Determinants of hyperparathyroidism in children after kidney transplantation.

INTRODUCTION: Hyperparathyroidism (HPT) can contribute to metabolic bone disease following kidney transplantation. We evaluated post-transplant trends in intact parathyroid hormone (iPTH) and determined predictors of HPT in pediatric kidney transplant (KTx) recipients. METHODS: In this single-center study, retrospective data were collected on 88 children from 2013 to 2019. Data collected included dialysis vintage, biochemical parameters, post-transplant trends in iPTH, 25(OH)Vitamin D levels and estimated glomerular filtration rate (eGFR ml/min/1.73 m2 ). Pre-transplant treatment for HPT was quantified with a Treatment Burden score (TB, score range: 0-100). After log-transforming skewed variables (iPTH and eGFR), multivariable linear regression was performed to determine predictors of log {iPTH} at 6 and 36 months (mo) post-transplant. RESULTS: Median age was 12.8 (range: 1.9-20.5) years, and dialysis vintage was 11.2 (range: 0.0-112.9) months. The majority were of Hispanic and African Ancestry (77.3%). Median post-transplant iPTH was 69.5 (range: 1.8-306.8) pg/ml at 6 mo with a gradual downward trend to 59.0 (range: 28.0-445.0) pg/ml at 36 mo. Significant multivariable predictors of higher log {iPTH} post-transplant included longer dialysis vintage, higher TB, and lower log{eGFR} at 6 mo, and higher TB, lower log{eGFR}, and deceased donor transplant at 36 mo. CONCLUSIONS: Recognition of risk factors for HPT and monitoring iPTH post-transplant may facilitate timely interventions to mitigate cardiovascular and bone disease in pediatric KTx recipients. KEY MESSAGE: Describe serial trends in intact PTH after kidney transplantation. Pre- and post-transplant factors that contribute to persistence or re-occurrence of hyperparathyroidism after kidney transplantation in children include longer dialysis vintage, high pre-transplant treatment burden and decreased post-transplant GFR. Recognition of these factors, and monitoring intact PTH after kidney transplantation, could facilitate timely interventions to mitigate cardiovascular and bone disease in children.

Transplantation: platform to study recurrence of disease.

Beyond the direct benefit that a transplanted organ provides to an individual recipient, the study of the transplant process has the potential to create a better understanding of the pathogenesis, etiology, progression and possible therapy for recurrence of disease after transplantation while at the same time providing insight into the original disease. Specific examples of this include: 1) recurrence of focal segmental glomerulosclerosis (FSGS) after kidney transplantation, 2) recurrent autoimmunity after pancreas transplantation, and 3) recurrence of disease after orthotopic liver transplantation (OLT) for cirrhosis related to progressive steatosis secondary to jejuno-ileal bypass (JIB) surgery. Our team has been studying these phenomena and their immunologic underpinnings, and we suggest that expanding the concept to other pathologic processes and/or transplanted organs that harbor the risk for recurrent disease may provide novel insight into the pathogenesis of a host of other disease processes that lead to organ failure.

Venous vascular reconstruction of a robotically procured right kidney with two renal veins transplanted into a pediatric recipient.

BACKGROUND: Right versus left kidney donor nephrectomy remains a controversial topic in renal transplantation given the increased incidence of right kidney vascular anomalies and associated venous thrombosis. We present the case of a 3-year-old pediatric recipient with urethral atresia and end-stage kidney disease who received a robotically procured living donor right pelvic kidney with two short same-size renal veins and a short ureter. METHODS: We utilized a completely deceased iliac vein system (common iliac vein with both external and internal veins) to extend the two renal veins. Due to the distance between both renal veins, the external iliac vein was anastomosed to the upper hilum renal vein, and the internal iliac vein was anastomosed to the lower hilum renal vein. The donor's short ureter was anastomosed to the recipient's ureter end-to-side. RESULTS: The patient had immediate graft function and there were no post-operative complications. Renal ultrasound was unremarkable at 48 hours post-transplant. Serum creatinine was 0.5 mg/dL at 3 months post-transplant. CONCLUSION: We demonstrate the successful transplantation of a robotically procured right pelvic donor kidney with two short renal veins using a deceased donor iliac vein system for venous reconstruction without increasing technical complications. This technique of venous reconstruction can be used in right kidneys with similar anatomical variations without affecting graft function.

