Health‐related quality of life among siblings of kidney transplant recipients

Studies are increasingly recognizing health‐related quality of life (HRQOL) as a key pediatric outcome in both clinical and research settings and an essential health outcome measure to assess the effectiveness of medical treatment. However, it has not yet been studied among the healthy siblings of kidney transplant recipients. The aim of this study, therefore, is to examine HRQOL among this population. We asked the following three groups to complete a validated measure of HRQOL among children (KIDSCREEN‐52 ) : siblings of children who had received kidney transplants (n = 50), kidney transplant recipients (n = 43), and a healthy control group (n = 84). We found that siblings of kidney transplant patients exhibited lower scores for financial resources and autonomy than kidney transplant recipients. They also served lower on physical well‐being, financial resources, autonomy, and parent relations/home life than the control group. However, they scored higher on social acceptance than kidney transplant recipients. Our study underscores the importance of assessing HRQOL in families including a child diagnosed with a chronic illness. Siblings require social and psychological support to promote coping and adaptation.     

Quality of life in pediatric liver transplantation in a single‐center in South America

Sanchez C, Eymann A, De Cunto C, D’Agostino D. Quality of life in pediatric liver transplantation in a single‐center in South America.
Pediatr Transplantation 2010: 14: 332–336. © 2009 John Wiley & Sons A/S. Abstract: HRQOL in children after LT has not been systematically measured in transplant recipients from South American countries. The aim of this study was to determine the HRQOL using a validated measure for children. The CHQOL‐PF50 was completed by the parents of 54 patients after the clinical assessment. Subscale mean scores were compared with both a normal population (n = 274) and a group of chronic illness patients with Juvenile Idiopathic Arthritis (n = 23). Compared with the normal population, LT recipients had lower subscales scores for general health perceptions, role/social emotional, mental health, and parental impact on time. Bodily pain was significantly lower in our study group. Both mean physical and psychosocial summary scores were lower compared to the normal population but similar to the JIA group. Within the LT population, gender, original diagnosis, type of immunosuppression, type of transplant and time elapsed since LT did not significantly influence any of the summary scores. Our study showed LT children’s physical and psycho‐social areas were lower compared with those of the general population. LT children had less limitations due to pain. Family functioning appeared normal.     

Quality of life in a cohort of patients diagnosed with renal failure in childhood and who received renal transplant

Tozzi AE, Mazzotti E, Di Ciommo VM, Dello Strologo L, Cuttini M. Quality of life in a cohort of patients diagnosed with renal failure in childhood and who received renal transplant. Abstract: Studies on HRQOL on kidney‐transplanted young adults who had a diagnosis of chronic renal failure (CRF) in the pediatric age are uncommon. We studied HRQOL and its predictors in a sample of young adults with CRF in childhood who underwent a renal transplant. We recruited patients ≥18 yr old with renal transplant. We measured HRQOL by a standardized questionnaire on lifestyle, Short Form‐36 (SF‐36; including a PCS and a MCS; scale: 0–100), the GHQ (for short‐term changes in mental health; scale: 0–36), and the MSPSS (with scales for family, friends, and significant others; scale: 0–100). We assessed the association of potential predictors of HRQOL through multiple linear regression models. We studied 66 patients aged 18–34 yr. The average PCS score was 76.4, and the average MCS score was 73.9. The mean GHQ total score was 14.8, and the total scale MSPSS mean score was 70. Severe comorbidities significantly affected the PCS score. Individuals with severe comorbidities had lower PCS scores.     

