Meeting Report: Consensus Recommendations for a Research Agenda in Exercise in Solid Organ Transplantation

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/108597/1/ajt12874.pd

Child and parental perspectives on communication and decision-making in pediatric chronic kidney disease: a focus group study

Background & Objectives: Effective communication and shared decision making improve quality of care and patient outcomes but can be particularly challenging in pediatric chronic disease because children depend on their parents and clinicians to manage complex health care and developmental needs. We aimed to describe the perspectives of children with chronic kidney disease (CKD) and their parents with regard to communication and decision making. / Study Design: Qualitative study. / Setting & Participants: Children with CKD (n = 34) and parents (n = 62) from 6 centers across 6 cities in Australia, Canada, and the United States participated in 16 focus groups. / Analytical Approach: Transcripts were analyzed thematically. / Results: We identified 4 themes: (1) disempowered by knowledge imbalance (unprepared and ill-informed, suspicion of censorship, and inadequacy as technicians), (2) recognizing own expertise (intuition and instinct unique to parental bond, emerging wisdom and confidence, identifying opportunities for control and inclusion, and empowering participation in children), (3) striving to assert own priorities (negotiating broader life impacts, choosing to defer decisional burden, overprotected and overruled, and struggling to voice own preferences), and (4) managing child’s involvement (respecting child’s expertise, attributing “risky” behaviors to rebellion, and protecting children from illness burden). / Limitations: Only English-speaking participants were recruited, which may limit the transferability of the findings. We collected data from child and parent perspectives; however, clinician perspectives may provide further understanding of the difficulties of communication and decision making in pediatrics. / Conclusions: Parents value partnership with clinicians and consider long-term and quality-of-life implications of their child’s illness. Children with CKD want more involvement in treatment decision making but are limited by vulnerability, fear, and uncertainty. There is a need to support the child to better enable him or her to become a partner in decision making and prepare him or her for adulthood. Collaborative and informed decision making that addresses the priorities and concerns of both children and parents is needed

Depressive Symptoms in Children with Chronic Kidney Disease

To assess depression in children with chronic kidney disease (CKD) and to determine associations with patient characteristics, intellectual and educational levels, and health related quality of life (HRQoL)

Genetic diagnosis of X-linked dominant hypophosphatemic rickets in a cohort study: Tubular reabsorption of phosphate and 1,25(OH)2D serum levels are associated with PHEX mutation type

<p>Abstract</p> <p>Background</p> <p>Genetic Hypophosphatemic Rickets (HR) is a group of diseases characterized by renal phosphate wasting with inappropriately low or normal 1,25-dihydroxyvitamin D₃(1,25(OH)₂D) serum levels. The most common form of HR is X-linked dominant HR (XLHR) which is caused by inactivating mutations in the <it>PHEX </it>gene. The purpose of this study was to perform genetic diagnosis in a cohort of patients with clinical diagnosis of HR, to perform genotype-phenotype correlations of those patients and to compare our data with other HR cohort studies.</p> <p>Methods</p> <p>Forty three affected individuals from 36 non related families were analyzed. For the genetic analysis, the <it>PHEX </it>gene was sequenced in all of the patients and in 13 cases the study was complemented by mRNA sequencing and Multiple Ligation Probe Assay. For the genotype-phenotype correlation study, the clinical and biochemical phenotype of the patients was compared with the type of mutation, which was grouped into clearly deleterious or likely causative, using the Mann-Whitney and Fisher's exact test.</p> <p>Results</p> <p>Mutations in the <it>PHEX </it>gene were identified in all the patients thus confirming an XLHR. Thirty four different mutations were found distributed throughout the gene with higher density at the 3' end. The majority of the mutations were novel (69.4%), most of them resulted in a truncated PHEX protein (83.3%) and were family specific (88.9%). Tubular reabsorption of phosphate (TRP) and 1,25(OH)₂D serum levels were significantly lower in patients carrying clearly deleterious mutations than in patients carrying likely causative ones (61.39 ± 19.76 vs. 80.14 ± 8.80%, p = 0.028 and 40.93 ± 30.73 vs. 78.46 ± 36.27 pg/ml, p = 0.013).</p> <p>Conclusions</p> <p><it>PHEX </it>gene mutations were found in all the HR cases analyzed, which was in contrast with other cohort studies. Patients with clearly deleterious <it>PHEX </it>mutations had lower TRP and 1,25(OH)₂D levels suggesting that the <it>PHEX </it>type of mutation might predict the XLHR phenotype severity.</p

Risks factors for developing post-transplant diabetes after pediatric kidney transplant in a Canadian tertiary care children’s hospital between 1995-2016

Background Post-transplant diabetes mellitus (PTDM) is a serious complication in kidney transplant recipients (KTR) due to its negative impact on graft and patient survival. Although reported in 3-20% of pediatric KTR, it is not characterized as well as in adults. This study describes the incidence and risk factors associated with the development of PTDM in pediatric KTR. Methodology Retrospective cohort study of non-diabetic pediatric patients, aged 6 months to 19 years, who underwent a first kidney transplant during 1995-2016. We estimated the cumulative incidence rate and used multivariable logistic regression to identify the diabetogenic risk factors for PTDM. Results A total of 142 KTR were included in this study. The cumulative incidence of PTDM was 31% and 14.1% in the first, and second year post-transplant, respectively. Significant risk factors for PTDM in the first-year post-transplant included: dysglycemia in the first 8-30 days post-transplant (adjusted odds ratio [aOR]: 3.02, 95% CI:1.21-7.53; p=0.018) and use of sirolimus in the first 30 days post-transplant (aOR: 5.33, 95% CI: 1.16-24.35; p=0.031). No significant association was found with typical diabetogenic factors. Conclusion The incidence of PTDM is high among pediatric KTR. Independent risk factors associated with PTDM, included meeting criteria for dysglycemia or diabetes and sirolimus use in the first month post-transplant. Typical diabetogenic risk factors for type 2 diabetes were not associated with an increased risk. This study provides important information for post-transplant medical care and future research