The podocyte: glomerular sentinel at the crossroads of innate and adaptive immunity.

Focal segmental glomerulosclerosis (FSGS) is a common glomerular disorder that manifests clinically with the nephrotic syndrome and has a propensity to recur following kidney transplantation. The pathophysiology and therapies available to treat FSGS currently remain elusive. Since the podocyte appears to be the target of apparent circulating factor(s) that lead to recurrence of proteinuria following kidney transplantation, this article is focused on the podocyte. In the context of kidney transplantation, the performance of pre- and post-reperfusion biopsies, and the establishment of in vitro podocyte liquid biopsies/assays allow for the development of clinically relevant studies of podocyte biology. This has given insight into new pathways, involving novel targets in innate and adaptive immunity, such as SMPDL3b, cGAS-STING, and B7-1. Elegant experimental studies suggest that the successful clinical use of rituximab and abatacept, two immunomodulating agents, in our case series, may be due to direct effects on the podocyte, in addition to, or perhaps distinct from their immunosuppressive functions. Thus, tissue biomarker-directed therapy may provide a rational approach to validate the mechanism of disease and allow for the development of new therapeutics for FSGS. This report highlights recent progress in the field and emphasizes the importance of kidney transplantation and recurrent FSGS (rFSGS) as a platform for the study of primary FSGS.

Benefit of B7-1 staining and abatacept for treatment-resistant post-transplant focal segmental glomerulosclerosis in a predominantly pediatric cohort: time for a reappraisal.

BACKGROUND: Primary FSGS manifests with nephrotic syndrome and may recur following KT. Failure to respond to conventional therapy after recurrence results in poor outcomes. Evaluation of podocyte B7-1 expression and treatment with abatacept (a B7-1 antagonist) has shown promise but remains controversial. METHODS: From 2012 to 2020, twelve patients developed post-KT FSGS with nephrotic range proteinuria, failed conventional therapy, and were treated with abatacept. Nine/twelve (< 21 years old) experienced recurrent FSGS; three adults developed de novo FSGS, occurring from immediately, up to 8 years after KT. KT biopsies were stained for B7-1. RESULTS: Nine KTRs (75%) responded to abatacept. Seven of nine KTRs were B7-1 positive and responded with improvement/resolution of proteinuria. Two patients with rFSGS without biopsies resolved proteinuria after abatacept. Pre-treatment UPCR was 27.0 ± 20.4 (median 13, range 8-56); follow-up UPCR was 0.8 ± 1.3 (median 0.2, range 0.07-3.9, p < 0.004). Two patients who were B7-1 negative on multiple KT biopsies did not respond to abatacept and lost graft function. One patient developed proteinuria while receiving belatacept, stained B7-1 positive, but did not respond to abatacept. CONCLUSIONS: Podocyte B7-1 staining in biopsies of KTRs with post-transplant FSGS identifies a subset of patients who may benefit from abatacept. A higher resolution version of the Graphical abstract is available as Supplementary information.

Retroperitoneal kidney transplantation with liver and native kidney mobilization: a safe technique for pediatric recipients.

