Donna J. Claes

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

Background There are no multi-center studies examining omentectomy and peritoneal dialysis (PD) catheter revision in the pediatric dialysis population. Methods We performed a retrospective study at eight centers within the Pediatric Nephrology Research Consortium (PNRC). Data review included all incident tunneled PD catheters placed between 1/1/2011 and 12/31/2016 in pediatric stage 5 chronic kidney disease (CKD 5) patients. The primary outcome was the need for catheter revision and/or replacement. Multivariable logistic regression was performed to evaluate predictors for catheter revision/replacement. Results Data from 184 children (62.5% male; median age 7.4 years) were analyzed. Omentectomy was completed in 63.6% (n = 117). Revision/replacement occurred in 34.2% (n = 63); median time to revision/replacement was 38.5 days after insertion. PD catheter revision/replacement catheter occurred in 23.9% who underwent omentectomy versus 52.2% without omentectomy (p = 0.0005). Children ≥ 6 years at the time of catheter insertion experienced fewer revisions/replacements (18.2% age ≥ 6 vs. 56.5% age < 6 years, p <0.001). After adjusting for covariates, omentectomy reduced the need for revision by 63%; revision was 3.66 times more likely in those < 6 years of age. Conclusions This multi-center study demonstrates that omentectomy at the time of PD catheter insertion in pediatric patients is strongly associated with reduced likelihood of PD catheter revision. Omentectomy should be considered at the time of PD catheter insertion, especially in young children who are at high risk for PD catheter malfunction. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information

Standard treatments for AMR-rituximab, intravenous immunoglobulin, and/or plasmapheresis-aim to suppress the production and modulate the effect of donor-specific antibodies and remove them, respectively. Proteasome inhibitors such as bortezomib are potent therapeutic agents that target plasma cells more effectively than rituximab to reduce measurable donor-specific antibody production. Little is known in adults, and no data exist in children about effects of proteasome inhibition to treat AMR on protective antibody titers. We present a pediatric renal transplant recipient who received bortezomib for relatively early AMR and whose antibody titers to measles and tetanus were tracked. The AMR was treated successfully, and we noted no clinical decrease in the overall level of protective immunity from pretransplant baseline levels at almost one yr after AMR treatment cessation. Larger studies will elucidate more clearly how proteasome inhibition to treat AMR affects protective immunity in pediatric transplant recipients.

Cystinuria is a relatively uncommon cause of pediatric stone disease, but has significant morbidity if not properly controlled because of its significant stone recurrence rate. Cystinuria is caused by the inability of the renal tubules to reabsorb filtered cystine, which is poorly soluble at a typical urine pH <7. Although many advances have been made in the understanding of the genetic and physiological basis of cystinuria, the cornerstones of treatment still involve stone prevention with dietary measures and pharmacological therapy, coupled with surgical interventions for stone removal. Pharmacological treatments can carry significant side effects that must be monitored and can limit therapy as well as impede compliance. Most patients will require surgical intervention for stone removal, although compliance with prevention strategies reduces the need for intervention.