Improving Clinical Care for Children With CKD: A Report From a National Kidney Foundation Scientific Workshop.

Development of clinical guidelines and recommendations to address the care of pediatric patients with chronic kidney disease (CKD) has rarely included the perspectives of providers from a variety of health care disciplines or the patients and parents themselves. Accordingly, the National Kidney Foundation hosted an in-person, one and a half-day workshop that convened a multidisciplinary group of physicians, allied health care professionals, and pediatric patients with CKD and their parents, with the goal of developing key clinical recommendations regarding best practices for the clinical management of pediatric patients living with CKD. The key clinical recommendations pertained to 5 broad topics: addressing the needs of patients and parents/caregivers; modifying the progression of CKD; clinical management of CKD-mineral and bone disorder and growth retardation; clinical management of anemia, cardiovascular disease, and hypertension; and transition and transfer of pediatric patients to adult nephrology care. This report describes the recommendations generated by the participants who attended the workshop.

Toward a better understanding of transition from paediatric to adult care in type 1 diabetes: A qualitative study of adolescents.

AIMS: Type 1 diabetes is associated with significant morbidity, with an increasing risk of acute diabetes-related complications in adolescence and emerging adulthood. Purposeful transition from paediatric to adult-oriented care could mitigate this risk but is often lacking. Detailed understanding of the perspectives of adolescents in their final year of paediatric care is essential to inform delivery of transition care programs. METHODS: We conducted semi-structured interviews with adolescents (aged 17 years) with type 1 diabetes at an academic institution from April 2017 to May 2018. Participants were recruited through convenience sampling. Sixty-one interviews were transcribed for analysis. Coding followed the principles of thematic analysis. RESULTS: Thirty-six percent of participants were male, and participants were from diverse socioeconomic backgrounds. We found three overarching themes in our analysis: first, difficulties navigating changing relationships with parents and healthcare teams; second, the need to increase type 1 diabetes self-management and differing comfort levels based on age of diagnosis; and third, perceived responsibilities for transition care preparation (for both the paediatric team and adolescents themselves) focused not only on type 1 diabetes-specific skills but also on healthcare system structures. CONCLUSIONS: Our findings suggest that novel transition programs addressing changing inter-personal relationships, disease-specific self-management (adapted for age of diagnosis), and healthcare system navigation, supported by parents and peers, may be needed to improve transition care for adolescents with type 1 diabetes.

Assessment of identity and quality of life in diabetic and renal transplant adolescents in comparison to healthy adolescents.

Identity development represents a central task of adolescence. Identity achievement is characterized by a coherent sense of who one is following a period of exploration and can help navigate the challenges of adulthood. This study examined identity within a quality of life (QOL) context in 85 adolescents with a renal transplant or with Type 1 diabetes in comparison to 90 healthy controls. Results revealed significant differences in ideological identity, with patients showing higher levels of diffusion and controls showing higher levels of foreclosure. No differences with respect to interpersonal identity, QOL, perceived control over the QOL domains, and perceived opportunities for growth and development were found. Future research should assess identity and QOL over a longer period of time to determine whether differences between chronically ill and healthy young adults can be detected.

Native nephrectomy prior to pediatric kidney transplantation: biological and clinical aspects.

BACKGROUND: Pre-transplant nephrectomy is performed to reduce risks to graft and recipient. The aims of this study were to evaluate (1) indications, surgical approach, and morbidity of native nephrectomy and (2) the effects of kidney removal on clinical and biological parameters. METHODS: This study was designed as a single-center retrospective cohort study in which 49 consecutive patients with uni- or bilateral native nephrectomies were identified from a total of 126 consecutive graft recipients in our pediatric kidney transplantation database between 1992 and 2011. Demographic, clinical, and laboratory details were extracted from charts and electronic records, including operation reports and pre- and post-operative clinic notes. RESULTS: Of the 49 nephrectomized patients, 47% had anomalies of the kidneys and urinary tract, 22% had cystinosis, 12% had focal segmental glomerulosclerosis, and 6% had congenital nephrotic syndrome. Nephrectomy decisions were based on clinical judgment, taking physiological and psychosocial aspects into consideration. Nephrectomy was performed in patients with polyuria (>2.5 ml/kg/h) and/or large proteinuria (>40 mg/m(2)/h), recurrent urinary tract infection or (rarely) hypertension. Urine output decreased from (median) 3.79 to 2.32 ml/kg/h (-34%), and proteinuria from 157 to 100 mg/m(2)/h (-40%) after unilateral nephrectomy (p=0.005). After bilateral nephrectomy, serum albumin, protein and fibrinogen concentrations normalized in 93, 73, and 55% of nephrectomized patients, respectively. Clinically relevant procedure-related complications (peritoneal laceration, hematoma) occurred in five patients. CONCLUSION: In summary, we demonstrate quantitatively that native nephrectomy prior to transplantation improved serum protein levels and anticipated post-transplant fluid intake needs in select children, reducing the risk of graft hypoperfusion and its postulated consequences for graft outcome.

