common clinical manifestation of glomerular disease in children. Currently, tacrolimus (TAC) is widely used in children with NS. However, pharmacokinetic data in children with nephrotic syndrome is limited. This study was intended to evaluate the population pharmacokinetics (PPK) of TAC in paediatric NS and to optimize dosing regimen. METHODS:Blood samples from NS children treated with TAC were collected and the blood concentrations of TAC were detected using HPLC-MS/MS. A PPK model was developed using NONMEM software. Pharmacogenetic analysis was carried out in the CYP3A5 gene. RESULTS:The data from 28 children were used for PPK analysis. A one-compartment model and first-order elimination were accorded with the TAC data in paediatric NS. A covariate analysis showed that body weight and CYP3A5 genotype significantly affected TAC pharmacokinetics. Monte Carlo simulation indicated that NS children with CYP3A5*3/*3 receiving 0.10 mg kg-1 dose-1 twice daily and NS children with CYP3A5*1 receiving 0.25 mg kg-1 dose-1 twice daily TAC could achieve the target concentrations of 5-10 ng ml-1 . CONCLUSION:The PPK of TAC was estimated in children with NS and a CYP3A5 genotype-based dosing regimen was set up based on simulations.
| S-EPMC6046506 | biostudies-literature
Project description:BackgroundThe association between gene polymorphisms and the risk of primary nephrotic syndrome (PNS) is uncovering recently. This study aims to investigate the relationship between single nucleotide polymorphisms (SNPs) on HLA-DQA1 gene and the risk of PNS.MethodsIn this study, we genotyped eight single nucleotide polymorphisms (SNPs) in the HLA-DQA1 gene in 501 PNS patients and 532 healthy people in Chinese population. Then we analyzed associations of these SNPs with the clinical features in primary nephrotic syndrome of children in Chinese population.ResultsSignificant associations with PNS were found on missense SNP rs1129740 (GG vs AA, odds ratio (OR) = 1.987, 95% confidence interval (CI) = 1.468-2.652, P = 0.00177049) and rs1047992 (AA vs GG, OR = 1.857, 95% CI = 1.325-2.391, P = 1.1073E-10) of the HLA-DQA1 gene.ConclusionsThis work suggests SNPs of HLA-DQA1 are risk factors for PNS in Chinese population, which implies roles of immune response in the pathogenesis of PNS.
| S-EPMC6430441 | biostudies-literature
Project description:Background:This study aimed to investigate the clinical significance of complement 3 (C3) deposition in renal tubules of children with primary nephrotic syndrome (PNS). Methods:The clinical and pathological characteristics of PNS were retrospectively reviewed in 99 PNS pediatric patients, who were divided into the C3 deposition and the non-C3 deposition groups. Results:A total of 39 patients (39.39%) had renal tubule C3 deposition. In the C3 deposition group, the ratios of urine N-acetylglucosaminidase/creatinine (UNAG/Cr), urine ?2 microglobulin/creatinine (U?2MG/Cr), and urine transferrin/creatinine (UTRF/Cr) were significantly higher than those of the non-C3 deposition group. The patients of the C3 deposition group had lower serum total protein and albumin, higher cholesterol and D-dimer (DD), lower proportion of CD3+CD8+ cells, and higher proportion of CD19+CD23+ cells. The number of the patients with interstitial fibrosis, renal cell vacuolar degeneration, renal tubular immunoglobulin deposition, and severe tubulointerstitial injury in the C3 deposition group was higher than that of the non-C3 deposition group. The C3 deposition intensity was positively correlated with the number of recurrences. Conclusion:PNS pediatric patients with C3 deposition in renal tubules have more severe disease condition, tubulointerstitial injury, and recurrence suggesting a worse long-term prognosis.
| S-EPMC5998187 | biostudies-literature
Project description:AimThe aim was to investigate the population pharmacokinetics of levamisole in children with steroid-sensitive nephrotic syndrome.MethodsNon-linear mixed effects modelling was performed on samples collected during a randomized controlled trial. Samples were collected from children who were receiving 2.5 mg kg(-1) levamisole (or placebo) orally once every other day. One hundred and thirty-six plasma samples were collected from 38 children from India and Europe and included in the analysis. A one compartment model described the data well.ResultsThe apparent clearance rate (CL/F) and distribution volume (V/F) were 44 l h(-1) 70 kg(-1) and 236 l 70 kg(-1) , respectively; estimated interindividual variability was 32-42%. In addition to allometric scaling of CL/F and V/F to body weight, we identified a significant proportional effect of age on CL/F (-10.1% per year). The pharmacokinetics parameters were not affected by gender, tablet strength or study centre. The median (interquartile range) maximum plasma concentration of levamisole was 438.3 (316.5-621.8) ng ml(-1) , and the median area under the concentration-time curve was 2847 (2267-3761) ng ml(-1) h. Median tmax and t½ values were 1.65 (1.32-2.0) h and 2.60 (2.06-3.65) h, respectively.ConclusionsHere, we present the first pharmacokinetic data regarding levamisole in children with steroid-sensitive nephrotic syndrome. The pharmacokinetic profile of levamisole in children was similar to findings reported in adults, although the elimination rate was slightly higher in children.
