Pierre Cochat

..In chronic kidney disease Stages 4 and 5, the best outcomes to date were achieved with combined liver-kidney transplantation...

..Surgery was required in two patients. Follow-up ranged from 3 to 10 years since diagnosis. In three cases antihypertensive drugs and methotrexate were stopped, and prednisone was reduced to 7.5 mg/day...

..In the future, primary hyperoxaluria type 1 may benefit from hepatocyte transplantation, chaperone molecules, etc...

..The improvement in the care of renal children therefore requires better medical knowledge, nurse training and population information...

..As glomerular filtration rate decreases due to progressive renal involvement, oxalate accumulates and results in systemic oxalosis...

..Compliance is a major issue regarding the progression of renal damage, but the overall outcome mainly depends on extra-renal involvement in relation to the metabolic defect...

..In addition, a large number of epidemiological studies and clinical trials have allowed guidelines and recommendations to be provided for chronic and end-stage renal failure, urinary tract infection, glomerular diseases, etc...

..Aggressive dialysis therapies are required to avoid progressive oxalate deposition in established end-stage renal disease (ESRD), and minimization of the time on dialysis will improve both the patient's quality of life and survival...

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..The aim of this survey was to evaluate the level of knowledge of medical students and their gaps about organ donation prior to any medical course...

..Such a treatment is safe and efficient but its biweekly intravenous administration is still uncomfortable, so that further alternative therapeutic approaches may be encouraged...

..In the medical evaluation of the donor, specific explorations, such as the search of a genetic mutation, could be necessary in the case of hereditary renal disease...

..In the near future the issue of DR after kidney Tx may benefit from alternatives to organ Tx, such as recombinant proteins, specific monoclonal antibodies, cell/gene therapy, and chaperone molecules...

..However further studies based on large series are required in order to demonstrate that renal fetal effects have limited clinical consequences...

..e. exposure to uraemic toxins, therapies, intermittent haemodynamic changes during sessions, prematurity, growth retardation)...

..The purpose of this study was to determine incidence, determinants, and progression of long-term chronic renal insufficiency (CRI) in a single-center series of pediatric liver transplant recipients...

..Our results underscore the severe prognosis of primary hyperoxaluria type 1 and the necessity for early diagnosis and treatment, as well as confirm a better prognosis of the p.Gly170Arg mutation...

..Body weight, concomitant medication, and UGT2B7 genotype contribute significantly to the interindividual variability of MMF disposition in pediatric renal-transplant patients...

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..Hyperuricemia after liver transplantation in children is a frequent problem which needs further investigation...

..In conclusion, we recommend the use of pediatric equations to estimate GFR from childhood to early adulthood...

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..Although its importance in chronic kidney disease (CKD) has been demonstrated in adults, there is little information in pediatric patients...

..Extrauterine growth restriction represents a new risk factor for long-term renal impairment in premature children...

..Early rhGH administration during CT gives better height SDS at both the end of rhGH therapy and in adulthood...

..Little is known about prognostic factors in SDNS and FRNS treated by CYP. The objectives of this study are to determine long-term outcomes and factors associated with sustained remission in these patients...

..However, previous studies pointed out renal-related anomalies which may benefit from early appropriate treatments. This study was conducted to evaluate the prevalence and severity of early renal dysfunction in ADPKD children...

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..Long-lasting MARS dialysis is feasible in children, decreases adverse effects of severe chronic cholestasis, and may help to preserve nutritional status prior to combined liver-kidney transplantation...

..Excellent long-term outcomes can be achieved in pediatric renal Tx, but specific problems such as malignancies, growth, and social outcome remain challenging...

..The aim of this review is to provide an overview of the mineral, bone and vascular abnormalities associated with CKD in children in terms of pathophysiology, diagnosis and clinical management...

..The pattern of malignancies that occur in the paediatric graft population is different from that in the general paediatric population and in the population of adult organ transplant recipients...

..We report on a patient followed for FS revealed by acute peritoneal syndrome because of ovarian dysgerminoma. Therapeutic options had led to an unusual course with recurrent neoplastic disease after renal transplantation...

..7 per year. Our study strongly suggests that early and aggressive conservative management may preserve renal function of compliant children with this disorder, thereby avoiding dialysis and postponing transplantation...

..The aim of the study was to assess mid- and long-term outcome of children with SK, with special highlight on the differential functional outcome of congenital and acquired forms of SK...

..The patient had a successful recovery without specific treatment. This case report highlights the wide spectrum of complications resulting from HHV-6 infection in immunosuppressed patients...

..Interruption of CyA treatment lead to rapid amelioration of kidney function in these children, often associated with steroid sparing, which may lead to additional benefit for growth velocity, blood pressure and physical appearance...

..Although circulating values of FGF23 are increased in early chronic kidney disease (CKD), the interplay between FGF23 levels, growth and nutritional biomarkers has not been evaluated in children with normal renal function...

..The objectives of this study were to describe the clinical and genetic features of familial hypomagnesemia with hypercalciuria and nephrocalcinosis and analyze phenotype-genotype associations in patients with CLDN16 or CLDN19 mutations...

