Population-based identification and temporal trend of children with primary nephrotic syndrome: The Kaiser Permanente nephrotic syndrome study

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.

Comparison of three spot proteinuria measurements for pediatric nephrotic syndrome: based on the International pediatric Nephrology Association 2022 Guidelines

“…Our participants were predominantly male, consistent with studies in which 57–65.87% of pediatric NS patients were male ( Table 2 ) [ 44–46 ]. The participants had a median age of 10.04 years, whereas previous pediatric NS studies have reported median ages of 4.5–6.9 years [ 44–46 ].…”

“…Our participants were predominantly male, consistent with studies in which 57–65.87% of pediatric NS patients were male ( Table 2 ) [ 44–46 ]. The participants had a median age of 10.04 years, whereas previous pediatric NS studies have reported median ages of 4.5–6.9 years [ 44–46 ]. Most children in our study were well nourished; we excluded children with malnutrition because creatinine levels are generally lower in malnutrition [ 47 ], which could skew the UPCR value.…”

Effect of high density lipoprotein cholesterol (HDL-C) on renal outcome in patients with nephrotic syndrome complicated with steroid-induced diabetes mellitus(SIDM)

“…The population incidence of INS is approximately 1–3 per 100,000 people per year [ 2 ]. Compared to that in children [ 3 , 4 ], the pathological etiology of INS in Chinese adults is attributed to membranous nephropathy (MN) (24.96%), IgA nephropathy (IgAN) (24.09%), minimal change disease (MCD) (10.71%), focal segmental glomerulosclerosis (FSGS) (2.45%), membranous proliferative glomerulonephritis (MPGN) (0.61%), postinfectious GN (0.51%), and C3 glomerulopathy (0.14%) in Chinese adults [ 5 ]. Adults with INS experience higher adjusted rates of end-stage renal disease (ESRD), cardiovascular outcomes, and death, with significant variation by underlying etiology in the risk of developing ESRD [ 6 ].…”

Effects of family centered nursing model on children with primary nephrotic syndrome: A systematic review and meta-analysis

“…[3] After admission, children generally stayed in hospital for a long time, which affected their normal life, learning and social interaction, mainly included behavioral abnormalities, excessive activity, crying, aggression, depression, etc. [4,5] In addition, the negative emotions of family members would also affect the daily behavior of children, so that they might feel helpless. Clinically, glucocorticoids are often used to treat children with PNS, but about 80– 90% of hormone-sensitive PNS children will relapse, and there is obvious abnormal coagulation phenomenon and easy to form venous thrombosis, which brings serious physiological and psychological trauma to children at the stage of growth and development.…”