| S-EPMC4541972 | biostudies-literature
Project description:ObjectiveThe study sought to develop a criteria-based scoring tool for assessing drug-disease knowledge base content and creation of a subset and to implement the subset across multiple Kaiser Permanente (KP) regions.Materials and methodsIn Phase I, the scoring tool was developed, used to create a drug-disease alert subset, and validated by surveying physicians and pharmacists from KP Northern California. In Phase II, KP enabled the alert subset in July 2015 in silent mode to collect alert firing rates and confirmed that alert burden was adequately reduced. The alert subset was subsequently rolled out to users in KP Northern California. Alert data was collected September 2015 to August 2016 to monitor relevancy and override rates.ResultsDrug-disease alert scoring identified 1211 of 4111 contraindicated drug-disease pairs for inclusion in the subset. The survey results showed clinician agreement with subset examples 92.3%-98.5% of the time. Postsurvey adjustments to the subset resulted in KP implementation of 1189 drug-disease alerts. The subset resulted in a decrease in monthly alerts from 32 045 to 1168. Postimplementation monthly physician alert acceptance rates ranged from 20.2% to 29.8%.DiscussionOur study shows that drug-disease alert scoring resulted in an alert subset that generated acceptable interruptive alerts while decreasing overall potential alert burden. Following the initial testing and implementation in its Northern California region, KP successfully implemented the disease interaction subset in 4 regions with additional regions planned.ConclusionsOur approach could prevent undue alert burden when new alert categories are implemented, circumventing the need for trial live activations of full alert category knowledge bases.
| S-EPMC7647201 | biostudies-literature
Project description:Background:Determining which babies should receive antibiotics for potential early onset sepsis (EOS) is challenging. We performed a meta-analysis quantifying how many EOS cases might be 'missed' using the Kaiser Permanente electronic calculator, compared with National Institute for Health and Care Excellence (NICE) guidelines. Methods:A systematic literature search was carried out for studies citing the article in which the calculator was publicised. Studies were eligible if they presented data evaluating the calculator, either by retrospective case review or prospective cohort study. The primary outcome measure was numbers of culture positive EOS cases where the calculator did not recommend empirical antibiotics, but NICE guidelines would have. Data were pooled using a random effect meta-analysis. A subgroup analysis was performed using data from studies of babies exposed to chorioamnionitis. Findings:Eleven studies were included. There were a total of 75 EOS cases across the studies and a minimum of 14 (best case scenario), and a maximum of 22 (worst case scenario) cases where use of the calculator would have resulted in delayed or missed treatment, compared to if NICE guidelines had been followed. The probability of missed/delayed treatment for an EOS case were best case 0.19 [95% confidence intervals 0.11 - 0.29], worst case 0.31 [95% CI 0.17 - 0.49]. The probability of missing cases was significantly more in babies exposed to chorioamnionitis. Interpretation:A large proportion of EOS cases were 'missed' by the calculator. Further evaluation of the calculator is recommended before it is introduced into UK clinical practice. Funding:None.
| S-EPMC7046522 | biostudies-literature
Project description:BACKGROUND:Primary nephrotic syndrome (PNS) is a common glomerular disease in children. T cell dysfunction plays a crucial role in the pathogenesis of PNS. Moreover, dysbiosis of gut microbiota contributes to immunological disorders. Whether the initial therapy of PNS affects gut microbiota remains an important question. Our study investigated compositional changes of gut microbiota after initial therapy. METHODS:Fecal samples of 20 children with PNS were collected before and after 4-week initial therapy. Total bacteria DNA were extracted and the V3-V4 regions of bacteria 16S ribosomal RNA gene were sequenced. The composition of gut microbiota before and after initial therapy was analyzed by bioinformatics methods. The function of altered gut microbiota was predicted with PICRUSt method. RESULTS:The richness and diversity of gut microbiota were similar before and after 4-week initial therapy. Gut microbiota at the phylum level was dominated by four phyla including Firmicutes, Proteobacteria, Bacteroidetes and Actinobacteria, but the increased relative abundance after initial therapy was found in Deinococcus-Thermus and Acidobacteria. At the genus level, the increased abundance of gut microbiota after initial therapy was observed in short chain fat acids (SCFA)-producing bacteria including Romboutsia, Stomatobaculum and Cloacibacillus (p?<?0.05). Moreover, the predicted functional profile of gut microbiota showed that selenocompound metabolism, isoflavonoid biosynthesis and phosphatidylinositol signaling system weakened after initial therapy of PNS. CONCLUSIONS:Initial therapy of PNS increased SCFA-producing gut microbiota, but might diminish selenocompound metabolism, isoflavonoid biosynthesis and phosphatidylinositol signaling system in children.
| S-EPMC6878711 | biostudies-literature