BACKGROUND: Pediatric kidney transplant (KT) using larger, deceased or living donor adult kidneys can be challenging in the pediatric population due to limited space in the retroperitoneum. Liver and native kidney (L/NK) mobilization techniques can be used in smaller and younger transplant recipients to aid in retroperitoneal placement of the renal allograft. Here, we compare the clinical outcomes of pediatric retroperitoneal KT with and without L/NK mobilization. METHODS: We retrospectively analyzed pediatric renal transplant recipients treated between January 2015 and May 2021. Donor and recipient demographics, intraoperative data, and recipient outcomes were included. Recipients were divided into two groups according to the surgical technique utilized: with L/NK mobilization (Group 1) and without L/NK mobilization (Group 2). Baseline variables were described using frequency distributions for categorical variables and means and standard errors for continuous variables. Tests of association with the likelihood of using L/NK mobilization were performed using standard χ2 tests, t tests, and the log-rank test. RESULTS: Forty-six pediatric recipients were evaluated and categorized into Group 1 (n = 26) and Group 2 (n = 20). Recipients in Group 1 were younger (6.7 ± 0.8 years vs. 15. 3 ± 0.7, P < 0.001), shorter (109.5 ± 3.7 vs. 154.2 ± 3.8 cm, P < 0.001) and weighed less (21.4 ± 2.0 vs. 48.6 ± 3.4 kg, P < 0.001) than those in Group 2. Other baseline characteristics did not differ between Groups 1 and 2. One urologic complication was encountered in Group 2; no vascular or surgical complications were observed in either group. Additionally, no stents or drains were used in any of the patients. There were no cases of delayed graft function or graft primary nonfunction. The median follow-up of the study was 24.6 months post-transplant. Two patients developed death-censored graft failure (both in Group 2, P = 0.22), and there was one death with a functioning graft (in Group 2, P = 0.21). CONCLUSIONS: Retroperitoneal liver/kidney mobilization is a feasible and safe technique that facilitates implantation of adult kidney allografts into pediatric transplant recipients with no increased risk of developing post-operative complications, graft loss, or mortality.

Predictors of advanced chronic kidney disease in infancy after definitive vesicoamniotic shunting for congenital lower urinary tract obstruction.

Background: Severe congenital lower urinary tract obstruction (cLUTO) is associated with poor postnatal outcomes, including chronic and end stage kidney disease, and high mortality. Studies of the impact of fetal intervention through vesicoamniotic shunting are marred by a device malfunction rate of up to 60%. In this study, we delineate the postnatal course and infant kidney function following definitive urinary diversion in utero. Materials and Methods: This is a retrospective, single-center cohort study of 16 male infants who survived the fetal intervention to birth, from 2010 to 2014 at a single center. All had patent shunts in place at birth. Perinatal and biochemical characteristics were collected with patients followed for one year, or until demise, with serial measures of serum creatinine (SCr) and serum cystatin C (CysC). Results: Of the 16 males, 81% were non-white (38% black, 43% Hispanic). Shunts were placed at a median of 20 weeks (IQR 19,23) gestation, with median fetal bladder volume of 39 cm3 (IQR 9.9,65). All neonates were born preterm [median 34 weeks (IQR 31,35)] and the majority with low birth weight [median 2340 grams (1,895, 2,600)]. 63% required positive pressure ventilation. Advanced chronic kidney disease stage 4-5 at 1 year of age was predicted by neonatal characteristics: peak SCr ≥2 mg/dl, time to peak SCr > 6 days, discharge SCr ≥1.0 mg/dl, CysC ≥2.5 mg/l, urine protein:creatinine ≥4.8 mg/mg, urine microalbumin:creatinine ≥2.2 mg/mg. In infancy, a nadir SCr ≥0.5 mg/dl occurring before 160 days (5.3 months) of age was also predictive of advanced chronic kidney disease stage 4-5 at 1 year. Three patients died in the neonatal period, with 1 receiving kidney replacement therapy (KRT). Three additional patients required KRT before 12 months of age. Conclusions: Even with definitive vesicoamniotic shunting for cLUTO, postnatal morbidity and mortality remain high, emphasizing the role of renal dysplasia, in spite of urinary diversion, in postnatal kidney dysfunction. Neonatal and infant biochemical parameters exhibit distinct trends that offer families and physicians a better understanding of the prognosis of childhood kidney function.

Spectrum of Clinical Manifestations in Children With WT1 Mutation: Case Series and Literature Review.

