Le lupus néonatal (évaluation de la prise en charge du risque cardiologique au CHU de Nantes)

Le lupus néonatal est un syndrome rare pouvant atteindre les fœtus et nouveau-nés de mères porteuses d'anticorps anti SSA/Ro et/ou SSB/La. Il peut être responsable de manifestations systémiques transitoires (atteinte cutanée, hématologique ou hépatique) ou permanentes comme le bloc auriculo-ventriculaire congénital (BAVc) survenant sur cœur sain et faisant toute la gravité de ce syndrome. Une étude rétrospective entre le 1er janvier 2000 et le 1er juin 2010 a été réalisée au CHU de Nantes portant sur l'analyse de dossiers obstétricaux de femmes à risque de lupus néonatal (femmes suivies au CHU de Nantes avec des anticorps anti SSA/Ro et/ou anti SSB/La) et ceux de leurs nouveau-nés par rapport aux propositions de suivis institués par la société Société Nationale Française de Médecine Interne (SNFMI). Notre étude a analysé 24 grossesses de 14 femmes à risque et rapporte 2 BAVc. Au cours de cette période, 5 autres BAVc ont été diagnostiqués chez 5 femmes asymptomatiques. Sur les 16 grossesses à risque de notre cohorte menées à terme, 3 suivis étaient conformes aux propositions de la SNFMI. A la naissance, un ECG a été réalisé pour 18 des 20 nouveau-nés, 14 NFS et un bilan hépatique ont été effectués. Une interruption médicale de grossesse a été réalisée devant la sévérité de l'atteinte cardiaque et une enfant est décédée au décours de la pose de pacemaker. 5 enfants ont été appareillés. Les résultats de cette étude rappellent que malgré sa rareté, le lupus néonatal est un syndrome potentiellement grave avec une morbi-mortalité importante principalement liée à l'atteinte cardiaque. La prise en charge du risque de lupus néonatal au CHU de Nantes n'est pas consensuelle et nécessite une harmonisation des pratiques multidisciplinaires, du début de la grossesse (avec un suivi intensif entre 16 et 26 SA) jusqu'aux premières années de vie de l'enfant (dépistage d'une éventuelle cardiomyopathie tardive).NANTES-BU Médecine pharmacie (441092101) / SudocSudocFranceF

Hypertension rénovasculaire pédiatrique (à propos de huit cas du CHU de Nantes)

L'hypertension artérielle concerne 1 à 3% des enfants et l'origine rénovasculaire est retrouvée dans 5 à 10% des étiologies secondaires. Les dossiers de 8 enfants atteints d'HTA rénovasculaire suivis au CHU de Nantes depuis 2001 ont été étudiés rétrospectivement : pour 2 enfants la maladie s'intégrait dans un tableau de neurofibromatose de type 1. Seulement 2 enfants étaient symptomatiques avec un syndrome hyponatrémie-hypertension lors du diagnostic alors que tous avaient une hypertension artérielle importante. Les lésions étaient bilatérales pour 3 cas avec 2 cas de syndrome mid-aortic . 1 cas présentait une lésion d'une artère sous-clavière. L'échographie doppler a orienté le diagnostic dans la moitié des cas et l'angioscanner l'a affirmé avec néanmoins quelques imprécisions. L'angioRM avait peu de rendement dans notre étude, lié au manque d'expérience de notre centre. Les prises en charge ont été variées et complexes : 1 cas a eu une néphrectomie, 2 cas d'autotransplantations rénales et 4 cas traités par angioplastie. Parmi eux, 1 enfant a nécessité jusqu'à 4 angioplasties du fait de resténoses et d'une thrombose intrastent, avec la pose d'un stent passif et d'un stent actif. En comparant nos données aux dernières séries de la littérature, nous essayons d'établir une démarche diagnostique et thérapeutique.NANTES-BU Médecine pharmacie (441092101) / SudocSudocFranceF

Syndrome néphrotique et lymphoprolifération EBV induite (efficacité d un traitement par anti-CD20)

La néphrose idiopathique est la première cause des néphropathies glomérulaires chez l enfant. La physiopathogénie de cette maladie est mal connue, faisant intervenir un dysfonctionnement du lymphocyte T, et un facteur de perméabilité de la membrane glomérulaire encore non identifié. Nous rapportons le cas d une enfant atteinte d une néphrose, dont la première poussée s est révélée au décours d une primo-infection à EBV compliquée d un syndrome lymphoprolifératif B avec la présence d une immunoglobuline monoclonale. Un traitement par anti-CD20 a permis la régression totale de l ensemble des symptômes. Cette observation soulève l hypothèse de l implication des lymphocytes B dans la néphrose, et de leur rôle potentiel dans la fabrication d un facteur circulant de perméabilité glomérulaire.NANTES-BU Médecine pharmacie (441092101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Chronic kidney insufficiency in children

