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Background Survival to hospital discharge in neonates born with kidney failure has not been previously described. Methods This was a retrospective, observational analysis of the Pediatric Health Information System (PHIS) database from 2005 to 2019. Primary outcome was survival at discharge; secondary outcomes were hospital and ICU length of stay (LOS). Univariate analysis was performed to describe the population by birth weight (BW) and characterize survival; multivariable generalized liner mixed modeling assuming a binomial distribution and logit link was performed to identify mortality risk factors. Results Of 213 neonates born with kidney failure (median BW 2714 g; GA 35 weeks; 68% male), 4 (1.9%) did not receive dialysis or peritoneal dialysis (PD) catheter placement, 152 (72.9%) received PD only, 49 (23.4%) received PD plus extracorporeal dialysis (ECD), and 8 (3.4%) were treated with an undocumented dialysis modality. Median age at dialysis initiation was 7 days; median hospital LOS and ICU LOS were 84 and 69 days, respectively. One-hundred and sixty-two patients (76%) survived to discharge. Non-survivors (n = 51) were more likely to have received ECD and mechanical ventilation, and had a longer duration of mechanical ventilation. Every day of mechanical ventilation increased the mortality odds by 2% (n = 189; adjusted OR 1.02; 1.01, 1.03); in addition, the odds of mortality were 2 times higher in those who received ECD vs. only PD (adjusted OR 2.25; 1.04, 4.86). Conclusions Survival to initial hospital discharge occurs in the majority of neonates born with kidney failure. Predictors of increased mortality included longer duration of mechanical ventilation, as well as the requirement for ECD. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): Donna J. Claes [sup.1] , Troy Richardson [sup.2] , Matthew W. Harer [sup.3] , Mahima Keswani [sup.4] , Alicia Neu [sup.5] , Allison C. Redpath Mahon [sup.6] , Michael [...]

Background Initiation of continuous kidney replacement therapy (CKRT) greater than 20% fluid overload is associated with increased morbidity and mortality. We aimed to reduce the number of patients initiated on CKRT greater than 20% fluid overload by 50% in one year by implementation of a quality improvement initiative. Methods This is a prospective quality improvement study set in a pediatric ICU of an urban children's hospital of patients initiated on CKRT over 2 years. The intervention included creation of an electronic health record order for daily calculation of net percent fluid overload, incorporation into daily rounds, and education programs tailored to physicians and bedside nursing. We measured adherence with the new order set, percent fluid overload at CKRT initiation, days on CKRT, timing of first nephrology consultation, and death prior to discharge. Results A total of 32% of patients were initiated on CKRT greater than 20% fluid overload pre-initiative and 9% post-initiative, a 72% reduction over 13 months. Patients initiated on CKRT greater than 20% fluid overload had median CKRT course of 8 (IQR 4-14) vs. 22 days (IQR 13.5-62). Conclusion Creating a system using EHR with education may reduce initiation of CKRT after development of severe fluid overload. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information., Author(s): Delphine R. Nelson [sup.1] , Mahima Keswani [sup.2] , Laura Finn [sup.2] , Kalyn Mahoney [sup.2] , Lisa Genualdi [sup.2] , Mathew F. Barhight [sup.3] Author Affiliations: (1) grid.414220.1, [...]

Background Continuous kidney replacement therapy (CKRT) is a mainstay of therapy for management of severe acute kidney injury (AKI) in critically ill pediatric patients. There is limited data on the risk of chronic kidney disease (CKD) after discharge in this population. Methods This is a single-center, retrospective cohort study of all pediatric patients ages 0-17 years who received CKRT from 2013 to 2017. The study excluded patients with pre-existing CKD, those who died prior to discharge, and those who had insufficient follow-up data. Patients were followed after hospital discharge and electronic health record data was collected and analyzed to assess for incidence of and risk factors for kidney sequelae. Results A total of 42 patients were followed at a median time of 27 months (IQR 17.2, 39.8). Of these, 26.2% had evidence of CKD and 19% were at risk for CKD. Lower eGFR at hospital discharge was associated with increased odds of kidney sequelae (aOR 0.985; 95% CI 0.972, 0.996). Ages 0- < 1 and 12-17 were not significantly different (aOR 0.235, 95% CI 0.024, 1.718) and had the highest incidence of kidney sequelae (50% and 77%, respectively). Ages 1-5 and 6-11 had a decreased odds of kidney sequelae compared to the 12-17 year age group (aOR 0.098; 95% CI 0.009, 0.703 and aOR 0.035; 95% CI 0.001, 0.39, respectively). Only 54.8% of patients (n = 23) were seen in the nephrology clinic after discharge. Conclusions Patients who receive CKRT for AKI have a significant risk of CKD, while follow-up with a pediatric nephrologist in these high-risk patients is sub-optimal. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): Jeanne Frisby-Zedan [sup.1] [sup.2] , Matthew F. Barhight [sup.1] [sup.2] , Mahima Keswani [sup.1] [sup.2] , Jennifer Arzu [sup.2] , Delphine Nelson [sup.1] [sup.2] Author Affiliations: (1) grid.413808.6, 0000 [...]

