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Le Touquet – Paris-Plage, France

Ackermann O.,Hepatologie Pediatrique | Ackermann O.,University Paris - Sud | Branchereau S.,Chirurgie pediatrique | Franchi-Abella S.,Radiologie Pediatrique | And 12 more authors.
American Journal of Transplantation | Year: 2012

Hepatic artery thrombosis (HAT), one of the most severe complications of pediatric orthotopic liver transplantation (OLT), often compromises graft and/or child survival. Of 590 OLT performed in 516 children over a 20-year period, 45 were complicated by early HAT, during the first 2 weeks after transplantation. Systematic Doppler ultrasonographic detection of HAT allowed successful surgical revascularization in 19 instances, resulting in a 20-year graft survival rate of 77% versus 24% of cases when revascularization was not attempted or failed. A combination of surgical emergency revascularization, biliary interventional radiology, biliary surgery and/or retransplantation resulted in an 80% 20-year patient survival rate, identical to that of transplanted children who did not experience early HAT. The majority of long-term survivors with their initial graft had normal liver tests, no biliary dilation on ultrasonography and minimal or moderate fibrosis on liver histology. A failed attempt at revascularization did not significantly alter patient survival. Despite these encouraging results, for the children and their parents to overcome the entire process in terms of reoperations, repeated radiological interventions, number of hospitalizations and emotional stress, remains an ordeal of such magnitude that it justifies renewed efforts to progress in the prevention of this complication. Early detection by systematic ultrasonography, urgent attempt at surgical revascularization and assiduous care for biliary complications allow a long-term survival identical to that of other transplanted children and the salvage of close to one-third of the original grafts. © Copyright 2012 The American Society of Transplantation and the American Society of Transplant Surgeons. Source

Cremer R.,Lille University Hospital Center | Cremer R.,Lille University of Science and Technology | Hubert P.,Reanimation Pediatrique | Hubert P.,University of Paris Descartes | And 6 more authors.
Intensive Care Medicine | Year: 2011

Purpose: Our goal is to assess the prevalence of questioning about the appropriateness of initiating or maintaining life-sustaining treatments (LST) in French-speaking paediatric intensive care units (PICUs) and to evaluate time utilisation related to decision-making processes (DMP). Methods: 18-month, multicentre, prospective, descriptive, observational study in 15 Frenchspeaking PICUs. Results: Among the 5,602 children admitted, 410 died (7.3%), including 175 after forgoing LST (42.7% of deaths). LST was questioned in 308 children (5.5%) with a prevalence of 13.3 per 100 patient-days. More than 30% of children survived despite the appropriateness of LST being questioned (23% despite a decision to forgo treatment). Median caregiver time spent on making and presenting the decisions was 11 h per child. Conclusions: In this study, on any given day in each 10-bed PICU, there was more than one child for whom a DMP was underway. Of children, 23% survived despite a decision to forgo LST being made, which underlines the need to elaborate a care plan for these children. Also, DMP represented a large amount of staff time that is undervalued but necessary to ensure optimal palliative practice in PICU. © jointly held by Springer and ESICM 2011. Source

Cremer R.,Lille University Hospital Center | Cremer R.,Lille University of Science and Technology | Binoche A.,Lille University Hospital Center | Le Reun C.,Lille University Hospital Center | Hubert P.,Reanimation Pediatrique
Reanimation | Year: 2014

Children survival after a decision of treatment limitation (DTL) in the paediatric intensive care unit (PICU) is a recent concern. Our objectives were to present data on the outcome of children who survived despite DTL, and review how information about DTL is transmitted between PICUs and the teams in charge of the children after PICU discharge. Interestingly, more than 20% of the children survive despite DTL; however, all these children remain dependent on their parents for all activities of daily life. Half of them would be referred to PICU if presenting any severe illness. Additionally, despite significant progress in the transmission of information regarding DLT by PICUs, improvement is still expected to better involve the teams in charge after PICU discharge. © 2013 Société de réanimation de langue française (SRLF) and Springer-Verlag France. Source

Boutron A.,Hopitaux universitaires Paris Sud | Labarthe F.,Pediatrie metabolique | Lamireau D.,Reanimation Pediatrique | de Villemeur T.B.,Neuropediatrie | And 3 more authors.
Molecular Genetics and Metabolism | Year: 2011

Background: Deficiency of mitochondrial trifunctional protein (MTP) is caused by mutations in the HADHA and HADHB genes, which have been mostly delineated at the genomic DNA level and have not been always elucidated. Aim: To identify mutations in a French cohort of 52 MTP deficient patients and the susceptibility of mutations generating premature termination codons (PTCs) to the nonsense mRNA mediated decay (NMD). Methods: Mutation screening in fibroblasts was performed at the cDNA level and real-time RT-PCR was used to compare the levels of the different PTC-bearing mRNAs before and after a treatment of fibroblasts by emetine, a translation inhibitor. Results: A mutation detection rate of 100% was achieved. A total of 22 novel mutations were identified, including a large-sized genomic deletion in HADHB gene. A high proportion of all identified mutations were non-sense, frameshift and splicing mutations, generating (PTCs), distributed essentially on HADHA coding regions. We could demonstrate that the majority of mutations resulting in PTCs conform to the established rules governing the susceptibility to NMD. Conclusion: Our results emphasize the value of cDNA analysis in the characterization of HADHA and HADHB mutations and further strengthen the model of haploinsufficiency as a major pathomechanism in MTP defects. © 2011 Elsevier Inc. Source

Burin Des Roziers N.,Etablissement Francais du Sang Ile de France | Chadebech P.,Etablissement Francais du Sang Ile de France | Chadebech P.,French Institute of Health and Medical Research | Bodivit G.,Etablissement Francais du Sang Ile de France | And 11 more authors.
Transfusion | Year: 2015

Background Pneumococcal hemolytic uremic syndrome (P-HUS) is a rare but severe complication of invasive pneumococcal disease (IPD) in young children. Consensual biologic diagnosis criteria are currently lacking. Study Design and Methods A prospective study was conducted on 10 children with culture-confirmed IPD. Five presented with full-blown P-HUS, three had an incomplete form with hemolytic anemia and mild or no uremia (P-HA), and two had neither HUS nor HA. Thomsen-Friedenreich (T), Th, and Tk cryptantigens and sialic acid expression were determined on red blood cells (RBCs) with peanut (PNA), Glycine soja (SBA), Bandeiraea simplicifolia II, and Maackia amurensis lectins. Plasma concentrations of the major endogenous T-antigen-binding protein, galectin-3 (Gal-3), were analyzed. Results We found that RBCs strongly reacted with PNA and SBA lectins in all P-HUS and P-HA patients. Three P-HUS and three P-HA patients showed also concomitant Tk activation. Direct antiglobulin test (DAT) was positive in three P-HUS (one with anti-C3d and two with anti-IgG) and two P-HA patients (one with anti-C3d and one with anti-IgG). RBCs derived from the two uncomplicated IPD patients reacted with PNA but not with SBA lectin. Gal-3 plasma concentrations were increased in all P-HUS patients. Conclusions The results indicate high levels of neuraminidase activity and desialylation in both P-HUS and P-HA patients. T-antigen activation is more sensitive than DAT for P-HUS diagnosis. Combining PNA and SBA lectins is needed to improve the specificity of T-antigen activation. High concentrations of Gal-3 in P-HUS patients suggest that Gal-3 may contribute to the pathogenesis of P-HUS. © 2014 AABB. Source

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