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Le Touquet – Paris-Plage, France

Auregan C.,Service des urgences pediatriques | Berteloot L.,Service de Radiologie Pediatrique | Pierrepont S.,Service de Nephrologie Pediatrique | Cheron G.,Service des urgences pediatriques | Cheron G.,University of Paris Descartes
Archives de Pediatrie | Year: 2015

Xanthogranulomatous pyelonephritis is a rare form of chronic pyelonephritis observed in only a few cases in children. Symptoms are mild, which explains the delay in diagnosis. Diagnosis is based on histology but can be suspected on CT. The treatment is medical and often surgical, with an uncertain renal prognosis. It is therefore imperative to diagnose early. We report the case of a 4-year-old child who presented with xanthogranulomatous pyelonephritis caused by Pseudomonas aeruginosa, which evolved into pyonephrosis, due to inadequate antibiotic therapy. This highlights the importance of understanding this disease and not treating urinary tract infections blindly. © 2014 Elsevier Masson SAS. Source

Laverdure N.,Service dHepato gastroenterologie et nutrition pediatrique | Scholtes-Brunel C.,Service de Virologie | Rivet C.,Service dHepato gastroenterologie et nutrition pediatrique | Heissat S.,Service dHepato gastroenterologie et nutrition pediatrique | And 5 more authors.
Journal of Clinical Virology | Year: 2015

Background: Hepatitis E is an emerging disease in developed countries and is usually asymptomatic, particularly in children. Chronic infection is possible in immunocompromised individuals. In the context of a liver transplant, it can simulate a rejection. In this case, antiviral therapy may be considered, thus highlighting the need to diagnose hepatitis E virus (HEV) infection in this population. Objectives: Given the lack of data in France, we have studied the the prevalence of antibodies to HEV in the paediatric liver transplant population. Study design: This was a retrospective study, carried out in Lyon between 1st January 2010 and 31 May 2013. HEV serology (anti-HEV IgM &IgG) and HEV PCR were studied in 96 children who had undergone liver transplants (84 isolated liver and 12 combined liver and kidney transplants). Results: Eight patients (8.3%; 62.5% girls; mean age:12.3 years) were HEV seropositive. The mean period since their transplantation was 10 years (range:2-21.8 years). Biliary atresia was the principal indication for transplantation. Seven of these eight children had received liver transplants. There were no differences between the epidemiological and clinical data concerning these patients and the remainder of the study population, particularly with respect to immunosuppression(7/8 tacrolimus; 50% dual immunosuppression). No cases of chronic hepatitis E were found, but 1/8 had chronic cytolysis(EBV&adenovirus infection). In all the patients tested(4/8), seroconversion had occurred after the transplant. There was no significant differences between the age groups in this study. Conclusions: This study showed that in France, the prevalence of antibodies to HEV in paediatric liver and combined liver and kidney transplant patients is 8.3%, as has been found by other European authors. © 2015 Elsevier B.V. Source

Zuber J.,University of Paris Descartes | Le Quintrec M.,Service de nephrologie et transplantation renale | Morris H.,Columbia University | Fremeaux-Bacchi V.,Laboratoire dImmunologie | And 2 more authors.
Transplantation Reviews | Year: 2013

Atypical hemolytic and uremic syndrome (aHUS) is associated with a high rate of recurrence and poor outcomes after kidney transplantation. Fortunately, recent advances in the understanding of the pathogenesis of aHUS have permitted an individualized risk assessment of post-transplant recurrence. Acquired or inherited dysregulation of the alternative complement pathway, thought to be the driving force of the disease, is identified in most aHUS patients. Notably, depending on the mutations involved, the risk of recurrence greatly varies, highlighting the importance of undertaking etiological investigations prior to kidney transplantation. In those with moderate to high risk of recurrence, the use of a prophylactic therapy, consisting in either plasmapheresis or eculizumab therapies, represents a major stride forward in the prevention of aHUS recurrence after kidney transplantation. In those who experience aHUS recurrence, a growing number of observations suggest that eculizumab therapy outperforms curative plasma therapy. The optimal duration of both prophylactic and curative therapies remains an important, yet unaddressed, issue. In this respect, the kidney transplant recipients, continuously exposed to endothelial-insulting factors, referred here as to triggers, might have a sustained high risk of recurrence. A global therapeutic approach should thus attempt to reduce exposure to these triggers. © 2013 Elsevier Inc. Source

Bensman A.,University Paris Est Creteil | Niaudet P.,Service de Nephrologie Pediatrique
Pediatric Nephrology | Year: 2010

It has been reported (this issue Pediatric Nephrology) that cyclosporine A (CyA) therapy in combination with corticosteroids, angiotensin-converting enzyme inhibitor, and an angiotensin receptor blocker decreased proteinuria in three patients with nephrotic syndrome (NS) due to WT1 mutations. Treatment with calcineurin inhibitors were found to induce a partial remission of proteinuria in several other children with genetic forms of NS, such as mutation in the podocine and in the phospholipase C epsilon gene. CyA therapy has also been reported to be beneficial to patients with Alport syndrome. Recent data have shown that the antiproteinuric effect of CyA in these cases may be due to a non-immunologic mechanism. CyA exerts an antiproteinuria effect by preventing the degradation of the actin organizing protein synaptodpodin and by a downregulation of TRPC6. This mechanism leads to the stabilization of the actin cytoskeleton in the kidney podocytes. This beneficial effect of CyA is interesting, but long-term results regarding function and nephrotoxicity are still missing. © IPNA 2010. Source

Benoit G.,French Institute of Health and Medical Research | Benoit G.,University of Montreal | Machuca E.,French Institute of Health and Medical Research | Machuca E.,University of Chile | And 3 more authors.
Annals of the New York Academy of Sciences | Year: 2010

A Mendelian inheritance underlies a nonnegligible proportion of hereditary kidney diseases, suggesting that the encoded proteins are essential for maintenance of the renal function. The identification of genes involved in congenital anomalies of the kidney and in familial forms of nephrotic syndrome significantly increased our understanding of the renal development and kidney filtration barrier physiology. This review will focus on the classical phenotype and clinical heterogeneity observed in the monogenic forms of these disorders. In addition, the role of susceptibility genes in kidney diseases with a complex inheritance will also be discussed. © 2010 New York Academy of Sciences. Source

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