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Introduction Kidney transplantation (KT) is the treatment of choice for end-stage renal disease. Pre-emptive KT is considered to be the most optimal treatment of ESRD particularly in children but reports on the results of paediatric pre-emptive KT are scarce. The objective of this study was to evaluate the impact of pre-emptive KT on the risk of graft failure in children with ESRD. Methods We analyzed all first kidney transplants performed in children Results A total of 1920 paediatric patients were included, of whom 387 (20.2%) received a pre-emptive KT. Median time of follow-up was 7.0 years [IQR (3.0–11.7)]. At 10 years post-transplant, graft survival was 85.2% in pre-emptive KT and 67.1% in non-pre-emptive KT (P Conclusion In France, pre-emptive KT in children is associated with a lower risk of graft failure than KT performed after the initiation of dialysis, and should be encouraged when feasible.

La cystinurie est une maladie renale autosomique recessive secondaire a un trouble de la reabsorption tubulaire des acides amines dibasiques. Elle se manifeste chez les patients homozygotes par des lithiases renales, responsables de coliques nephretiques, entrainant a long terme des insuffisances renales. Nous avons suivi, au sein du service de gynecologie-obstetrique de l’hopital de La Conception et du centre de diagnostic prenatal de l’hopital de La Timone, une patiente qui presentait a l’echographie obstetricale du troisieme trimestre un colon hyperechogene. L’enquete genetique a confirme le diagnostic de cystinurie chez le foetus et chez les autres membres de la fratrie. Le diagnostic precoce de cystinurie permet, par des recommandations simples (hydratation et alcalinisation), de prevenir les complications de cystinurie.

The aim of this study was to develop a population pharmacokinetic model of tacrolimus in pediatric kidney transplant patients, identify factors that explain variability, and determine dosage regimens. Pharmacokinetic samples were collected from 50 de novo pediatric kidney transplant patients (age 2-18 years) who were on tacrolimus treatment. Population pharmacokinetic analysis of tacrolimus was performed using NONMEM, and the impact of variables (demographic and clinical factors, and CYP3A4-A5, ABCB1, and ABCC2 polymorphisms) was tested. The pharmacokinetic data were described by a two-compartment model that incorporated first-order absorption and lag time. The apparent oral clearance (CL/F) was significantly related to body weight (allometric scaling); in addition, it was higher in patients with low hematocrit levels and lower in patients with CYP3A5*3/*3. The population pharmacokinetic-pharmacogenetic model developed in de novo pediatric kidney transplant patients demonstrated that, in children, tacrolimus dosage should be based on weight, hematocrit levels, and CYP3A5 polymorphism. Individualization of therapy will enable the optimization of tacrolimus exposure, with resultant beneficial effects on kidney function in the initial post-transplantation period.

Objective We report a case of laparoscopic nephrectomy of a cross-fused ectopic kidney in a 4-year-old girl with renal hypertension and Fanconi anemia. Materials and methods We performed a transperitoneal laparoscopy. Ectopic kidney resection was done after dissection of the pathological kidney and after clamping vessels, using an ultrasonic device. Hospitalization time was 4 days. Results At 6 months, blood pressure was normalized and the patient showed an adequate growth curve. Conclusion The transperitoneal route is very effective when a nephrectomy is necessary. It offers perfect exposure with limited risk of complications.

BACKGROUND Little is known about the socioprofessional situation of adult-aged kidney-transplanted children. This nationwide French cohort study documented the socioprofessional outcomes of adults who underwent kidney transplantation before age 16 years between 1985 and 2002. METHODS Of 890 patients, 624 were eligible for a questionnaire and 374 completed it (response rate=60%; men=193 and women=181). The data were compared with the French general population using an indirect standardization matched for gender, age, and period. RESULTS The median ages were 27.1 years at survey time and 12.3 years at first transplantation. Of the participants, 31.1% lived with a partner (vs. 52.2%; P

Hyper-IgD and periodic fever syndrome (HIDS) is a hereditary autoinflammatory syndrome, characterized by recurrent inflammatory attacks. Treatment of HIDS is difficult. Recently, the IL-1ra analogue anakinra was reported to be successful in aborting the IgD inflammatory attacks in a vaccination model. We report a clinical case of spectacular reduction of febrile attacks in a severe HIDS patient.