Background In its first 3 years, the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) Collaborative demonstrated a statistically significant increase in the likelihood of compliance with a standardized follow-up care bundle and a significant reduction in peritonitis. We sought to determine if compliance with care bundles and low peritonitis rates could be sustained in centers continuously participating for 84 months. Methods Centers that participated from collaborative launch through the 84-month study period and provided pre-launch peritonitis rates were included. Children on maintenance peritoneal dialysis were eligible for enrollment. Changes in bundle compliance were assessed using a logistic regression model or a generalized linear mixed model (GLMM). Changes in average annualized peritonitis rates over time were modeled using GLMMs. Results Nineteen centers contributed 1055 patients with 1268 catheters and 17,247 follow-up encounters. The likelihood of follow-up compliance increased significantly over the study period (OR 1.05 95% confidence interval (CI) 1.03, 1.07; p < 0.001). Centers achieved [greater than or equal to] 80% follow-up bundle compliance by 28 months and maintained a mean compliance of 84% between 28 and 84 months post-launch. Average monthly peritonitis rates decreased from 0.53 (95% CI 0.37, 0.70) infections per patient-year pre-launch to 0.30 (95% CI 0.23, 0.43) at 84 months post-launch, p < 0.001. Conclusions Centers participating in the SCOPE Collaborative for 84 months achieved and maintained a high level of compliance with a standardized follow-up care bundle and demonstrated a significant and continued reduction in average monthly peritonitis rates. Graphical abstract, Author(s): Alicia M. Neu [sup.1] , Troy Richardson [sup.2] , Heidi Gruhler De Souza [sup.2] , Allison Redpath Mahon [sup.3] , Mahima Keswani [sup.4] , Joshua Zaritsky [sup.5] , Raj [...]

Establishing an early and accurate genetic diagnosis among patients with differences of sex development (DSD) is crucial in guiding the complex medical and psychosocial care they require. Genetic testing routinely utilized in clinical practice for this population is predicated upon physical exam findings and biochemical and endocrine profiling. This approach, however, is inefficient and unstandardized. Many patients with DSD, particularly those with 46,XY DSD, never receive a molecular genetic diagnosis. Rapid genome sequencing (rGS) is gaining momentum as a first‐tier diagnostic instrument in the evaluation of patients with DSD given its ability to provide greater diagnostic yield and timely results. We present the case of a patient with nonbinary genitalia and systemic findings for whom rGS identified a novel variant of the WT1 gene and resulted in a molecular diagnosis within two weeks of life. This timeframe of diagnosis for syndromic DSD is largely unprecedented at our institution. Rapid GS expedited mobilization of a multidisciplinary medical team; enabled early understanding of clinical trajectory; informed planning of medical and surgical interventions; and guided individualized psychosocial support provided to the family. This case highlights the potential of early rGS in transforming the evaluation and care of patients with DSD. [ABSTRACT FROM AUTHOR]

