Treatment of allograft renal cell carcinoma with partial nephrectomy in a pediatric kidney transplant.

Renal cell carcinoma (RCC) is a common malignancy among kidney transplant recipients that often occurs in the native kidney. The incidence of RCC in the renal allograft is rare and carries the double risk of returning to dialysis and the development of metastatic cancer. The majority of reported cases of RCC in transplanted kidneys are in adult recipients and its occurrence in the pediatric age group is an uncommon event. There are currently no established guidelines on the treatment of RCC in transplant recipients. We report our experience of a 15-year-old male who developed allograft RCC 12 years later after transplantation. MRI confirmed the presence of the mass near the hilum of the renal allograft and biopsy revealed a Papillary Renal Cell Carcinoma (PRCC) type I. A partial allograft nephrectomy was successfully performed with negative tumor margins. The patient's serum creatinine 12 months post-operation was 1.9 mg/dL and presently he has no evidence of residual disease, recurrence, or metastasis. Partial nephrectomy is an effective treatment option for renal allograft RCC as it spares the patient from returning to dialysis until retransplantation is possible and necessary.

Extraperitoneal pediatric kidney transplantation of adult renal allograft using an en-bloc native liver and kidney mobilization technique.

BACKGROUND: We describe the safety and efficacy of performing pediatric kidney transplantation with a modified extraperitoneal approach that includes mobilization of the native liver and kidney. METHODS: We retrospectively identified pediatric renal transplants performed using this technique between 2015 and 2019. Data on patient demographics, surgical technique, and intraoperative details were collected. Outcomes were measured by morbidity and re-operation at 90 days, as well as serum creatinine, allograft survival, and overall survival at 1 year. RESULTS: Twenty-one patients with a median age of 5 (IQR 3-9) years, weighing 17.5 (IQR 14.5-24) kg were included. Median donor age was 24 (IQR 19-31) years. No intraoperative complications occurred. One child required a right native nephrectomy to allow sufficient space. Postoperatively, all patients had immediate graft function without urine leak or allograft thrombosis. 90-day morbidity and re-operation rates were zero. Both 1-year allograft and overall survival were 100% (on follow-up of all 21 patients through 1 year post-transplant), with a median serum creatinine of 0.58 (IQR 0.47-0.70) mg/dl at 1 year post-transplant. CONCLUSIONS: Pediatric kidney transplantation of adult renal allografts using an extraperitoneal approach with native liver and kidney mobilization has promising allograft and patient survival outcomes that eliminates peritoneal violation and may diminish the need for native nephrectomy.

Risk Assessment of Severe Congenital Anomalies of the Kidney and Urinary Tract (CAKUT): A Birth Cohort.

Recent advances in the early diagnosis of fetal CAKUT with an increase in fetal surgical interventions have led to a growing number of neonatal survivors born with severe renal dysfunction. This, in turn, has required the development of multi-disciplinary treatment paradigms in the individualized management of these infants with advanced stage kidney disease from birth. Early multi-modal management includes neonatal surgical interventions directed toward establishing adequate urine flow, respiratory support with the assessment of pulmonary hypoplasia, and establishing metabolic control to avoid the need for dialysis intervention. The development of specialized imaging to assess for residual renal mass with non-invasive 3-dimensional techniques are rapidly evolving. The use of non-radioactive imaging offers improved safety and allows for early prognostic-based planning including anticipatory guidance for progression to end stage renal disease (ESRD). The trajectory of kidney function during the neonatal period as determined by peak and nadir serum creatinine (SCr) and cystatin C (CysC) during the first months of life provides a guide toward individualized prospective management. This is a single center experience based on a birth cohort of 42 subjects followed prospectively from birth for an average of 6.1 ± 2.8 years at the University of Miami/Holtz Children's Hospital during the past decade. There was an 8:1 male: female ratio. The birth cohort was divided into 3 subgroups according to CKD Stages at the current age: CKD 1-2 (Group 1) (eGFR ≥ 60 ml/min/1.73 m2) (N = 15), CKD stage 3-5 (Group 2) (eGFR ≤ 59 ml/min/1.73 m2) (N = 12), and ESRD-Dialysis and/or Transplantation (Group 3) (N = 15). A neonatal CysC >3.0 mg/L predicted progression to ESRD while a nadir SCr >0.6 mg/dL predicted progression to CKD 3-5 with the highest specificity and sensitivity by ROC-AUC analysis (P < 0.0001). Medical management was directed toward nutritional support with novel formula designs, early introduction of growth hormone and strict control of mineral bone disorder. One of the central aspects of the management was to avoid dialysis for as long as feasible with a primary goal toward pre-emptive transplantation.

Assessing the link between modified 'Teach Back' method and improvement in knowledge of the medical regimen among youth with kidney transplants: The application of digital media.