Good outcome of the single‐center pediatric kidney transplant program in Abu Dhabi

Renal transplantation is the treatment of choice for ESRD in children. It is associated with better quality of life, growth of children, and improved long‐term survival. The aim of the study was to evaluate the outcomes of pediatric renal transplantation at a tertiary care center in UAE. A retrospective chart review was undertaken for all the pediatric renal transplants performed at Sheikh Khalifa Medical City, Abu Dhabi, UAE, over the past 9 years. The study evaluated the demographic data, outcomes, and complications of pediatric renal transplantation. The post‐transplantation outcomes including surgical complications, documented infections, graft rejection, graft and patient survival, effect on growth, and eGFR were reviewed. Between 2010 and 2018, 30 pediatric patients underwent renal transplantation. The follow‐up period ranged from 1 to 9 years with a mean of 3.3 years. The mean age of the patients at the time of transplant was 9.8 years, and 56.7% were males. Prior to the transplantation, the majority of the recipients were on peritoneal dialysis (70.0%). Main source of renal donation at our center was from LRD, chiefly from parents. Patient survival at 1 and 5 years was 100% and 96.7%, respectively. Graft survival at 1 and 5 years was 96.7% and 83.3%, respectively. During the 9‐year follow‐up period, 5 (16.7%) recipients experienced rejection episode. This study demonstrates that during 5‐year period, pediatric kidney transplantation program has achieved optimal patient (96.7%) and graft (83.3%) survival rates and is comparable to well‐established centers.     

Size‐reduced lung transplantation in children – an option worth to consider!

Benden C, Inci I, Weder W, Boehler A. Size‐reduced lung transplantation in children – an option worth to consider!
Pediatr Transplantation 2010: 14:529–533. © 2009 John Wiley & Sons A/S. Abstract: Lung transplantation is an accepted therapy for pediatric end‐stage lung disease. However, there is a shortage of suitable donor organs. Therefore, the use of downsized lung allografts seems a valuable option. We report our experience of downsized pediatric lung transplantation in comparison with standard full‐size pediatric lung transplantation over one decade. Pediatric recipients undergoing downsized or standard lung transplantation were included (January 1997–December 2006). We compared pretransplant clinical data and surgical and post‐operative complications and post‐transplant outcome. Ten pediatric lung transplants were performed (median patient age 15.6 yr [12.3–17.8]). Nine of 10 patients had CF. Five patients underwent standard full‐size lung transplantation; five had downsized lung transplants. “Downsized” recipients had significantly lower median height and weight Z‐scores. Donor/recipient length difference was significantly greater in the “Downsized” Group (p < 0.05). All patients had comparable post‐transplant functional outcome without additional surgical complications or morbidities in “downsized” recipients. Median post‐transplant survival was 65 months (5–77) in the “Standard” Group compared to 86 months (64–121) in the “Downsized” Group (p = 0.1). Our data suggest that downsized lung transplantation in pediatric recipients may have post‐transplant outcomes comparable to full‐size lung transplantation without significant complications.     

Comparison of Histidine‐Tryptophan‐Ketoglutarate and University of Wisconsin preservation solutions in pediatric liver transplantation

UW and HTK solutions are the two primary organ preservation solutions most used in the United States. This study analyzes use of the two solutions in all pediatric liver transplants performed at a single center between 2001and 2017. Outcome measures included early graft function, as well as graft and patient survival. Bile duct complications were reviewed. Operative technique, immunosuppressive protocols, and donor acceptance criteria remained uniform among participating surgeons throughout the study period. There were 104 pediatric liver transplants with complete data during the study period, 75 preserved with HTK (68%) and 29 with UW (26%). Demographics were similar. Cold and warm ischemia times were similar. Peak ALT post‐transplant was higher in the UW group at both peak and post‐transplant day 3. The peak TB levels were similar. Bile duct strictures were more common in the UW group (44% vs 16%, P < .01). Early graft survival was statistically similar at 7‐, 90‐ and 365‐days post‐transplant. Cox regression graft survival was similar at 10‐years. This study suggests that use of HTK in pediatric liver transplantation is safe with outcomes similar to UW, though bile duct stricture rates may be lower with HTK.     

