Lorraine Bell

Recurrence of Membranoproliferative Glomerulonephritis Type II in Renal Allografts: The North American Pediatric Renal Transplant Cooperative Study Experience

Membranoproliferative glomerulonephritis type II (MPGN II) is an uncommon form of complement-dependent acquired renal disease. Although it has been recognized since the 1970s that MPGN II recurs almost universally in renal transplants, data regarding the long-term consequences of disease recurrence are limited. Therefore, a retrospective comparative analysis of 75 patients with MPGN II contained in the North American Pediatric Renal Transplant Cooperative Study transplantation database was performed. Five-year graft survival for patients with MPGN II was significantly worse (50.0 +/- 7.5%) compared with the database as a whole (74.3 +/- 0.6%; P < 0.001). Living related donor organs had a significantly better 5-yr survival (65.9 +/- 10.7%) compared with cadaveric donor organs (34.1 +/- 9.8%; P = 0.004). The primary cause of graft failure in 11 (14.7%) patients was recurrent disease. Supplemental surveys were obtained on 29 (38%) of 75 patients. Analysis of these data indicated that recurrent disease occurred in 12 (67%) of the 18 patients with posttransplantation biopsies. Although there was no correlation between pretransplantation presentation, pre- or posttransplantation C3 levels, and either disease recurrence or graft failure, there was a strong association between heavy proteinuria and disease recurrence. The presence of glomerular crescents in allograft biopsies had a significant negative correlation with graft survival. At last follow-up, patients with recurrent disease had significantly higher serum creatinine and qualitatively more proteinuria than patients without biopsy-proven disease. These data indicate that recurrent MPGN II has a significant negative impact on renal allograft function and survival.

Update on childhood urinary tract infection and vesicoureteral reflux.

Urinary tract infection (UTI) is a leading cause of serious bacterial infection in young children. Vesicoureteral reflux (VUR), a common pediatric urologic disorder, is believed to predispose to UTI, and both are associated with renal scarring. The complex interaction of bacterial virulence factors and host defense mechanisms influence renal damage. However, some renal parenchymal abnormalities associated with VUR are noninfectious in origin. Long-term, renal parenchymal injury may be associated with hypertension, pregnancy complications, proteinuria, and renal insufficiency. Optimal management of VUR and UTI is controversial because of the paucity of appropriate randomized controlled trials; there is a need for well-designed studies. The recently launched Randomized Intervention for children with VesicoUreteral Reflux (RIVUR) study hopefully will provide insight into the role of antimicrobial prophylaxis of UTI in children with VUR.

Adolescent dialysis patient transition to adult care: a cross-sectional survey

Adolescent dialysis patients transitioning to adult care are particularly vulnerable, dependent on complex, demanding and life-sustaining treatment. There is little published information on transition practices for this group. Therefore, a survey was carried out to assess the current status. Results are presented for 58 paediatric dialysis centres in North America and Europe. The majority of centres (53%) did not have a fixed cut-off age for transfer. For those that did, it ranged from 17 to 22xa0years, with the median 20.5xa0years and mean [± standard deviation (SD)] of 19.9 (±1.5) years. Only one third of centres reported a transition programme. Less than 20% of young adult patients were perceived to function autonomously at transfer. The paediatric centres had minimal knowledge of resources at the adult receiving sites. For the majority of programs there was a system in place to assist with application for social and health benefits (83%), an adult dialysis unit linked to the paediatric programme (62%) and an opportunity for patients to choose (78%) and visit (83%) the adult unit prior to transfer. Seventy-four percent of centres without a transition programme believed there was a need for one. This is an area clearly in need of attention.

Prednisone withdrawal in pediatric kidney transplant recipients on tacrolimus-based immunosuppression: Four-year data

Abstract: Corticosteroids have been used in renal transplant immunosuppression for over 40u2003yr. Despite their adverse effects, steroid therapy continues to be part of early as well as maintenance immunosuppression in most pediatric renal transplant centers. The association of steroids with growth retardation, weight gain, and acne may be particularly distressing during the critical years of adolescence and young adulthood, increasing the risk of medication non‐adherence. This study reviews the outcomes of pediatric renal transplant patients treated with low‐dose tacrolimus, mycophenolate mofetil, or azathioprine, and planned prednisone withdrawal. Thirty‐seven pediatric renal transplant recipients were withdrawn from steroids. The mean follow‐up after steroid withdrawal was 42±19 months. Graft and patient survival were 100%. The mean serum creatinine levels and calculated creatinine clearances remained stable throughout the period of observation. The mean creatinine clearance was 96±24u2003mL/min/1.73u2003m2 at steroid withdrawal and 93±20u2003mL/min/1.73u2003m2 at the latest follow‐up. Five patients restarted prednisone; in four (11%) it was for suspected or confirmed acute rejection. Improvements were observed in serum lipid profiles, blood pressure, and body mass index. Most patients experienced catchup or stable growth after prednisone withdrawal. Four patients developed viral infections; all were successfully treated. The potential benefits of steroid withdrawal in pediatric renal transplantation are supported by our results.