Background: Mutations of the Wilms tumor suppressor-1 gene (WT1) are associated with life-threatening glomerulopathy, disorders of sexual development, Wilm's tumor, and gonadal malignancies. Our objectives were to describe the clinical presentations, age of progression, and onset of complications of WT1 mutation through a case series and literature review. Methods: A retrospective study included all patients followed at the University of Miami/Holtz Children's Hospital from January 2000 to December 2020 with a diagnosis of WT1 mutation. A literature review of WT1 mutation cases was analyzed for clinical manifestations, karyotype, and long-term outcomes. Results: The WT1 mutation was identified in 9 children, median age at presentation of 0.9 years (range 1 week to 7 years). A total of four had female phenotypes, and 5 had abnormalities of male external genitalia, while all had XY karyotypes. All progressed to end-stage kidney disease (ESKD) and received a kidney transplant at a median age of 5 years (1.5-15 years). During a median time of follow-up of 9 years (range 2-28 years), there were 2 allograft losses after 7 and 10 years and no evidence of post-transplant malignancy. From 333 cases identified from the literature review, the majority had female phenotype 66% (219/333), but the predominant karyotype was XY (55%, 183/333). Of the female phenotypes, 32% (69/219) had XY sex reversal. Wilm's tumor occurred in 24%, predominantly in males with gonadal anomalies. Conclusions: Early recognition of WT1 mutation is essential for comprehensive surveillance of potential malignancy, avoidance of immunosuppressants for glomerulopathy, and establishing long-term multidisciplinary management.

Case report: Bordetella holmesii: A rare pathogen causing infective endocarditis associated glomerulonephritis.

Infective endocarditis (IE) can cause multiorgan dysfunction and chronic kidney disease, in addition to cardiac sequelae. The presentation may be vague and can manifest as acute glomerulonephritis. While the most common pathogens of infective endocarditis are Staphylococcus and Streptococcus species, we report a rare pathogen Bordetella holmesii causing infective endocarditis associated glomerulonephritis. A 20-year-old male patient with tetralogy of Fallot with pulmonary atresia and aortopulmonary collaterals underwent several cardiac surgeries including prosthetic pulmonary valve replacement in the past. He was admitted for 3 days at an outside hospital for fever, cough, and hemoptysis, and diagnosed with streptococcal pharyngitis, for which he received antibiotics. Five weeks later, he presented to our institution with lower extremity edema and gross hematuria. On examination, he was afebrile, normotensive, had a 7-kg weight gain with anasarca, and a systolic murmur, without rash. Investigations revealed elevated serum creatinine, nephrotic range proteinuria, hematuria, and hypocomplementemia, consistent with acute glomerulonephritis. Given his cardiac history, blood cultures were collected from three sites. Broad-spectrum antibiotics were initiated when he subsequently developed fever. Renal pathology on biopsy showed diffuse proliferative immune complex-mediated glomerulonephritis. Transesophageal echocardiogram visualized a vegetation on the pulmonary valve. Bordetella holmesii was ultimately cultured from the prior and current hospitalization. A serum sample detecting microbial cell-free DNA sequencing confirmed Bordetella holmesii at very high levels. After completing 6 weeks of intravenous antibiotics with concurrent angiotensin receptor blockade, his kidney function recovered with improvement in hypocomplementemia and proteinuria. This case report highlights the early recognition and comprehensive evaluation of a rare organism causing IE-associated GN, which allowed for renal recovery and preserved cardiac function.

Pediatric kidney transplants with multiple renal arteries show no increased risk of complications compared to single renal artery grafts.

