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Author(s): Magdalena Riedl Khursigara [sup.1] , Erin Chung [sup.2] [sup.3] , James Tjon [sup.2] [sup.3] , Damien Noone [sup.1] [sup.4] , Rose Chami [sup.5] [sup.6] , Christoph Licht [sup.1] [sup.4] [...], Background C3 glomerulonephritis (C3GN) can be a devastating disease with poor response to immunosuppressive therapy. Complement inhibition with eculizumab has had equivocal results in patients with C3GN. Case-Diagnosis/Treatment We report a case of a 6-year-old boy with C3GN presenting with nephrotic syndrome, severe hypertension and impaired kidney function. He did not respond to initial treatment with prednisone and mycophenolate (mofetil and sodium), and subsequent treatment with standard dosing of eculizumab. Pharmacokinetic studies identified a lack of eculizumab exposure and subsequent intensification of treatment with weekly dosing of eculizumab led to significant clinical improvement: his kidney function normalized, hypertension (weaned off 3 antihypertensive drugs), edema and proteinuria improved. Additionally, exposure to mycophenolic acid (MPA), active metabolite of mycophenolate, determined by area under the concentration-time curve of MPA was low throughout, despite significant dosing escalation. Conclusions This case report demonstrates that individualized therapy guided by therapeutic drug monitoring might be needed in patients with nephrotic range proteinuria treated with eculizumab and mycophenolate (mofetil and sodium), an important finding that needs to be considered for further treatment trials.

Author(s): Amrit Kirpalani [sup.1] [sup.2] , Chia Wei Teoh [sup.1] [sup.2] , Vicky Lee Ng [sup.2] [sup.3] , Anne I Dipchand [sup.2] [sup.4] , Mina Matsuda-Abedini [sup.1] [sup.2] Author Affiliations: [...], Over the past few decades, there has been increasing recognition of kidney disease in children with non-kidney solid organ transplantation. The risk of kidney disease in children undergoing heart or liver transplantation is higher than the general population as the underlying disease and its associated management may directly impair kidney function. Both heart and liver failures contribute to hypoperfusion and kidney ischemia before patients reach the point of transplant. The transplant surgery itself can often be complicated by acute kidney injury (AKI), which may be further exacerbated by a complicated postoperative course. In the short- and long-term post-transplant period, these children are at risk of acute illness, exposed to nephrotoxic medications, and susceptible to rare but severe infections and immunologic insults that may contribute to AKI and chronic kidney disease (CKD). In some, CKD can progress to kidney failure with replacement therapy (KFRT). CKD and KFRT are associated with increased morbidity and mortality in this patient population. Therefore, it is critical to monitor for and recognize the risk factors for kidney injury in this population and mitigate these risks. In this paper, the authors provide an overview of kidney disease pertaining to heart and liver transplantation in children with guidance on monitoring, diagnosis, prevention, and management.

Author(s): Charushree Prasad [sup.1] , Deborah M. Levy [sup.2] [sup.3] , Diane Hebert [sup.1] [sup.3] , Rose Chami [sup.4] [sup.5] , Chia Wei Teoh [sup.1] [sup.3] Author Affiliations: (1) grid.42327.30, [...]

This article discusses the use of valganciclovir, a medication used to prevent cytomegalovirus (CMV) infection in pediatric solid organ transplant patients. The article highlights the challenges and risks associated with valganciclovir, including the potential for neutropenia (low white blood cell count). The study conducted by Patel et al. retrospectively reviewed the use of valganciclovir in pediatric heart, kidney, and liver transplant recipients. The study found that weight-based dosing of valganciclovir resulted in a lower incidence of neutropenia compared to dosing based on body surface area and renal function. The authors suggest that weight-based dosing may be a more effective strategy to prevent neutropenia in pediatric solid organ transplant patients. [Extracted from the article]

Background: Live attenuated varicella vaccine (LAVV) has historically been contraindicated in children who are immunocompromised due to solid organ transplant (SOT) because of safety concerns. Recently, clinical guidelines were developed that support post‐transplant varicella vaccination in selected SOT recipients based on emerging evidence of LAVV safety. This qualitative study sought to explore barriers and facilitators to implementing the new guidelines, as well as acceptability of LAVV among healthcare providers (HCPs) and parents. Methods: HCPs and parents of transplant recipients were recruited from four sites using purposive sampling. Data from semi‐structured interviews were analyzed using an Interpretive Description approach that incorporated data from the interviews, academic knowledge and clinical experience, and drew from Grounded Theory and Thematic Analysis. The theoretical framework used was Adaptive Leadership. Results: Thirty‐four participants (16 HCPs and 18 parents) were included in the analysis. Parents developed skills in adaptive leadership that included strategies to protect their child against infectious diseases. Foundational information that live vaccines were absolutely contraindicated post‐transplant "stuck" with parents and led them to develop strategies other than vaccination to keep their child safe. Some parents struggled to understand that information previously presented as a certainty (contraindication of LAVV) could change. Their approach to adaptive leadership informed their appraisal of the new vaccination guidelines and willingness to accept vaccination. Conclusions: HCPs should adopt a family‐centered approach to communicating changing guidelines that considers parents' approach to adaptive leadership and discusses the changing nature of medical evidence. Trust between HCPs and parents can facilitate these conversations. [ABSTRACT FROM AUTHOR]

Author(s): Chia Wei Teoh[sup.1] , Irwin Gill[sup.1] , Rania Haydar[sup.1] , Melanie Cotter[sup.2] , Deirdre Devaney[sup.3] , Niamh Marie Dolan[sup.1] , Michael Riordan[sup.1] , Mary Waldron[sup.1] , Atif Awan[sup.1] Author [...]

Transition of care of adolescent kidney transplant recipients - a call to action Kidney transplantation is the standard of care for children and adolescents with end-stage kidney disease (ESKD). Ideally, this can be provided within a joint pediatric-adult kidney transplant transition clinic with the eventual transfer of care to a young adult kidney transplant clinic, where ongoing integration of the young adult into the adult care model can be supported. [Extracted from the article]

Objective: Self‐management for patients who have undergone solid organ transplantation is demanding and a challenge for adolescents transitioning into adult‐oriented healthcare systems. This study explores whether adolescent and young adult solid organ transplant patients support the use of online peer support programs that encourage peer mentorship as an approach to improve disease self‐management. Methods: A qualitative descriptive design comprised of semi‐structured interviews with adolescent and young adult transplant patients. Individual interviews were audio‐recorded, transcribed verbatim, and subject to content analysis. Emergent categories and themes were refined through member checking and team consensus following saturation. Results: Interviews were conducted across organ groups with 15 participants (60% female) ages 14 to 22 years. Participants expressed unanimous support for an online peer support mentorship program to aid disease self‐management in the pediatric transplant patient population. Three themes emerged from the interviews: (a) self‐management care can be "taxing"; (b) there would be value in peer mentorship for adolescent transplant patients; and (c) online peer mentorship is the "best" option but still requires relationship building. Logistical preferences of an online peer mentorship program were solicited. The preferred peer "match" was someone of the same organ transplant group and gender who was able to have weekly contact via texting. Conclusions: Creating tailored, online peer mentorship programs is gaining evidence to justify further development. Findings from this study will support program modifications for adolescent and young adult solid organ transplant patients. Next steps will involve usability and feasibility testing of an adapted online program for this patient group. [ABSTRACT FROM AUTHOR]