Balancing organ quality, HLA-matching, and waiting times : Impact of a pediatric priority allocation policy for deceased donor kidneys in quebec

Deceased donor kidney allocation policy must balance the desire for high-quality organs, good human leukocyte antigen (HLA) matching, and minimal waiting times. We describe a 10-fold reduction in waiting times and an improvement in nonimmunologic indices of organ quality for child recipients after a change in organ allocation policy in Quebec, Canada. The new policy gives first priority to children (<18 yr) irrespective of HLA matching or waiting time. HLA matching after the policy change was predictably much worse. This study highlights the trade-offs that must be considered both in setting allocation policy and in decisions for individual recipients. We also consider potential unintended negative effects of such a policy change.

Laparoscopic live donor nephrectomy: The pediatric recipient in a dual-site program

Abstract:u2002 Background: At our institution, laparoscopic live donor nephrectomy (LLDN) is done at a different hospital site than pediatric recipient transplantation, whereas open donor nephrectomy (OLDN) is done in the adjacent operating room. The purpose of this study was to evalute the safety of a dual‐site renal transplantation program by comparing the outcomes of pediatric recipients of LLDN vs. OLDN. Methods: This is a retrospective study of consective pediatric recipients (nu2003=u200310) of LLDN (June 2002 to June 2005) compared to the 10 most recent pediatric recipients of OLDN (March 2001 to June 2005). Renal function was assessed with calculated creatinine clearance using the Schwartz formula and the following outcomes were assessed: delayed graft function, ureteral complications, acute rejection and patient and graft survival. Results are expressed as median (IQR). Results: When comparing the laparoscopic vs. open group, there were no significant differences in recipient age, height, weight, preoperative calculated creatinine clearance and warm ischemia time. Twelve month postoperative creatinine clearance was 88 ml/min/1.73u2003m2 (57–99) in the laparoscopic group (nu2003=u20038) and 66 ml/min/1.73u2003m2 (60–86) in the open group (nu2003=u20039), pu2003=u20030.2. In the LLDN group vs. the OLDN group, delayed graft function was 0% vs. 10% (pu2003=u20031.0), ureteral complications were 20% vs. 30% (pu2003=u20031.0), and acute rejection was 20% vs. 40% (pu2003=u20030.6). In the laparoscopic group, one‐yr patient and graft survival were both 100%, as compared to 100% and 89%, respectively, in the open group. Conclusion: A dual‐site laparoscopic donor nephrectomy program is not associated with adverse pediatric recipicent outcomes when compared to a same‐site open donor approach.

Transitioning the Adolescent Dialysis Patient to Adult Care

There have been dramatic improvements in xadsurvival rates for a range of childhood illnesses during the last quarter century, including end-stage kidney disease (ESKD) [1–3]. In the United States alone, over 500,000 adolescents with chronic medical conditions transfer to adult-focused care every year [4]. Health-care transition (HCT) refers to a purposeful, planned process in which adolescent and young adult patients assume progressively increasing responsibility for their health condition management. The goal, highlighted in a number of consensus statements [5–7], is to maximize lifelong functioning and potential through the provision of high quality, developmentally appropriate uninterrupted health services, as the patient moves from pediatric to adult-focused care. An effective transition process is key to optimizing quality of life and survival for youth with serious ongoing medical conditions.

Transition to Adult Care

This chapter presents the case of an 18-year-old girl receiving hemodialysis in a pediatric dialysis unit. She will soon need to transfer to an adult center. Her situation is complicated by a history of congenital heart disease repaired in infancy but still requiring regular cardiology follow-up. While the patient has a charming personality and is well liked by the dialysis team, she also has difficulty adhering to her dialysis regimen, and as a result her listing for transplant has been delayed. The chapter focuses on five clinical questions: (1) the difference between transfer and transition; (2) the optimal timing for transition planning; (3) anticipation of potential problems she may encounter after transfer to the adult unit, where the intensive, personalized support from her pediatric dialysis team, that she still requires, will likely be lacking; (4) measures that can be taken by the pediatric unit personnel prior to transfer that could mitigate the risks; and (5) the overall aspects of her care that require coordination if transfer is to be successful. Major components of a multifaceted approach to transition are presented, including specific, step-by-step recommendations to guide preparation for transfer. A short list of clinical pearls and a bibliography of key publications conclude the chapter.