Background: Kidney allografts with multiple renal arteries (MRA) are not infrequent and have been historically associated with a higher risk of developing vascular and urologic complications. Reports of kidney transplantation using MRA allografts in the pediatric population remain scarce. The aim of this study was to evaluate if transplantation of allografts with MRA with a surgical intent of creating a single arterial inflow using vascular reconstruction techniques when required, and without the routine use of surgical drains or ureteral stents, is associated with an increased risk of complications when compared to single renal artery (SRA) grafts. Methods: We retrospectively analyzed all pediatric renal transplant recipients performed by a single surgeon at our center between January 2015 and June 2022. Donor and recipient demographics, intraoperative data, and recipient outcomes were included. Recipients were divided into two groups based on SRA vs. MRA. Baseline variables were described using frequency distributions for categorical variables and means and standard errors for continuous variables. Comparisons of those distributions between the two groups were performed using standard chi-squared and t-tests. Time-to-event distributions were compared using the log-rank test. Results: Forty-nine pediatric transplant recipients were analyzed. Of these, 9 had donors with MRA (Group 1) and 40 had donors with SRA (Group 2). Native kidney and liver mobilization was performed in 44.4% (4/9) of Group 1 vs. 60.0% (24/40) of Group 2 cases (p = 0.39). There were no cases of delayed graft function or graft primary nonfunction. No surgical drainage or ureteral stents were used in any of the cases. One patient in Group 2 developed a distal ureter stricture. The geometric mean serum creatinine at 6- and 12-months posttransplant was 0.7 */ 1.2 and 0.9 */ 1.2 mg/dl in Group 1 and 0.7 */ 1.1 and 0.7 */ 1.1 mg/dl in Group 2. Two death-censored graft failures were observed in Group 2, with no significant difference observed between the two groups (p = 0.48). Conclusions: Our study demonstrates that pediatric renal transplantation with MRA grafts, using a surgical approach to achieve a single renal artery ostium, can be safely performed while achieving similar outcomes as SRA grafts and with a low complication rate.

How to Deal With Kidney Retransplantation-Second, Third, Fourth, and Beyond.

Kidney transplantation is the best health option for patients with end-stage kidney disease. Ideally, a kidney transplant would last for the lifetime of each recipient. However, depending on the age of the recipient and details of the kidney transplant, there may be a need for a second, third, fourth, or even more kidney transplants. In this overview, the outcome of multiple kidney transplants for an individual is presented. Key issues include surgical approach and immunologic concerns. Included in the surgical approach is an analysis of transplant nephrectomy, with indications, timing, and immunologic impact. Allograft thrombosis, whether related to donor or recipient factors merits investigation to prevent it from happening again. Other posttransplant events such as rejection, viral illness (polyomavirus hominis type I), recurrent disease (focal segmental glomerulosclerosis), and posttransplant lymphoproliferative disease may lead to the need for retransplantation. The pediatric recipient is especially likely to need a subsequent kidney transplant. Finally, noncompliance/nonadherence can affect both adults and children. Innovative approaches may reduce the need for retransplantation in the future.

Systemic causes of non-dermatologic chronic pruritus in the pediatric population and their management: An unexplored area.

Chronic pruritus associated with systemic diseases in the pediatric population has been infrequently addressed in the literature. This review focuses on chronic pruritus presenting without cutaneous manifestations. Common systemic etiologies include diseases with hepatic, renal, and hematologic origins. This encompasses several congenital liver disorders, end-stage renal disease (ESRD), and lymphoproliferative disorders such as Hodgkin's lymphoma. In this paper, an expert panel describes the clinical characteristics, pathophysiology, and therapeutic treatment ladders for chronic pruritus associated with the aforementioned systemic etiologies. Novel therapies are also reviewed. Our aim is to shed light on this unexplored area of pediatric dermatology and instigate further research.

Pre-clerkship Pediatric Exposure Enhances Understanding of Social Determinants of Health.

The Child and Adolescent Motivation and Enrichment Program (CHAMP), an initiative pairing pre-clerkship medical students with pediatric patients undergoing hemodialysis (HD), provides support to HD patients while also providing medical students with opportunities to increase their comfort in the clinical environment and awareness of social determinants of health (SDH). SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s40670-021-01329-4.

A Medical Student Initiative to Enhance the Pediatric Hemodialysis Experience.

