Dyslipidaemia in children on renal replacement therapy

Background Information on lipid abnormalities in end-stage renal disease (ESRD) mainly originates from adult patients and small paediatric studies. We describe the prevalence of dyslipidaemia, and potential determinants associated with lipid measures in a large cohort of paediatric ESRD patients. Methods In the ESPN/ERA-EDTA registry, lipid measurements were available for 976 patients aged 2-17 years from 19 different countries from the year 2000 onwards. Dyslipidaemia was defined as triglycerides >100 mg/dL (2-9 years) or >130 mg/dL (9-17 years), high-density lipoprotein (HDL) cholesterol 145 mg/dL. Missing data were supplemented using multiple imputation. Results The prevalence of dyslipidaemia was 85.1% in peritoneal dialysis (PD) patients, 76.1% in haemodialysis (HD) patients and 55.5% among renal allograft recipients. Both low and high body mass index (BMI) were associated with a less favourable lipid profile. Younger age was associated with a worse lipid profile among PD patients. HDL levels significantly improved after transplantation, whereas no significant improvements were found for triglyceride and non-HDL levels. In transplant recipients, use of cyclosporin was associated with significantly higher non-HDL and HDL levels than tacrolimus usage (P 90 mL/min/1.73 m2 (P < 0.0001). Conclusions Dyslipidaemia is common among paediatric ESRD patients in Europe. Young age and PD treatment are associated with worse lipid profiles. Although lipid levels generally improve after transplantation, dyslipidaemia may persist due to decreased graft function, high BMI or to the use of certain immunosuppressant

Outcomes of renal replacement therapy in boys with prune belly syndrome : findings from the ESPN/ERA-EDTA Registry

As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry data. Data were available for 88 male PBS patients aged <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD). Median age at onset of RRT in PBS was lower [7.0; interquartile range (IQR) 0.9-12.2 years] than in COU (9.6; IQR: 3.0-14.1 years) and RHD (9.4; IQR: 2.7-14.2 years). Unadjusted 10-year patient survival was 85% for PBS, 94% for COU, and 91% for RHD. After adjustment for country, period, and age, PBS mortality was similar to that of RHD but higher compared with COU [hazard ratio (HR) 1.96, 95% confidence interval (CI) 1.03-3.74]. Seventy-four PBS patients (84%) received a first kidney transplant after a median time on dialysis of 8.4 (IQR 0.0-21.1) months. Outcomes with respect to time on dialysis before transplantation, chance of receiving a first transplant within 2 years after commencing RRT, and death-censored, adjusted risk of graft loss were similar for all groups. This study in the largest cohort of male patients with PBS receiving RRT to date demonstrates that outcomes are comparable with other congenital anomalies of the kidney and urinary tract, except for a slightly higher mortality risk compared with patients with COU.Peer reviewe

Distinctive vasculopathy with systemic involvement due to levamisole long-term therapy: A case report

Background: Levamisole belongs to the antihelminthic class of drugs that are sometimes administered to patients with frequently relapsing or steroid-dependent nephrotic syndrome, owing to its steroid-sparing effects. Neutropenia and skin lesions, compatible with vasculitis, have been reported as drug complications, but they are rarely associated with any systemic involvement. Case presentation: We report a case of a 9-year-old Arab boy with steroid-dependent nephrotic syndrome who was treated with levamisole after his third relapse. The drug was initially well tolerated, but mild isolated neutropenia occurred 6 months after levamisole administration. This was followed by cutaneous vasculitis of both ears and the left cheek. The patient also developed hepatosplenomegaly and anemia. Levamisole was discontinued, and his disease remained in remission. All the systemic manifestations disappeared gradually over the course of 1 month. The patient remained in remission until 1 year after levamisole withdrawal, when clinical nephrosis recurred. Conclusions: Despite levamisole's being a useful drug for maintaining remission in steroid-dependent nephrotic syndrome, patients on long-term levamisole therapy should be monitored closely to prevent serious complications that can easily be resolved by simple drug withdrawal. © 2018 The Author(s)

Prediction of High-Grade Vesicoureteral Reflux after Pediatric Urinary Tract Infection: External Validation Study of Procalcitonin-Based Decision Rule

BACKGROUND: Predicting vesico-ureteral reflux (VUR) 653 at the time of the first urinary tract infection (UTI) would make it possible to restrict cystography to high-risk children. We previously derived the following clinical decision rule for that purpose: cystography should be performed in cases with ureteral dilation and a serum procalcitonin level 650.17 ng/mL, or without ureteral dilatation when the serum procalcitonin level 650.63 ng/mL. The rule yielded a 86% sensitivity with a 46% specificity. We aimed to test its reproducibility. STUDY DESIGN: A secondary analysis of prospective series of children with a first UTI. The rule was applied, and predictive ability was calculated. RESULTS: The study included 413 patients (157 boys, VUR 653 in 11%) from eight centers in five countries. The rule offered a 46% specificity (95% CI, 41-52), not different from the one in the derivation study. However, the sensitivity significantly decreased to 64% (95%CI, 50-76), leading to a difference of 20% (95%CI, 17-36). In all, 16 (34%) patients among the 47 with VUR 653 were misdiagnosed by the rule. This lack of reproducibility might result primarily from a difference between derivation and validation populations regarding inflammatory parameters (CRP, PCT); the validation set samples may have been collected earlier than for the derivation one. CONCLUSIONS: The rule built to predict VUR 653 had a stable specificity (ie. 46%), but a decreased sensitivity (ie. 64%) because of the time variability of PCT measurement. Some refinement may be warranted

Phénotype rénal associé aux mutations du gène TCF-2 (HNF-1b) dans la première cohorte pédiatrique

LIMOGES-BU Médecine pharmacie (870852108) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Effets secondaires et efficacité des immunosuppresseurs utilisés dans le traitement du syndrome néphrotique idiopathique chez l'enfant

PARIS-BIUP (751062107) / SudocSudocFranceF