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Background Congenital anomalies of the kidney and urinary tract (CAKUT) are the most frequent causes of childhood chronic kidney disease (CKD). Using a large CAKUT cohort, we sought to identify the predictors of CKD and to develop a prediction model that informs a risk-stratified clinical pathway. Methods This was a retrospective cohort study including cases with multicystic dysplastic kidneys (MCDK), unilateral kidney agenesis (UKA), kidney hypoplasia (KH), and posterior urethral valves (PUV). We identified risk factors for CKD (estimated glomerular filtration rate [eGFR] Results We identified 452 eligible cases of CAKUT with 22% developing CKD. Strongest associations with CKD included primary diagnosis (OR 3.5, 95% CI 2.6-4.6), preterm delivery (OR 2.3, 95% CI 1.2-4.4), non-kidney anomalies (OR 1.8, 95% CI 1.1-3), first eGFR Conclusion Using a large combined CAKUT cohort we identified risk factors for CKD. Our prediction model provides the first steps towards a risk-stratified clinical pathway. Graphical abstract, Author(s): Douglas G. Matsell [sup.1] , Marisa Catapang [sup.1] , Brian Becknell [sup.2] Author Affiliations: (1) grid.17091.3e, 0000 0001 2288 9830, University of British Columbia, British Columbia Children's Hospital Research [...]

Background Post infectious glomerulonephritis (PIGN) is the most common form of acute glomerulonephritis in children. The objective of this study was to evaluate the risk factors for kidney injury in children with PIGN referred to a tertiary care center. Methods This was a retrospective cohort study. The primary outcome was acute kidney injury (AKI) at initial presentation; secondary outcome was composite kidney injury, defined as reduced estimated glomerular filtration rate (eGFR), proteinuria, or hypertension at last follow-up. Binary logistic regression defined risk factors associated with the primary and secondary outcomes. Results We identified 125 PIGN cases with a mean age of 8.3±3.5 years at presentation and 252 ± 501 days of follow-up. Sixty-six percent (79/119) presented with AKI and 57% (71/125) were admitted to hospital. Shorter time to seeing a nephrologist (OR 6.7, 95%CI 1.8-24.6), nadir C3 < 0.12 g/L (OR 10.2, 95%CI 1.9-53.7), starting an antihypertensive medication (OR 7.6, 95%CI 1.8-31.3), and nephrotic range proteinuria (OR 3.8, 95%CI 1.2-12.4) were independent risk factors for AKI when adjusted for each other. At last follow-up 35% (44/125) of the cohort had the composite outcome, with older age at presentation (OR 1.2, 95%CI 1.04-1.4) and nadir C3 < 0.17 g/L (OR 2.6, 95%CI 1.04-6.7) being independent risk factors when adjusted for AKI. Conclusion PIGN is an important cause of AKI in children and adolescents. The severity of initial illness is associated with the extent of kidney injury in both the short- and longer-term. Findings will help identify cases requiring lengthier surveillance. Graphical abstract, Author(s): Douglas G. Matsell [sup.1] , Dan Cojocaru [sup.1] , Marisa Catapang [sup.1] Author Affiliations: (1) grid.414137.4, 0000 0001 0684 7788, Department of Pediatrics, Division of Nephrology, British Columbia Children's [...]

Background Multicystic dysplastic kidney (MCDK) disease and unilateral renal agenesis (URA) are well-known causes of a solitary functioning kidney (SFK) and are associated with long-term kidney injury. The aims of this study were to characterize the natural history of SFK at our center, define the risk factors associated with chronic kidney injury, and identify distinguishing features between URA and MCDK that predict outcome. Methods This was a retrospective cohort study of 230 SFK patients. We compared MCDK (n=160) and URA (n=70) according to clinical features at diagnosis and kidney outcomes over follow-up. Univariate and multivariate binary regression analysis was used to determine independent risk factors for chronic kidney injury, defined as the composite outcome of hypertension, proteinuria, or chronic kidney disease (eGFR Results URA had a higher prevalence of comorbid genetic syndromes (15 vs. 6%, p=0.04), non-renal anomalies (39 vs. 11%, p Conclusions In our population, URA was more likely to have associated syndromes or anomalies, and to have worse outcomes over time than MCDK. URA diagnosis was an independent risk factor for chronic kidney injury. Our results will be used to develop a standardized clinical pathway for SFK management. Graphical abstract, Author(s): Douglas G. Matsell [sup.1] , Carol Bao [sup.1] , Teagan Po White [sup.1] , Ella Chan [sup.1] , Eli Matsell [sup.1] , Dan Cojocaru [sup.1] , Marisa Catapang [sup.1] [...]