BACKGROUND: Children and young adults receiving hemodialysis (HD) face unique challenges including frequent school absenteeism, psychosocial issues, and social isolation, placing them at risk for decreased academic achievement and health literacy. OBJECTIVE: To address this, we implemented the Child and Adolescent Motivation and Enrichment Program (CHAMP) at Holtz Children's Hospital in Miami, FL. The objective of this study is to describe the organizational structure and program design of CHAMP and provide preliminary program opinions. METHODS: Medical students served as longitudinal one-on-one mentors to patients receiving HD. Face-to-face intervention, books, board games, and electronic tablets were used to enhance patients' educational and recreational experience. We surveyed participating patients, medical students, and unit nurses regarding their opinions of CHAMP. KEY RESULTS: Patients responded to a series of questions on a Likert scale scored from 1 to 5 and reported the highest scores on questions pertaining to having fun with mentors (mean = 4.88), enjoying mentor visits (mean = 4.78), and learning during visits (mean = 3.88). Mentors reported the highest level of agreement (mean = 4.82) that CHAMP helped them gain empathy for patients with chronic and/or special health care needs. Nurses scored highly on the point that "overall, the program was useful and helped the patient" (mean = 6.86 of a possible 7). CONCLUSION: CHAMP is an academic and psychosocial enrichment program for children and adolescents receiving HD. The program is regarded highly by participating patients, medical students, and unit nurses. Patients report enjoying and learning from mentor sessions, whereas nurses report improved interactions with patients. Medical students who participate as mentors also gain important exposure to the field of pediatric nephrology. The program design as described herein positions CHAMP for replication at academic medical centers nationwide, allowing for optimization of the health and well-being of the pediatric HD population. [HLRP: Health Literacy Research and Practice. 2021;5(1):e60-e69.] Plain Language Summary: Pediatric patients undergoing hemodialysis (HD) are at risk for decreased academic achievement and health literacy. To address this, we implemented the Child and Adolescent Motivation and Enrichment Program, a longitudinal mentorship program pairing medical students as one-on-one mentors to patients undergoing HD. Preliminary results from this program demonstrate satisfaction and enjoyment by participating patients, medical students, and dialysis unit nurses.

Case Report: Uroenteric Fistula in a Pediatric-en-bloc Kidney Transplant Manifests as Deceptive Watery Diarrhea and Normal Anion Gap Acidosis.

Introduction: The diagnosis of a post-surgical uroenteric fistula can be challenging and may be delayed for months after symptoms begin. A normal anion gap metabolic acidosis has been reported in up to 100% of patients after ureterosigmoidostomy, and bladder substitution using small bowel and/or colonic segments. Here, we describe a rare case of a pediatric patient who developed a uroenteric fistula from the transplant ureters into the small bowel, after an en-bloc kidney transplantation resulting in profound acidosis and deceptive watery diarrhea. Case Presentation: The patient is an 8-year-old girl with end stage kidney disease (ESKD) secondary to focal segmental glomerulosclerosis. Through a right retroperitoneal approach, she underwent a right native nephrectomy and a pediatric deceased donor en-bloc kidney transplant including two separate ureters. One month later, she had a renal allograft biopsy for suspected rejection. During the week after the biopsy, she experienced abdominal pain followed by watery diarrhea and metabolic acidosis requiring continuous bicarbonate/acetate infusions. An extensive gastro-intestinal evaluation for the cause of the diarrhea including endoscopy was inconclusive. The urine output decreased to <500 ml daily; although, the kidney function remained normal. After 2 weeks of unexplained watery diarrhea a magnetic resonance urogram with contrast was performed which demonstrated extravasation of urine from both ureters with fistulization into the small bowel. She underwent corrective surgery which identified the fistulous tract, which was resected and both ureters were re-implanted. The diarrhea and acidosis resolved, and she has maintained normal renal allograft function for over 1 year. Conclusion: An important aspect in the early diagnosis of a uroenteric fistula is the sudden onset of severe hyperchloremic metabolic acidosis that results when urine is diverted into the intestinal tract. The mechanism is similar to that described in cases of urinary diversions and/or bladder augmentation using the intestine. Important diagnostic tools are the measurements of solute excretion and pH in the urine as compared to the "watery diarrhea" or bowel output. Summary: We describe a case of a uroenteric fistula in a pediatric-en-bloc kidney transplant patient that went undiagnosed for almost 3 weeks due to the deceptive nature of the watery diarrhea which was actually urine. A uroenteric fistula should be considered in the differential diagnosis of diarrhea and hyperchloremic metabolic acidosis as a complication of kidney transplant. The simultaneous comparison of stool and urine pH and solute excretions may lead to the diagnosis, appropriate imaging and surgical intervention.