The impact of age at transfer from pediatric to adult-oriented care on renal allograft survival.

Immaturity among individuals transferred from pediatric to adult-oriented care at a young age may leave them vulnerable to higher graft failure risks than in individuals transferred older. We sought to determine the impact of age at transfer on renal allograft failure rates. We evaluated graft failure rates among 440 kidney recipients recorded in the UNOS database (1987-2007), who had been transferred from pediatric to adult care. Transfers were identified using the center codes recorded at yearly data collection. Failure rates for those transferred early (<21 yr old) were compared with rates for those transferred late (≥21 yr old); time-dependent Cox models were used to estimate the additional risk of graft failure associated with early vs. late transfer. The age-standardized failure rate was 12.9 per 100 person-years among those transferred early, and 8.7 per 100 person-years among those transferred late. Compared with individuals the same age who had transferred late, graft failure rates were 58% higher ([95% confidence interval: 7%, 134%], p = 0.02) among those who had transferred early. Younger age at transfer to adult care is associated with higher graft failure rates. Transfer to adult-oriented care at <21 yr of age should be undertaken with caution.

Cryptogenic organizing pneumonia after rituximab therapy for presumed post-kidney transplant lymphoproliferative disease.

Cryptogenic organizing pneumonia (COP, formerly bronchiolitis obliterans organizing pneumonia) is rare in children. We describe an 11-year-old girl with Epstein-Barr virus (EBV) reactivation/presumed post-transplant lymphoproliferative disease (PTLD) 15 months after undergoing a deceased donor kidney transplantation. Treatment with reduced immunosuppression, ganciclovir, and cytomegalovirus immunoglobulin was complicated by severe graft rejection, prompting therapy with methylprednisolone, anti-thymocyte globulin and four weekly doses of rituximab (total 1500 mg/m(2)). Tacrolimus- and prednisone-based anti-rejection prophylaxis was complemented with low-dose sirolimus. When the lactate dehydrogenase and uric acid levels rose 10 weeks after the first rituximab infusion and bilateral pulmonary nodules were detected by computerized tomography, recurrence of PTLD was suspected. Open lung biopsy of the clinically asymptomatic patient identified the nodules as COP, characterized by abundant CD3(+) T-cells, few B-cells, and the absence of EBV, cytomegalovirus, or adenovirus antigens. With normalization of the peripheral B-cell count, EB viremia reappeared and persisted, despite minimal immunosuppression. Four years later, the patient was diagnosed with classical Hodgkin lymphoma-type PTLD with multiple pulmonary and abdominal nodes. This first report of rituximab-associated, pediatric COP highlights the risk of pulmonary complications after treatment with B-cell depleting agents in solid organ transplant recipients, and the importance of a histopathologic diagnosis and vigilant follow-up of such lesions.

Improving the transition from pediatric to adult diabetes care: the pediatric care provider's perspective in Quebec, Canada.

OBJECTIVES: The transition from pediatric to adult care is a high-risk period for the emerging adult with diabetes. We aimed to determine adequacy of pediatric transition care structures and explore the pediatric diabetes care provider's perceptions of transition care. RESEARCH DESIGN AND METHODS: In-depth interviews with pediatric diabetes care providers from 12 diabetes centers in Quebec were conducted. We queried alignment with Got Transition's six core elements of healthcare transition, experiences, and barriers to transition care. Interview transcripts were reviewed for themes. RESULTS: Three centers (25%) reported having any elements of formal and structured transition care preparation and planning. When referrals were within center (n=8), pediatric providers perceived that transition was smoother; information sharing relied heavily on verbal communication rather than documented medical summaries. Barriers included lack of adult providers, less flexibility in adult care scheduling, patient struggles with multiple new adult responsibilities, and insufficient understanding by adult providers of these challenges. There was a perception that the quality of pediatric care was better than adult care. Moving out of the pediatric care geographical region appeared to increase risk for poor follow-up. Patient satisfaction and regular follow-up in adult care were thought to be good measures of transition success. Programs that included overlap between pediatric and adult care were perceived as ideal. CONCLUSIONS: Important gaps in transition care practices persist. Efforts should focus on improving education in transition practices for pediatric care providers and establishing formal transition policies and structures at the institutional level.