Background Peritoneal dialysis (PD) is the preferred chronic dialysis modality amongst pediatric patients. Peritonitis is a devastating complication of PD. Adult data demonstrates early onset peritonitis (EP) is associated with higher rates of subsequent peritonitis and technique failure. Limited data exists regarding EP in the pediatric population, here defined as peritonitis occurring within 60 days of catheter insertion. Methods PD catheter insertion practices and EP episodes were examined from the Standardizing Care to Improve Outcomes in Pediatric End Stage Renal Disease (SCOPE) collaborative database. Results There were 98 episodes of EP amongst 1106 PD catheters inserted. Multivariable analysis demonstrated a significant association between early use of the PD catheter and EP (P = 0.001). Age less than 1 year at the time of catheter insertion (P < 0.001), first catheter placed (P < 0.001) for the patient, use of a plastic adapter (P = 0.003), placement of sutures at the exit site (ES) (P = 0.032), and dressing change prior to 7 days post-operatively (P < 0.001) were all significantly associated with early PD catheter use. Concurrent placement of a hemodialysis catheter was associated with a decreased risk for early PD catheter use (P = 0.010). Conclusions In this large cohort of pediatric PD recipients, 8.4% of PD catheters were associated with the development of EP. The finding of an association between early use of the PD catheter and EP represents a potentially modifiable risk factor to reduce infection rates within this patient population., Author(s): Mahima Keswani [sup.1] , Allison C. Redpath Mahon [sup.2] , Troy Richardson [sup.3] , Jonathan Rodean [sup.3] , Olivera Couloures [sup.4] , Abigail Martin [sup.5] , Richard T. Blaszak [...]

Background Pauci-immune glomerulonephritis (GN) represents a severe form of glomerular injury and is the most common cause of crescentic GN in adults. To date, the clinicopathologic features of pauci-immune GN are not well characterized in the pediatric population. Methods Twenty-six biopsies from 21 pediatric patients with pauci-immune GN were identified retrospectively from the pathology archives of the University of Chicago (biopsy incidence 5 % among pediatric patients). Results There was distinct female predominance (2.5:1) among the patient cohort. Serologic studies identified anti-neutrophil cytoplasmic antibodies (ANCA) in 85 % of patients, and 80 % had systemic manifestations of vasculitis. The median estimated glomerular filtration rate (eGFR) at presentation was 43 ml/min/1.73 m.sup.2. Based on a previously proposed classification of ANCA-associated GN, we identified a spectrum of injury, including crescentic (n = 9), focal (n = 7), mixed (n = 5) and sclerotic GN (n = 5). Necrotizing arteritis was identified in a minority of patients (n = 3). The majority of those patients for whom data were available had been treated with cyclophosphamide and corticosteroids, with or without rituximab. Of the 21 pediatric patients, 58 % had developed chronic kidney disease at follow-up (eGFR Conclusion Pediatric patients with pauci-immune GN are similar to their adult counterparts in terms of clinical manifestations and histopathologic findings. Among the 21 patients in our study, those with focal GN had the best outcomes while patients with crescentic, mixed or sclerotic GN overwhelmingly had a poor long-term outcome for kidney function., Author(s): Mazdak A. Khalighi[sup.1] , Shihtien Wang[sup.2] , Kammi J. Henriksen[sup.1] , Margret Bock[sup.2] , Mahima Keswani[sup.2] , Anthony Chang[sup.1] , Shane M. Meehan[sup.1] Author Affiliations: (1) Department of Pathology, [...]

Peritoneal dialysis (PD) catheter obstruction often leads to surgical revision and may require transition to hemodialysis. The purpose of this study was to evaluate risk factors (including omentectomy) associated with early PD catheter obstruction (< 6 months from insertion). A retrospective review of all PD catheters inserted at a single high-volume referral center (2005–2018) was performed. 185 PD catheters were placed in 123 patients (45 female). Potential risk factors for early catheter obstruction were analyzed using Chi-square analysis (p < 0.05 considered statistically significant). Median age at catheter insertion was 3.42 years (3 days–39 years). Early catheter obstruction occurred in 42 cases (22.7%). Median time to early obstruction was 24 days (3–118 days). Previous PD catheter placement (p = 0.9) or prior abdominal surgery (p = 0.89) was not associated with obstruction. Weight ≥ 10 kg (p = 0.011) and age ≥ 1 year (p = 0.048) were associated with a significantly higher incidence of obstruction. Overall, omentectomy was associated with a trend in reduction of early obstruction in patients with weight ≥ 10 kg (p = 0.08) and significantly in patients ≥ 1 year (p = 0.028). Early PD catheter obstruction appears to occur more often in older patients with a higher weight. Concomitant omentectomy seems beneficial at reducing early catheter obstruction events in those patients. Retrospective comparative study. III. [ABSTRACT FROM AUTHOR]

