Publications by authors named "Rachel Engen"

Article Synopsis
  • Vesicoureteral reflux (VUR) is a problem that can happen to kids who get kidney transplants, but not much is known about how it affects their new kidneys.
  • A study looked at 74 kids who got kidney transplants between 2007 and 2020 to see how VUR affected their health, checking for infections and kidney function.
  • The results showed that kids with high-grade VUR were more likely to develop urinary tract infections (UTIs), especially severe ones, but both groups had the same chances of keeping their transplanted kidney alive.
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Background: Obesity is associated with increased complications, rejection, and graft loss after kidney transplantation in adult and pediatric recipients. Elevated body mass index (BMI) is a common contraindication to transplant at adult kidney transplant programs; however, there is no data on such limitations for pediatric patients.

Methods: Between October and December 2022, we conducted a survey of Pediatric Nephrology Research Consortium centers assessing the use of BMI in pediatric kidney transplant evaluation.

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Background: Norovirus is the most common cause of viral gastroenteritis. Studies in adult kidney recipients have documented significant morbidity associated with norovirus infection, but there are few studies in pediatric recipients.

Methods: Multicenter retrospective cohort study of pediatric kidney transplant recipients with norovirus, confirmed by stool PCR, between January 1, 2008, and December 31, 2018.

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Background: Long-term outcomes in pediatric kidney transplantation remain suboptimal, largely related to chronic rejection. Creatinine is a late marker of renal injury, and more sensitive, early markers of allograft injury are an active area of current research.

Methods: This is an educational review summarizing existing strategies for monitoring for rejection in kidney transplant recipients.

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Background: There have been over 51 000 pediatric solid organ transplants since 1988 in the United States alone, leading to a growing population of long-term survivors who face complications of childhood organ failure and long-term immunosuppression.

Aims: This is an educational review of existing literature.

Results: Pediatric solid organ transplant recipients are at increased risk for risk for cardiovascular and kidney disease, skin cancers, and growth problems, though the severity of impact may vary by organ type.

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Background: Alemtuzumab is a lymphocyte depleting agent used for induction in kidney transplant, but long-term information on its use in pediatric recipients remains sparse.

Methods: We performed a single-center retrospective cohort study of 57 pediatric kidney transplant recipients receiving alemtuzumab 20 mg/m/dose ×2 doses for induction immunosuppression. All patients underwent surveillance biopsies, and 91.

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Background: Antibody-mediated rejection (AMR) is a major cause of kidney allograft loss. There is a paucity of large-scale pediatric-specific data regarding AMR treatment outcomes.

Methods: Data were obtained from 14 centers within the Pediatric Nephrology Research Consortium.

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Multi-organ transplantation involves the transplant of two or more organs from a single donor into a single recipient; in most cases, one of these organs is a kidney. Multi-organ transplantation is uncommon in pediatric transplantation but can be life-saving or significantly life-improving for children with rare diseases, including primary heart, liver, pancreas, or intestinal failure with secondary kidney failure, metabolic disorders, and genetic conditions causing multi-organ dysfunction. This manuscript reviews the current state of pediatric multi-organ transplantation that includes a kidney, with a focus on indications, evaluation, and key differences in management compared to kidney-alone transplantation.

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Children registered for kidney transplants prior to the age of 18 years retain "pediatric" allocation status after their 18th birthday. There are no data on the impact of this policy. We performed a retrospective cohort study of 7097 candidates listed for kidney transplant prior to 18 years of age who remained on the waitlist after their 18th birthday between January 1, 2015, and April 1, 2022, using United Network for Organ Sharing data.

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Background: The 2014 Kidney Allocation System (KAS) introduced longevity matching for adult candidates using the Estimated Post-Transplant Survival (EPTS) score, which includes candidate age, time on dialysis, diabetes status, and number of previous solid organ transplants. The proposed continuous distribution framework may expand the use of this attribute to pediatric candidates, but there is no data on its performance among pediatric kidney transplant recipients.

Methods: We performed a retrospective cohort study of 6800 pediatric kidney transplant recipients from 2001 to 2011 using Organ Procurement and Transplantation Network (OPTN) data.

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IgA nephropathy (IgAN) is associated with a risk for posttransplant recurrence. Data are limited regarding graft loss attributable to recurrence of IgAN among pediatric and young adult kidney transplant (KT) recipients. This was a retrospective cohort study of patients aged 0 to 25 years from the Scientific Registry of Transplant Recipients who received a primary KT for IgAN.

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Background: Multiorgan transplantation is increasingly common, driving recent increased attention to multiorgan allocation policies.