Health care transition for adolescents with CKD-the journey from pediatric to adult care.

The design of Health Care Transition (HCT) services for adolescents and emerging adults with CKD or end-stage kidney disease (ESKD) needs to take into account patient cognition/developmental stage, family factors, and health resources within the hospital setting and community. Patient and family education is fundamental and teaching and learning tools must be literacy-accessible. Adolescents and emerging adults with CKD/ESDK have complex medical and dietary regimes, and therapeutic adherence is important for optimizing their health, quality of life, and longevity. Health providers need to identify ways of engaging them to become successful disease self-mangers. Interdisciplinary collaboration between the pediatric- and adult-focused health care teams and the services of a dedicated transition coordinator are paramount to ensure clear communication between the patient and the health professionals involved. Valid measurement tools to monitor and assess the HCT process and health outcomes need to be developed. The aims of planned HCT for adolescents and/or emerging adults with CKD/ESKD are anchored by the goals of optimizing health outcomes, health-related quality of life, and continuous quality improvement. The care of young people with CKD/ESKD can be both challenging and rewarding; we offer strategies for planned HCT services geared to these vulnerable patients.

Transition of care to adult services for pediatric solid-organ transplant recipients.

The importance of transition to adult health care for young people with chronic conditions is increasingly recognized. Ensuring effective engagement with adult services for adolescents and young-adult solid-organ transplant recipients is as critical for immediate graft survival as it is for their future health and well-being. This article (1) examines the definitions of adolescence and emerging adulthood and some of the challenges of these phases of life, (2) discusses elements that may influence motivation and engagement and enhance communication and adherence for adolescents and young adults, (3) highlights important areas in education, vocational planning, and quality of life for transplant recipients, (4) reviews tasks and challenges during the transition, and (5) provides specific transition recommendations, for both transplant health care professionals and for primary care providers practicing outside transplant centers.

Outcomes from pandemic influenza A H1N1 infection in recipients of solid-organ transplants: a multicentre cohort study.

BACKGROUND: There are few data on the epidemiology and outcomes of influenza infection in recipients of solid-organ transplants. We aimed to establish the outcomes of pandemic influenza A H1N1 and factors leading to severe disease in a cohort of patients who had received transplants. METHODS: We did a multicentre cohort study of adults and children who had received organ transplants with microbiological confirmation of influenza A infection from April to December, 2009. Centres were identified through the American Society of Transplantation Influenza Collaborative Study Group. Demographics, clinical presentation, treatment, and outcomes were assessed. Severity of disease was measured by admission to hospital and intensive care units (ICUs). The data were analysed with descriptive statistics. Proportions were compared by use of chi(2) tests. We used univariate analysis to identify factors leading to pneumonia, admission to hospital, and admission to an ICU. Multivariate analysis was done by use of a stepwise logistic regression model. We analysed deaths with Kaplan-Meier survival analysis. FINDINGS: We assessed 237 cases of medically attended influenza A H1N1 reported from 26 transplant centres during the study period. Transplant types included kidney, liver, heart, lung, and others. Both adults (154 patients; median age 47 years) and children (83; 9 years) were assessed. Median time from transplant was 3.6 years. 167 (71%) of 237 patients were admitted to hospital. Data on complications were available for 230 patients; 73 (32%) had pneumonia, 37 (16%) were admitted to ICUs, and ten (4%) died. Antiviral treatment was used in 223 (94%) patients (primarily oseltamivir monotherapy). Seven (8%) patients given antiviral drugs within 48 h of symptom onset were admitted to an ICU compared with 28 (22.4%) given antivirals later (p=0.007). Children who received transplants were less likely to present with pneumonia than adults, but rates of admission to hospital and ICU were similar. INTERPRETATION: Influenza A H1N1 caused substantial morbidity in recipients of solid-organ transplants during the 2009-10 pandemic. Starting antiviral therapy early is associated with clinical benefit as measured by need for ICU admission and mechanical ventilation. FUNDING: None.

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.