OBJECTIVE: The objective of this study was to facilitate functional health literacy (FHL) with a modified "Teach Back" method. A computer-based program was developed for adolescent and young adult kidney transplant recipients (KTR) to knowledgeably answer questions about their medical condition, medications, and create a simple synopsis of their personal health record with the help of the heath care provider (HCP). METHODS: In a pre-post quasi-experimental design, 16 patients received the computer intervention in which they navigated questionnaires and brief informational video clips. Knowledge scores were assessed at baseline and 3 months. The binomial sign test was used to evaluate change in knowledge and purpose of medications. RESULTS: Mean age was 17.3 ± 2.4 years and 94% were non-Caucasian. Seven of 16 patients were academically below grade level. Twelve of 16 patients improved their overall knowledge (P = 0.0002) and purpose of medications (P = 0.0017). CONCLUSIONS: A Modified "Teach Back" during clinic visits was associated with improvements in FHL. PRACTICE IMPLICATIONS: This modified 'teach back' program has the potential to improve FHL which could contribute to long-term preservation of kidney transplants.

Issues in solid-organ transplantation in children: translational research from bench to bedside.

In this review, we identify important challenges facing physicians responsible for renal and cardiac transplantation in children based on a review of the contemporary medical literature. Regarding pediatric renal transplantation, we discuss the challenge of antibody-mediated rejection, focusing on both acute and chronic antibody-mediated rejection. We review new diagnostic approaches to antibody-mediated rejection, such as panel-reactive antibodies, donor-specific cross-matching, antibody assays, risk assessment and diagnosis of antibody-mediated rejection, the pathology of antibody-mediated rejection, the issue of ABO incompatibility in renal transplantation, new therapies for antibody-mediated rejection, inhibiting of residual antibodies, the suppression or depletion of B-cells, genetic approaches to treating acute antibody-mediated rejection, and identifying future translational research directions in kidney transplantation in children. Regarding pediatric cardiac transplantation, we discuss the mechanisms of cardiac transplant rejection, including the role of endomyocardial biopsy in detecting graft rejection and the role of biomarkers in detecting cardiac graft rejection, including biomarkers of inflammation, cardiomyocyte injury, or stress. We review cardiac allograft vasculopathy. We also address the role of genetic analyses, including genome-wide association studies, gene expression profiling using entities such as AlloMap®, and adenosine triphosphate release as a measure of immune function using the Cylex® ImmuKnow™ cell function assay. Finally, we identify future translational research directions in heart transplantation in children.

Issues in solid-organ transplantation in children: translational research from bench to bedside

In this review, we identify important challenges facing physicians responsible for renal and cardiac transplantation in children based on a review of the contemporary medical literature. Regarding pediatric renal transplantation, we discuss the challenge of antibody-mediated rejection, focusing on both acute and chronic antibody-mediated rejection. We review new diagnostic approaches to antibody-mediated rejection, such as panel-reactive antibodies, donor-specific cross-matching, antibody assays, risk assessment and diagnosis of antibody-mediated rejection, the pathology of antibody-mediated rejection, the issue of ABO incompatibility in renal transplantation, new therapies for antibody-mediated rejection, inhibiting of residual antibodies, the suppression or depletion of B-cells, genetic approaches to treating acute antibody-mediated rejection, and identifying future translational research directions in kidney transplantation in children. Regarding pediatric cardiac transplantation, we discuss the mechanisms of cardiac transplant rejection, including the role of endomyocardial biopsy in detecting graft rejection and the role of biomarkers in detecting cardiac graft rejection, including biomarkers of inflammation, cardiomyocyte injury, or stress. We review cardiac allograft vasculopathy. We also address the role of genetic analyses, including genome-wide association studies, gene expression profiling using entities such as AlloMap®, and adenosine triphosphate release as a measure of immune function using the Cylex® ImmuKnow™ cell function assay. Finally, we identify future translational research directions in heart transplantation in children.

Histomorphometric analysis of postnatal glomerulogenesis in extremely preterm infants.

Until now oligonephropathy to indicate "too few nephrons" has been associated with intrauterine growth restriction and experimentally induced abnormalities of renal development. The purpose of this study was to determine whether there is evidence of abnormal postnatal glomerulogenesis in extremely low birth weight preterm infants. Renal autopsy tissue was studied by computer-assisted morphometry from 56 extremely premature infants (birth weight < or = 1000 g) and 10 fullterm infants as controls. Preterm infants were divided into two groups (short survival < or = 40 days vs. long survival > or = 40 days). Each group was subdivided into those with renal failure (RF) and those with normal renal function. Forty-two of 56 preterm infants (75%) were adequate for gestational age. Glomerulogenesis as measured by radial glomerular counts (RGC) was markedly decreased in all preterm infants as compared to term controls and correlated significantly with gestational age (r = 0.87; P < 0.001). Active glomerulogenesis with "basophilic S-shaped bodies" was absent in longer surviving preterm and all term infants. RGC of preterm infants surviving > or =40 days with RF were significantly less than RGC of those with long survival and no RF (P < 0.001). Only this latter group demonstrated increased glomerular size as measured by mesangial tuft area and Bowman's capsule area compared to all other groups (P < 0.001). The kidney continues to form postnatally in preterm neonates, but glomerulogenesis ceases after 40 days. Moreover, it is further inhibited by RF. Compensatory mechanisms in longer surviving preterm infants include glomerular hypertrophy and mesangial proliferation that could lead to hyperfiltration.