Many teenagers who struggle with chronic fatigue have symptoms suggestive of autonomic dysfunction that may include lightheadedness, headaches, palpitations, nausea, and abdominal pain. Inadequate sleep habits and psychological conditions can contribute to fatigue, as can concurrent medical conditions. One type of autonomic dysfunction, postural orthostatic tachycardia syndrome, is increasingly being identified in adolescents with its constellation of fatigue, orthostatic intolerance, and excessive postural tachycardia (more than 40 beats/min). A family-based approach to care with support from a multidisciplinary team can diagnose, treat, educate, and encourage patients. Full recovery is possible with multi-faceted treatment. The daily treatment plan should consist of increased fluid and salt intake, aerobic exercise, and regular sleep and meal schedules; some medications can be helpful. Psychological support is critical and often includes biobehavioral strategies and cognitive–behavioral therapy to help with symptom management. More intensive recovery plans can be implemented when necessary.

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Background Acute kidney injury (AKI) is a well-documented complication of pediatric hematopoietic stem cell transplantation (HSCT). Dialysis after HSCT is associated with a lower overall survival (OS); however, the association between less severe AKI and OS is unclear. Method We retrospectively studied 205 consecutive pediatric HSCT patients to determine the incidence and impact of all stages of AKI on OS in pediatric HSCT recipients. We used the peak pRIFLE grade during the first 100 days to classify AKI (R=risk, I= injury, F= failure, L= loss of function, E= End-stage renal disease) and used the modified Schwartz formula to estimate glomerular filtration rate. Results AKI was observed in 173 of the 205 patients (84%). The 1-year OS decreased significantly with an increasing severity of pRIFLE grades (p < 0.01). There was no difference in the OS between patients without AKI and the R/I group. Regardless of the dialysis status, stages F/L/E had significantly lower OS compared with patients without AKI or R/I (p < 0.01). There was no difference in OS among patients with dialysis and F/L/E without dialysis (p 0.65). Stages F/L/E predicted mortality independent of acute graft versus host disease, gender, and malignancy. Conclusion The OS of children after HSCT decreases significantly with an increasing severity of AKI within the first 100 days posttransplant. While our data did not show an increased risk of mortality with stages R/I, stages F/L/E predicted mortality regardless of dialysis. Prevention and minimization of AKI may improve survival after pediatric HSCT.

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Antibody-mediated rejection leads to allograft loss after kidney transplantation. Bortezomib has been used in adults for the reversal of antibody-mediated rejection; however, pediatric data are limited. This retrospective study was conducted in collaboration with the Midwest Pediatric Nephrology Consortium. Pediatric kidney transplant recipients who received bortezomib for biopsy-proven antibody-mediated rejection between 2008 and 2015 were included. The objective was to characterize the use of bortezomib in pediatric kidney transplant recipients. Thirty-three patients received bortezomib for antibody-mediated rejection at nine pediatric kidney transplant centers. Ninety percent of patients received intravenous immunoglobulin, 78% received plasmapheresis, and 78% received rituximab. After a median follow-up of 15 months, 65% of patients had a functioning graft. The estimated glomerular filtration rate improved or stabilized in 61% and 36% of patients at 3 and 12 months post-bortezomib, respectively. The estimated glomerular filtration rate at diagnosis significantly predicted estimated glomerular filtration rate at 12 months after adjusting for chronic histologic changes (P .001). Fifty-six percent of patients showed an at least 25% reduction in the mean fluorescence intensity of the immune-dominant donor-specific antibody, 1-3 months after the first dose of bortezomib. Non-life-threatening side effects were documented in 21 of 33 patients. Pediatric kidney transplant recipients tolerated bortezomib without life-threatening side effects. Bortezomib may stabilize estimated glomerular filtration rate for 3-6 months in pediatric kidney transplant recipients with antibody-mediated rejection.

Background: En bloc transplantation of small pediatric kidneys in children may help expand the existing deceased donor pool; however, studies examining the long-term outcomes of en bloc transplantation in children are few.Methods: We used the Scientific Registry of Transplant Recipients to identify 149 pediatric en bloc recipients transplanted from 10/1/1987-12/31/2017. We used propensity scores to match 148 en bloc with 581 non-en bloc deceased donor recipients (matching variables: transplant age, gender, race, pretransplant dialysis, transplant center and year). We evaluated patient and graft survival using Kaplan-Meier and Fleming-Harrington weighted log-rank test, and examined survival benefit of en bloc transplantation versus remaining on the waitinglist using the sequential Cox approach. We divided the study period into three 10-year intervals to assess the effect of era on outcomes.Results: Compared with non-en bloc recipients, en bloc recipients had lower 1-year graft survival (78.9% vs. 88.9; p 0.007), however when stratified by transplant era, lower 1-year survival was only observed in the oldest era (1987)(1988)(1989)(1990)(1991)(1992)(1993)(1994)(1995)(1996)(1997). En bloc recipients had superior 10-year patient

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