Lymphocele after pediatric kidney transplantation: Incidence and risk factors

Lymphocele is a well‐known postoperative complication after kidney transplantation. The aim of this study was to analyze time trend incidence, risk factors, and outcome of post‐transplant lymphocele in a large pediatric cohort. This is a retrospective single institution review of 241 pediatric kidney transplants performed from 2000 to 2013. Etiology of end‐stage renal disease, recipient age and gender, transplant year, BMI percentile for age, type of dialysis, living/non‐living related donor, acute rejection, and multiple transplantations were analyzed in association with lymphocele formation. Fourteen of 241 (5.81%) children developed a postoperative lymphocele. There has been a reduction in the incidence of lymphocele after 2006 (3.22% vs. 8.55%, p < 0.05). Significant risk factors for lymphocele were older age (≥11 yr), transplant before 2006, male gender, BMI percentile for age ≥95%, and multiple transplantations (p < 0.05). The one‐yr graft survival was significantly reduced in the group with lymphocele compared with control (81.2% vs. 92.51%, p < 0.04). This is the first pediatric report showing the following risk factors associated with post‐transplant lymphocele: age ≥11 yr, male gender, BMI for age ≥95%, and multiple transplantations. A lymphocele can contribute to graft loss in the first‐year post‐transplant.     

The use of vascular homografts in pediatric small bowel transplantation: Single‐center experience over a decade

Intestinal transplantation in children has evolved with more isolated small intestine transplants being performed compared to combined liver‐intestine transplants. Consequently, surgical techniques have changed, frequently requiring the use of vascular homografts of small caliber to revascularize the isolated small intestine, the impact of which on outcomes is unknown. Among 106 pediatric intestine and multivisceral transplants performed at our center since 2003, 33 recipients of an isolated small intestine graft were included in this study. Outcome parameters were thrombotic complications, graft, and patient survival. A total of 29 of 33 (87.9%) patients required arterial and/or venous homografts from the same donor, mainly iliac or carotid artery and iliac or innominate vein, respectively (donor's median age 1.1 years [2 months to 23 years], median weight 10 kg [14.7‐48.5]). Post‐transplant, there were three acute arterial homograft thromboses and one venous thrombosis resulting in two peri‐operative graft salvages and two graft losses. Three of four thromboses occurred in patients with primary hypercoagulable state, including the two graft losses. Overall, at a median of 4.1 years (1‐10.2) from transplant, 29 of 33 (88%) patients are alive with 26 of 33 (79%) functioning grafts. The procurement of intact, size‐matched donor vessels and the management of effective post‐transplant anticoagulation are critical.     

Post‐transplant lymphoproliferative disorder: No relationship to recombinant human growth hormone use in Australian and New Zealand pediatric kidney transplant recipients

PTLD is a potentially life‐limiting complication of pediatric transplantation. Previous registry‐based studies in renal transplantation have suggested a link between rhGH use and PTLD. In this study, demographic and transplant data on those aged <18 yr and transplanted between 1991 and 2008 were collected from the ANZDATA Registry. Associations between gender, age at time of transplant, recipient CMV and EBV status, use of monoclonal antibody therapy, and use of rhGH were studied as potential predictors of PTLD. Among 650 transplants, there were 20 cases (3.1%) of PTLD, with half presenting within two yr post‐transplant. Eight patients exposed to rhGH at any time developed PTLD, and this association was not statistically significant (RR = 1.5[0.6–3.4], p = 0.36). On multivariate analysis, there were no significant predictors for PTLD. In this study, previously identified potential risk factors were not identified as significant predictors for the development of PTLD. Although limited sample size may affect our ability to infer safety, this large retrospective cohort study does not suggest an increased risk of PTLD in pediatric kidney transplant recipients who received rhGH treatment.     