Background:: Post-infectious glomerulonephritis (PIGN) is an immune complex-mediated glomerular injury that typically resolves. Dominant C3 deposition is characteristic of PIGN, but with the emergence of C3 glomerulonephritis (C3GN) as a distinct entity, it is unclear how the pathologic similarities between PIGN and C3GN should be reconciled. Therefore, nephrologists and nephropathologists need additional guidance at the time of biopsy. Methods: We studied 23 pediatric and young adult patients diagnosed with PIGN. Patients were divided into two groups, one with co-dominance between C3 and immunoglobulins and the other meeting proposed diagnostic criteria for C3GN. Clinical and pathological features were compared. Results: No clinical and/or pathological features could distinguish between those with C3-co-dominant deposits and those with C3 dominance. Nearly all patients in both groups regained their baseline renal function without clinical intervention. Conclusions: Although the identification of abnormalities of the alternative pathway of complement is characteristic of C3GN, testing is not widely available and the turnaround time often exceeds 1 month. Our study found that PIGN with either codominant or dominant C3 deposition in a cohort of young patients has excellent short-term outcomes. Close clinical observation for persistent abnormalities, such as hypocomplementemia, prolonged hematuria or proteinuria, is recommended to single out patients that may harbor intrinsic complement abnormalities. [ABSTRACT FROM AUTHOR]

Background: Exit site infections are a risk factor for the development of peritonitis in patients on long-term peritoneal dialysis. Visual assessments of an exit site utilising currently available tools (Twardowski and Mid-European Pediatric Peritoneal Dialysis Study Group (MEPPS)) are necessary to objectively characterise the appearance of an exit site. The aim of this study was to assess the interobserver agreement of exit site evaluations utilising both exit site scoring tools., Methods: Exit site evaluations were independently performed by two evaluators during outpatient visits at 13 sites within the Standardizing Care to Improve Outcomes in Pediatric End Stage Kidney Disease collaborative. The frequency and percentage of evaluations where both reviewers agreed were calculated. A sub-analysis was performed looking at evaluations where disagreement occurred., Results: A total of 371 paired exit site evaluations were collected over 6 months. For the majority of evaluations (range: 78%-97% Twardowski, 78%-97% MEPPS), both reviewers agreed that no abnormality was present across all domains. When the analysis was restricted to evaluations where at least one reviewer noted an abnormality, interobserver agreement fell across all domains (range: 31%-61% Twardowski, 56%-66% MEPPS). Disagreements more commonly occurred regarding the presence versus absence of an abnormality, rather than a difference in the severity of an abnormality., Conclusions: Whereas interobserver agreement is high when the appearance of a peritoneal dialysis catheter exit site is characterised as 'normal', interobserver disagreement is common when the appearance of the exit site is 'abnormal'. Further work is warranted to improve interobserver agreement of exit site assessments and to identify domains conferring an increased risk of infection., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: ABO incompatible (ABOi) liver transplantation (LT) was initially associated with a higher incidence of vascular, biliary, and rejection complications and a lower survival than ABO compatible (ABOc) LT. Various protocols have been proposed to manage anti-isohemagglutinin antibodies and hyperacute rejection. We present our experience with a simplified protocol using only plasmapheresis., Methods: A retrospective review of all patients who received an ABOi LT at our institution was performed. Comparisons were made based on era (early: 1997-2008, modern: 2009-2020) and severity of disease (status 1 vs. exception PELD at transplant). A pair-matched comparison was done to patients who received an ABOc LT. p  < 0.05 was considered significant., Results: 17 patients received 18 ABOi LT (3 retransplants). Median age at transplant was 7.4 months (1.1-28.9). 66.7% patients were listed as status 1. Hepatic artery thrombosis (HAT) occurred in one patient (5.6%), there were 2 cases of portal vein thrombosis (PVT) (11.1%), and 2 biliary strictures (11.1%). Patient and graft survival improved in the ABOi modern era, although not significantly. In the pair-matched comparison, complications (HAT p  = 0.29; PVT p  = 0.37; biliary complications p  = 0.15) and survival rates were similar. Patient and graft survivals were 100% in the non-status 1 ABOi patients compared to 67% ( p  = 0.11) and 58% ( p  = 0.081) respectively for patients who were transplanted as status 1., Conclusion: ABO incompatible liver transplants in infants with a high PELD score have excellent outcomes. Indications for ABO incompatible transplants should be liberalized to prevent deaths on the waiting list or deterioration of children with high PELD scores., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Lemoine, Brandt, Keswani and Superina.)