..Thus, cysteamine decreases and delays the onset of complications and improves life expectancy in cystinosis. Hence, cysteamine therapy should be introduced as early as possible during childhood and maintained lifelong...

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..The aim of this study was to investigate the influence of age at disease onset in the outcome of paediatric SLE (pSLE)...

..Normal growth is a major objective of the management of transplanted children. The mean final height increased during the 20 last years to be between -1.63 and -0.92 SDS depending on age and period of the transplantation...

..Novel bone imaging techniques seem feasible in children, and further longitudinal studies are required to thoroughly explore long-term cardiovascular and bone consequences of phosphate-calcium metabolism deregulation during CKD...

..Further prospective controlled trials will delineate the potential benefit of new immunosuppressive treatment...

..We can hypothesize that inulin had activated the innate immune system. Inulin may, thus, have been the initiating event of the renal relapse, acting like an infection, in a patient with IgA-mediated immunological dysregulation...

..Nine years later, he had no sign of PAN, normal blood pressure and normal renal function in the absence of any treatment...

..5 for all patients). We reevaluated the coefficient of the 2009-Schwartz formula according to sex and age in a pediatric population...

..Monitoring of plasma testosterone in patients receiving unlicensed paediatric drugs for managing hyperparathyroidism and presenting with a change in genitals is therefore recommended...

..Patients who do not benefit from the TA can be classified as very probably steroid-dependent. TA seems to be a useful therapeutic strategy in those patients for whom noncompliance is strongly suspected...

..Nephrotoxicity should be considered when there is any unexplained renal impairment, especially in children...

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..We show that NPHP3 mutations in both infantile and adolescent nephronophthisis point to a common pathophysiological mechanism despite their different clinical presentations...

..Quantitative PCR of AGXT exons 1 and 3 on the patient's and parents genomic DNA revealed the presence of two copies of the gene. This is the first case of PH1 caused by segmental maternal isodisomy of 2q37.3...

..Autosomal recessive and dominant hereditary forms are caused by Epithelial Na Channel and Mineralocorticoid Receptor mutation respectively, while secondary PHA1 is usually associated with urological problems...

..In end-stage renal disease patients, the best outcomes have been achieved with combined liver-kidney transplantation which corrects the enzyme defect...

..The parents' involvement in the decision-making process differed between centres. CONCLUSIONS: The criteria used to decide whether to withhold or to withdraw LST are not standardized, and no specific guidelines exist...

..Median glomerular filtration rate at three months, one, five, and 10 yr was 90.8, 106, 87.8, and 66.1 mL/1.73 m(2) /min. We believe that en bloc transplantation may be an option for children with end-stage kidney disease...

..Hence, an isolated increase in the serum alkaline phosphatase level following transplantation should not be of concern in this population of patients...

..More generally, they also discuss the available technical options based on a review of the literature...

..E. coli 0157:H7 and SLT 2 were detected in the stool. SLT 2 was also found in the mother's stool. This is the first report of mother-to-child transmission of SLT-producing E. coli...

..However, a fortuitous association between these two conditions is also possible...

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..Our aim was to study the frequency, clinical characteristics and outcome of each genetic subtype...

..Although several studies have been devoted to adult transplant patients, few data are available on the long-term skin malignancies following pediatric organ transplantation...

..These were all negative for protein. The mother, who was a nurse, finally confessed to adding human albumin to the urine samples...

..Thus, epidemiologic and mechanistic studies are needed to determine the true prevalence of paraneoplastic glomerulopathies and investigate new pathophysiologic approaches...

..This case emphasizes the need for activity scores to identify patients at risk for progressive systemic vasculitis requiring early and long-term aggressive immunosuppressive therapy...

..Interestingly, the absolute GFR of children receiving pediatric grafts increased along with body growth, leading to a stable relative GFR up to 6 years post-Tx...

..CONCLUSION: Silicate-containing milk thickeners can be responsible for urinary calculi and/or nephrocalcinosis...

..Twenty-one demands originated from the parents. Over a 4-year period the attendance of a dental specialist resulted in a significant improvement in the oral health of the renal transplant children...

..As long as corticosteroids are believed to be essential after renal Tx, rhGH should be considered to optimize longitudinal growth in children...

..The second paper deals exclusively with the interviews with doctors and analyses their lifetime's experience and perception...

..The present publication reviews the arguments based on which this decision was taken...

..The variability in attitudes is most probably due to the absence of clear guidelines based on prospective and controlled trials...

..Combined liver and kidney transplantation (LKT) corrects the underlying metabolic defect and restores renal function in these children...

..These preliminary data suggest that basiliximab, given in combination with cyclosporine and corticosteroids, is an effective immunosuppressive regimen for the prevention of acute rejection in pediatric renal transplantation...

..The first dose is given before surgery and the second on day 4 after transplantation...

..Mutations in UMOD may critically affect the function of uromodulin, resulting in abnormal accumulation within tubular cells and reduced urinary excretion...

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..Of high interest is the impact of genetic polymorphism on the development of renal dysfunction...

..The data also demonstrate that mutations in either of these genes may cause early deafness, and they highlight the importance of genetic screening for recessive forms of dRTA independent of hearing status...