Health-related quality of life in pediatric liver transplant recipients compared with other chronic disease groups

Limbers CA, Neighbors K, Martz K, Bucuvalas JC, Webb T, Varni JW, Alonso EM, on behalf of the Studies of Pediatric Liver Transplantation (SPLIT) Functional Outcomes Group (FOG). Health‐related quality of life in pediatric liver transplant recipients compared with other chronic disease groups.
Pediatr Transplantation 2011: 15: 245–253. © 2010 John Wiley & Sons A/S. Abstract: This cross‐sectional, multicenter cohort study compares the level of HRQOL of pediatric LT recipients to children with other chronic health conditions. LT sample included 873 children who survived at least 12 months following LT. Six chronic disease samples were compiled from numerous studies, including over 800 patients with JRA, type 1 diabetes, cancer in remission, cardiac disease, end‐stage renal disease, and inflammatory bowel disease. Generic HRQOL was measured from both the parental and patient perspective using the PedsQL? 4.0 Generic Core Scales. Pediatric LT patients reported better physical health than children with JRA. According to parents, pediatric LT recipients had better HRQOL than children on renal dialysis on all domains except school functioning. Across all domains but emotional functioning, pediatric LT recipients reported significantly lower HRQOL than children with type 1 diabetes. Overall, pediatric LT patients reported HRQOL comparable to that of children who had undergone renal transplantation and patients with cancer in remission. Pediatric LT patients manifested impaired HRQOL similar to that of children with chronic diseases and these data suggest that they face ongoing challenges that warrant monitoring and indicate a need for interventions to improve their HRQOL.     

Bortezomib in the treatment of antibody‐mediated rejection in pediatric kidney transplant recipients: A multicenter Midwest Pediatric Nephrology Consortium study

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.     

Transplantation of adult‐size kidneys in small pediatric recipients: A single‐center experience

RTx of adult‐size kidneys presents a size mismatch in small pediatric recipients, and there are potential surgical complications. This study reveals the outcomes of intra‐ and extraperitoneal RTx in low‐weight (less than 15 kg) pediatric recipients. We studied 51 pediatric patients weighing less than 15 kg who received a living‐related donor renal transplant between 2009 and 2017. The intraperitoneal (group A, n = 24) and extraperitoneal (group B, n = 27) approaches were compared. In group A, the mean age, Ht, and weight were 3.8 ± 1.6 years, 83.7 ± 6.5 cm, 10.5 ± 1.8 kg; in group B, 5.0 ± 1.9 years, 95.3 ± 7.3 cm, and 13.0 ± 1.4 kg. Single renal artery grafts (21 in group A and 16 in group B) and double renal artery grafts (three in group A and 11 in group B) were performed. Of the patients with double renal artery transplants, one in group A and six in group B underwent ex vivo arterial reconstruction. The eGFR (mL/min/1.73 m²) at 1‐week post‐transplant in group A was significantly higher than that in group B; the eGFRs at 4 weeks post‐transplant did not differ. One graft was lost in group B because of vascular thrombosis. Post‐transplant complications included ileus and transplant ureteral stenosis. There was no significant difference in 5‐year graft survival rate (group A 100%, group B 91.7%). Both transplant approaches are feasible to adapt to a size mismatch between the adult‐size donor kidney and low‐weight pediatric recipients.     

Outcomes of pediatric living donor kidney transplantation: A single‐center experience

Renal transplantation is the treatment of choice for children with ESRD offering advantages of improved survival, growth potential, cognitive development, and quality of life. The aim of our study was to compare the outcomes of LDKT vs DDKT performed in children at a single center. Retrospective chart review of pediatric patients who underwent kidney transplantation from 2005 to 2014 was performed. Ninety‐one renal transplants were accomplished, and 31 cases (38.27%) were LDKT, and in 96.7% of the cases, the graft was obtained through laparoscopy. Thirty‐four receptors weighted <25 kg. LDKT group had statistically significant lower cold ischemia times than DDKT one. Complication rate was 9.67% for LDKT and 18.33% for DDKT. eGFR was better in LDKT. Patient survival rate was 100% for LDKT and 98.3% for DDKT, and graft survival rate was 96.7% for LDKT and 88.33%‐80% for DDKT at a year and 5 years. Our program of pediatric kidney transplantation has achieved optimal patient and graft survival rates with low rate of complications. Living donor pediatric kidney transplants have higher patient and better graft survival rates than deceased donor kidney transplants.     