Background Our multidisciplinary clinical pathway for treatment of childhood nephrotic syndrome (NS) was established with the goal of standardizing local clinical practice. This descriptive study aimed to assess nutrient intakes of children with newly diagnosed NS compared with nutrition goals defined by our pathway. Methods Our pathway includes evidence-based recommendations that target daily intakes during corticosteroid induction therapy: energy (Estimated Energy Requirements (EER) x Sedentary Physical Activity (PA)), sodium (1 mg/kcal), calcium (Dietary Reference Intake (DRI) + 500 mg elemental calcium), and vitamin D (DRI +800-1000 IU). After dietitian-led education at initial diagnosis, 3-day food records were completed at 4 weeks post-diagnosis. Daily nutrient intakes were compared to pathway targets and DRIs. Results Thirty-six children (median age 4.8 years, 44% female) with newly diagnosed NS submitted food records. Mean energy and sodium intakes were 103±22% and 99±53% of pathway targets, respectively. Fourteen (39%) children exceeded pathway sodium recommendations, with four (11%) exceeding them by greater than 50%. Seven (19%) children met DRI for calcium, while six (17 %) met pathway targets for calcium. No children met DRI for vitamin D from diet alone; and only one met the target with supplementation. Conclusions This is the first study to describe dietary intakes of children with newly diagnosed NS. Our clinical pathway targets for energy and sodium were achievable; however, calcium and vitamin D intakes fell short of pathway guidelines and DRIs. Prescription of supplemental calcium and vitamin D may be needed to achieve target intakes of calcium and vitamin D. Graphical abstract, Author(s): Nonnie Polderman [sup.1] [sup.2] , Meredith Cushing [sup.1] , Kirsten McFadyen [sup.1] , Marisa Catapang [sup.1] , Robert Humphreys [sup.1] , Cherry Mammen [sup.1] , Douglas G. Matsell [sup.1] [...]

Author(s): Douglas G. Matsell [sup.1] , Marisa Catapang [sup.1] Author Affiliations: (1) grid.17091.3e, 0000 0001 2288 9830, Division of Nephrology, British Columbia Children's Hospital, University of British Columbia, , 4480 [...]

Background Historically, children with nephrotic syndrome (NS) across British Columbia (BC), Canada have been cared for without formal standardization of induction prednisone dosing. We hypothesized that local historical practice variation in induction dosing was wide and that children treated with lower doses had worse relapsing outcomes. Methods This retrospective cohort study included 92 NS patients from BC Children's Hospital (1990-2010). We excluded secondary causes of NS, age 3000 mg/m.sup.2 doses. Conclusions Results strongly justify our development of a standardized, province-wide NS clinical pathway to reduce practice variation and minimize under-treatment. The lowest induction prednisone dosing threshold to minimize future relapsing risks is likely between 2000 and 2500 mg/m.sup.2. Further prospective studies are warranted., Author(s): Matthew Sibley [sup.1] , Abishek Roshan [sup.1] , Alanoud Alshami [sup.1] , Marisa Catapang [sup.1] , Jasper J. Jöbsis [sup.1] , Trevor Kwok [sup.1] , Nonnie Polderman [sup.1] , [...]

Background Most cases of childhood nephrotic syndrome (NS) are due to minimal change disease (MCD), while a minority of children have focal segmental glomerulosclerosis (FSGS) and an unfavorable clinical course, requiring a kidney biopsy to confirm diagnosis. We hypothesized that clinical characteristics at diagnosis and initial response to corticosteroid treatment accurately predict FSGS and can be used to guide consistent practice in the indications for kidney biopsy. Methods This was a case control study (1990-2012). Inclusion criteria included age 1-17 years, meeting the diagnostic criteria for NS, and having biopsy-proven FSGS or MCD. Clinical characteristics at diagnosis included age, kidney function [estimated glomerular filtration rate (eGFR)], hypertension, hematuria, nephritis (reduced eGFR, hematuria, hypertension), and response to steroids. Results From a total of 169 children who underwent kidney biopsy for NS we included 65 children with MCD and 22 with FSGS for analysis. There were no significant between-group differences in age, sex, or eGFR at the time of diagnosis. The FSGS group had a higher proportion of hypertension (40 vs. 15%; p = 0.02), hematuria (80 vs. 47%; p = 0.01), and nephritis (22 vs. 2%; p = 0.004) and was more likely to be steroid resistant after 6 weeks of treatment than the MCD group (67 vs. 19%; p < 0.001). As predictors of FSGS, hematuria had a high sensitivity of 0.80 [95% confidence interval (CI) 0.56-0.93] and low specificity of 0.53 (95% CI 0.39-0.66), nephritis had a low sensitivity of 0.22 (95% CI 0.07-0.48) and high specificity of 0.98 (95% CI 0.88-0.99), and steroid resistance had a low sensitivity of 0.67 (95% CI 0.43-0.85) and high specificity of 0.81 (95% CI 0.68-0.90). The combination of steroid resistance after 6 weeks of therapy and/or nephritis at diagnosis yielded the optimal sensitivity and specificity at 0.80 (95% CI 0.56-0.93) and 0.75 (95% CI 0.60-0.86), respectively, confirmed by the highest receiver operator characteristic area under the curve of 0.77. Conclusion Steroid resistance after 6 weeks of therapy and/or nephritis at initial presentation is an accurate predictor of FSGS in children with NS and will be used as the indication for kidney biopsy in our newly developed clinical pathway. This approach will maximize the yield of diagnostic FSGS biopsies while minimizing the number of unnecessary MCD biopsies., Author(s): Alanoud Alshami [sup.1] , Abishek Roshan [sup.1] , Marisa Catapang [sup.1] , Jasper J. Jöbsis [sup.1] , Trevor Kwok [sup.1] , Nonnie Polderman [sup.1] , Jennifer Sibley [sup.1] , [...]