Methods: In this review, we summarize current multiorgan transplant allocation policies in the United States, with attention to recent and proposed changes and their impact on pediatric candidates.

Results: Existing multiorgan transplant policies attempt to balance equity and utility.

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Background: The United States organ allocation policies prioritize kidney-pancreas and other multiorgan candidates above pediatric kidney-alone candidates, but the effects of these policies are unclear.

Methods: We used OPTN data to describe trends in multiorgan and kidney-pancreas transplantation and identify 377 next-sequential pediatric kidney-alone candidates between 4/1/2015 and 10/31/2019 for individual-level analysis.

Results: Eleven percent of all kidneys were allocated as part of a multiorgan or kidney-pancreas transplant and 6% of pediatric kidney candidates were impacted.

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Neutropenia is generally defined as an absolute neutrophil count in the circulation of less than 1500/mm and occurs in up to 25%-30% of pediatric solid organ transplant recipients (SOT) within the first year after transplantation. In the SOT population, neutropenia is most often a result of drug-induced bone marrow suppression but can also be secondary to viral infection, nutritional deficiencies, lymphoproliferative infiltrate, and inherited causes. Outcomes for patients with neutropenia vary by degree of neutropenia and type of solid organ transplant.

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Background: A new Kidney Allocation System (KAS) was implemented in the United States in 2014 with the goal of improving equity and utility.

Methods: In this study, we compare outcomes for kidney-alone candidates less than 18 years of age, at the time of listing, in the 5 years prior to and following policy implementation using Organ Procurement and Transplantation Network data.

Results: While the pediatric deceased donor transplant rate increased under KAS, this increase was due solely to improved access for children aged 11-17 years; there was an 18.

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Background: FSGS is a common indication for kidney transplant with a high-risk of posttransplant recurrence.

Methods: In this review, we summarize current knowledge about FSGS recurrence after kidney transplantation, including epidemiology, pretransplant planning, posttransplant management, and investigational treatments.

Results: FSGS recurs in 14%-60% of first transplants, likely associated with a circulating permeability factor.

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Objective: To assess the outcomes of neonates in a contemporary multi-institutional cohort who receive renal replacement therapy (RRT) for hyperammonemia.

Study Design: We performed a retrospective analysis of 51 neonatal patients with confirmed inborn errors of metabolism that were treated at 9 different children's hospitals in the US between 2000 and 2015.

Results: Twenty-nine patients received hemodialysis (57%), 21 patients received continuous renal replacement therapy (41%), and 1 patient received peritoneal dialysis (2%).

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Background: Pediatric kidney transplant recipients are at risk for the development of post-transplant lymphoproliferative disorders (PTLD), a group of potentially devastating diseases that present on a spectrum of severity ranging from nondestructive PTLD to more histologically destructive lesions. Currently, there is inadequate evidence to guide evaluation and management of nondestructive PTLD.

Methods: This is a single-center case series of pediatric kidney transplant recipients between January 1, 2008 and December 31, 2019, who were diagnosed with PTLD.

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Background: Neutropenia is common in the first year after pediatric kidney transplant and is associated with an increased risk of infection, allograft loss, and death. Granulocyte colony-stimulating factor (G-CSF) increases neutrophil production, but its use in pediatric solid organ transplant recipients remains largely undescribed.

Methods: We performed a multicenter retrospective cohort study of children with neutropenia within the first 180 days after kidney transplant.

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Background: Eculizumab is approved for the treatment of atypical hemolytic uremic syndrome (aHUS). Its use off-label is frequently reported. The aim of this study was to describe the broader use and outcomes of a cohort of pediatric patients exposed to eculizumab.

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Introduction: There are no guidelines regarding management of failed pediatric renal transplants.

Materials & Methods: We performed a first of its kind multicenter study assessing prevalence of transplant nephrectomy, patient characteristics, and outcomes in pediatric renal transplant recipients with graft failure from January 1, 2006, to December 31, 2016.

Results: Fourteen centers contributed data on 186 pediatric recipients with failed transplants.

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Molecular mismatch analysis for assessment of histocompatibility in transplantation requires high-resolution HLA typing. Algorithms to "guesstimate" high-resolution from low-resolution typing exist, but their accuracy remains unknown. We converted high-resolution, sequence-based, HLA typing of 310 subjects from an ethnically heterogeneous population to low-resolution equivalents and tested the ability of the NMDP HaploStats and HLA Matchmaker programs to impute/reproduce the measured high-resolution HLA type, using the more common "winner-takes-all" approach.

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