De novo leukocyte chemotactic factor 2 amyloidosis in a pediatric renal allograft, 15 years post‐transplant

Leukocyte chemotactic factor 2 amyloidosis (ALECT2) is a recently described form of systemic amyloidosis, which most commonly affects the kidney and liver. The LECT2 protein is produced during inflammatory processes, but its precise function in renal diseases in unclear. ALECT2, however, is known to be a relatively common form of renal amyloidosis, after amyloid light chain and serum amyloid A types and is most often seen in patients of Hispanic ethnicity. ALECT2 can occur de novo or as recurrent disease in kidney transplants. We present the first case, to our knowledge, of de novo ALECT2 in a pediatric kidney transplant patient, 15 years post‐transplant.     

Plasmapheresis‐resistant acute humoral rejection successfully treated with anti‐C5 antibody

Even if kidney graft survival has improved during the last decades, sensitized pediatric patients are an emerging problem. We describe a 17‐yr‐old male who lost his first graft due to chronic rejection becoming hyperimmunized (CDC PRA 99.61%). A desensitization protocol based on high‐dose IVIG, PP, and two Mabthera^® infusions was performed with minor response (CDC PRA post‐desensitization 80%). One month after his second non‐living transplant, he developed a biopsy‐proven AMR; post‐transplant immunological monitoring showed the presence of donor‐specific anti‐DQ5 antibodies (DSA, MFI 20.000). He received methylprednisolone pulses and 45 PP sessions without clinical response; eculizumab was then used to salvage a kidney undergoing severe PP‐resistant rejection. A biopsy performed after the fourth eculizumab infusion showed complete resolution of AMR. Eculizumab infusions were then continued for the first year post‐transplantation. Two yr after transplantation, graft function is stable. Anti‐C5 therapy may represent an effective therapeutic option in pediatric patients with PP‐resistant AMR.     

Impact of active antibody‐mediated rejection treatment on donor‐specific antibodies in pediatric kidney transplant recipients

AMR is a major cause of graft loss after kidney transplantation. We evaluated a retrospective cohort of 13 pediatric kidney transplant patients diagnosed with active AMR. All 13 patients were treated with plasmapheresis (PP), IVIg, and rituximab. Anti‐HLA DSAs were measured at the time of transplantation, AMR diagnosis, 30 days post‐rejection treatment, 90 days post‐rejection treatment, and 24 ± 12 months post‐AMR. A total of 68 DSAs were identified from 13 patients at the time of active AMR diagnosis. The primary objective of this study was to differentiate treatment response rates between class I and class II anti‐HLA DSA post‐AMR treatment. Overall, DSAs were significantly reduced at 30 days, and the reduction was sustained at 90 days post‐treatment, even for class II anti‐HLA and strongly positive DSAs. A significant difference between class I and class II anti‐HLA DSA was observed at 30 days; however, between class significance was lost at 90‐day follow‐up due to continued class II anti‐HLA DSA treatment response. Low DSA strength was predictive of treatment response. eGFR demonstrated significant improvement 90 days after AMR diagnosis compared to the initial value at the time of AMR, and the effect was sustained for 12 months. These results suggest that the AMR treatment is effective in pediatric kidney transplant recipients with an early diagnosis of active AMR across both class I and class II anti‐HLA DSAs.     