Practice patterns and influence of allograft nephrectomy in pediatric kidney re-transplantation: A pediatric nephrology research consortium study.

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. The 76 recipients that underwent transplant nephrectomy were not significantly different from the 110 without nephrectomy in donor or recipient demographics. Fifty-three percent of graft nephrectomies were within a year of transplant. Graft tenderness prompted transplant nephrectomy in 91% (P < .001). Patients that underwent nephrectomy were more likely to have a prior diagnosis of rejection within 3 months (43% vs 29%; P = .04). Nephrectomy of allografts did not affect time to re-listing, donor source at re-transplant but significantly decreased time to (P = .009) and incidence (P = .0002) of complete cessation of immunosuppression post-graft failure. Following transplant nephrectomy, recipients were significantly more likely to have rejection after re-transplant (18% vs 7%; P = .03) and multiple rejections in first year after re-transplant (7% vs 1%; P = .03). CONCLUSIONS: Practices pertaining to failed renal allografts are inconsistent-40% of failed pediatric renal allografts underwent nephrectomy. Graft tenderness frequently prompted transplant nephrectomy. There is no apparent benefit to graft nephrectomy related to sensitization; but timing / frequency of immunosuppression withdrawal is significantly different with slightly increased risk for rejection following re-transplant.

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.

Donor considerations in pediatric kidney transplantation.

This article reviews kidney transplant donor options for children with end-stage kidney disease (ESKD). Global access to kidney transplantation is variable. Well-established national policies, organizations for organ procurement and allocation, and donor management policies may account for higher deceased donor (DD transplants) in some countries. Living donor kidney transplantation (LD) predominates in countries where organ donation has limited national priority. In addition, social, cultural, religious and medical factors play a major role in both LD and DD kidney transplant donation. Most children with ESKD receive adult-sized kidneys. The transplanted kidney has a finite survival and the expectation is that children who require renal replacement therapy from early childhood will probably have 2 or 3 kidney transplants in their lifetime. LD transplant provides better long-term graft survival and is a better option for children. When a living related donor is incompatible with the intended recipient, paired kidney exchange with a compatible unrelated donor may be considered. When the choice is a DD kidney, the decision-making process in accepting a donor offer requires careful consideration of donor history, kidney donor profile index, HLA matching, cold ischemia time, and recipient's time on the waiting list. Accepting or declining a DD offer in a timely manner can be challenging when there are undesirable facts in the donor's history which need to be balanced against prolonging dialysis in a child. An ongoing global challenge is the significant gap between organ supply and demand, which has increased the need to improve organ preservation techniques and awareness for organ donation.

Care of Pediatric Solid Organ Transplant Recipients: An Overview for Primary Care Providers.

As the number of living pediatric solid organ transplant (SOT) recipients continues to grow, there is an increased likelihood that primary care providers (PCPs) will encounter pediatric SOT recipients in their practices. In addition, as end-stage organ failure is replaced with chronic medical conditions in transplant recipients, there is a need for a comprehensive approach to their management. PCPs can significantly enhance the care of immunosuppressed hosts by advising parents of safety considerations and avoiding adverse drug interactions. Together with subspecialty providers, PCPs are responsible for ensuring that appropriate vaccinations are given and can play an important role in the diagnosis of infections. Through early recognition of rejection and posttransplant complications, PCPs can minimize morbidity. Growth and development can be optimized through frequent assessments and timely referrals. Adherence to immunosuppressive regimens can be greatly improved through reinforcement at every encounter, particularly among adolescents. PCPs can also improve long-term outcomes by easing the transition of pediatric SOT recipients to adult providers. Although guidelines exist for the primary care management of adult SOT recipients, comprehensive guidance is lacking for pediatric providers. In this evidence-based overview, we outline the main issues affecting pediatric SOT recipients and provide guidance for PCPs regarding their management from the first encounter after the transplant to the main challenges that arise in childhood and adolescence. Overall, PCPs can and should use their expertise and serve as an additional layer of support in conjunction with the transplant center for families that are caring for a pediatric SOT recipient.

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.