“Inverted” positioning of renal allograft during kidney transplantation in children and adolescents: A single‐institution comparative analysis

Renal transplantation is the treatment of choice in children with end‐stage renal failure. Limitations in patient anatomy or a short donor renal vein may necessitate intraoperative inversion of the kidney. There is little evidence to support the use of this surgical technique, and no evidence in the pediatric population. This study identifies the perioperative and post‐operative outcomes of inverted renal transplants in pediatric patients. We reviewed all patients having a renal transplant between January 2012 and December 2016 and collected short‐ and long‐term outcomes of patients who received an inverted allograft. Early graft function was defined as the time to reach creatinine nadir. During this time, our hospital performed 81 transplants, and 50 (62%) were from deceased donors, including the 6 (12%) patients who received inverted renal grafts. Half (3/6) were female, 5/6 (83%) were dialysis‐dependent, and the median age at surgery was 13 years (range 9‐16 years). There was no significant difference in mean creatinine nadir values (P = 0.518) and the time to creatinine nadir mean values (P = 0.190) between the upright and inverted renal transplant groups. There were also no significant differences in rates of post‐operative complications between the upright and inverted allograft recipients. Inversion of renal allografts in pediatric patients is a viable surgical technique to compensate for shortcomings in patient anatomy or in special cases of renal transplantation involving a short donor renal vein. Future research should focus on outcomes of a larger group of pediatric inverted renal transplant patients.     

Risk factors for the development of antibody‐mediated rejection in highly sensitized pediatric kidney transplant recipients

ABMR remains a significant concern for early graft loss, especially for those who are HS against HLA antigens. We sought to determine the risk factors leading to ABMR in HS pediatric kidney transplant recipients. From January 2009 to December 2015, 16 HS pediatric kidney transplant patients at our center (age range 2‐21) were retrospectively reviewed for outcomes and risk factors for ABMR. All HS patients received desensitization with high‐dose IVIG/rituximab prior to transplant. Two groups were examined: ABMR⁺ (n = 7) and ABMR^? (n = 9). Patient survival was 100%; however, one patient in the ABMR⁺ group suffered graft loss from ABMR 16 months post‐transplant. ABMR⁺ patients had higher Class I PRA at the time of transplant (Class I: 73.1 ± 19.1 vs 49.1 ± 28.3, P = .075), although not statistically significant. ABMR⁺ patients were more likely to have a history of transplant nephrectomy (P = .013). The characteristic that most strongly correlated with ABMR was the DSA‐RIS (P = .045), a scoring system used to quantify cumulative intensity of all DSA. In conclusion, DSA, as quantified by the RIS at the time of transplant, should be considered as part of the initial allocation strategy and patients with high RIS monitored closely for ABMR post‐transplant.     

The effect of peri‐transplant plasmapheresis in the prevention of recurrent FSGS

Many pediatric centers utilize a variety of protocols including preemptive plasmapheresis to prevent the recurrence of FSGS post‐transplant. But the effectiveness of this expensive, time‐consuming process of plasmapheresis in the prevention of FSGS recurrence is still unclear. We retrospectively reviewed all pediatric cases of FSGS in our center that received a kidney transplant and compared the transplant and patient outcomes of those transplanted after 2006 who received pretransplant plasmapheresis to those prior to 2006 who did not. Of the 57 children with FSGS, 31 and 26 were transplanted before and after 2006, respectively. The cohorts differed significantly in keeping with the center immunosuppression protocol changes, and prior to 2006, the recipients were significantly younger. All children with FSGS transplanted after 2006 underwent three and one sessions of 1.0 plasma volume/exchange plasmapheresis with fresh frozen plasma replacement prior to the transplant in living and deceased donors, respectively, in addition to five sessions of every other day post‐transplant pheresis. The incidence (27% vs 26%, P = 1.0) and time to recurrence of FSGS in the kidney allograft (P = .22) were not significantly different in patients that did and did not undergo prophylactic plasmapheresis. We need to re‐evaluate the role of preemptive plasmapheresis in the prevention of FSGS recurrence in a prospective multicenter study.