Chronic kidney insufficiency (CKI) in children

Critical serum creatinine values in very preterm newborns

International audienceBackground: Renal failure in neonates is associated with an increased risk of mortality and morbidity. But critical values are not known. Objective: To define critical values for serum creatinine levels by gestational age in preterm infants, as a predictive factor for mortality and morbidity. Study Design: This was a retrospective study of all preterm infants born before 33 weeks of gestational age, hospitalized in Nantes University Hospital NICU between 2003 and 2009, with serum creatinine levels measured between postnatal days 3 to 30. Children were retrospectively randomized into either training or validation set. Critical creatinine values were defined within the training set as the 90th percentile values of highest serum creatinine (HSCr) in infants with optimal neurodevelopmental at two years of age. The relationship between these critical creatinine values and neonatal mortality, and non-optimal neural development at two years, was then assessed in the validation set. Results and Conclusion: The analysis involved a total of 1,461 infants (gestational ages of 24-27 weeks (n=322), 28-29 weeks (n=336), and 30-32 weeks (803)), and 14,721 creatinine assessments. The critical values determined in the training set (n=485) were 1.6, 1.1 and 1.0 mg/dL for each gestational age group, respectively. In the validation set (n=976), a serum creatinine level above the critical value was significantly associated with neonatal mortality (Odds ratio: 8.55 (95% confidence interval: 4.23-17.28); p<0.01) after adjusting for known renal failure risk factors, and with non-optimal neurodevelopmental outcome at two years (odds ratio: 2.06 (95% confidence interval: 1.26-3.36); p=0.004) before adjustment. Creatinine values greater than 1.6, 1.1 and 1.0 mg/dL respectively at 24-27, 28-29, 30-32 weeks of gestation were associated with mortality before and after adjustment for risk factors, and with non-optimal neurodevelopmental outcome, before adjustment

Why should the meningococcal B vaccine be recommended, and therefore reimbursed, for infants in France?

International audienc

Critical values of creatinine (indicated by black circle) and receiver operating curve for creatinine to predict mortality, according to gestational age, in validation set.

<p>Critical values of creatinine (indicated by black circle) and receiver operating curve for creatinine to predict mortality, according to gestational age, in validation set.</p

Kidney and liver transplantation in patients with autosomal recessive polycystic kidney disease: a multicentric study

International audienceBackground and objectives. In contrast to the improvement in our understanding of the pathogenesis and presentation of autosomal recessive polycystic kidney disease (ARPKD), data regarding the issue of kidney and liver transplantation in patients with ARPKD remain particularly scarce. Here, we report the results and outcome of renal and/or liver transplantation in a series of patients with ARPKD. Methods. Fourteen ARPKD patients (age: 3-25 years) who underwent renal transplantation with or without liver transplantation were retrospectively identified in five French nephrology departments. The patients' medical charts were reviewed and relevant data were collected. Results. The clinical and radiological presentation of the 14 patients was highly variable illustrating the heterogeneity of ARPKD. Six patients underwent kidney and/or liver transplantation in adulthood. First renal graft survival was 92, 78 and 14% at 1, 5 and 10 years after renal transplantation, respectively. Mortality rate was relatively high (3/14; 21%) in these young patients and was directly related to infectious complications (recurrent angiocholitis) of severe Caroli's disease (dilatation of intra- and/or extra-hepatic bile ducts), a typical feature of ARPKD. Conclusions. Our data suggest that ARPKD patients evaluated for renal transplantation should be carefully screened for severe Caroli's disease. Even though the limited number of patients included in our study precludes any definite recommendation, pre-emptive liver transplantation may be a therapeutic option in ARPKD patients with severe Caroli's disease evaluated for renal transplantation

ADJUVITE: a double-blind, randomised, placebo-controlled trial of adalimumab in early onset, chronic, juvenile idiopathic arthritis-associated anterior uveitis

International audienceOBJECTIVES: To assess the efficacy and safety of adalimumab on uveitis in patients with early onset, chronic, juvenile idiopathic arthritis (JIA)-associated or idiopathic anterior uveitis and an inadequate response to topical steroids and methotrexate (MTX). METHODS: Patients aged 4 years or more with ocular inflammation quantified by laser flare photometry (LFP) >=30 photon units/ms were double-blindly randomised (1:1) to 2 groups, one treated with placebo and one with adalimumab subcutaneously at a dose of 24 mg/m2 in patients aged \textless13 years, 40 mg in the others, every other week. The primary outcome was response at month 2 (M2) defined as a 30% reduction of inflammation on LFP in the assessable eye with more severe baseline inflammation and no worsening on slit lamp examination. From M2 to M12, all patients received adalimumab. RESULTS: At M2, among 31 patients included in intention-to-treat analysis, there were 9/16 responders on adalimumab and 3/15 on placebo (P=0.038, ?2 test; relative risk=2.81, 95% CI 0.94 to 8.45; risk difference: 36.3%, 95% CI 2.1 to 60.6); there was no significant difference using the Standardised Uveitis Nomenclature classification criteria of improvement. Thirty patients continued the trial after M2 and received adalimumab (open-label phase), 29 reached M12. There were seven serious adverse events none related to study treatment. CONCLUSIONS: This trial is in favour of using adalimumab in patients with early onset, chronic anterior uveitis, which is in most cases associated with JIA, in case of inadequate response to topical therapy and MTX. LFP could be a valuable tool to assess early treatment efficacy. TRIAL REGISTRATION NUMBER: NCT01385826

Cardiac involvement in pediatric hemolytic uremic syndrome.

International audienceBackgroundCardiac involvement is a known but rare complication of pediatric hemolytic uremic syndrome (HUS). We conducted a nationwide observational, retrospective case–control study describing factors associated with the occurrence of myocarditis among HUS patients.MethodsCases were defined as hospitalized children affected by any form of HUS with co-existent myocarditis in 8 French Pediatric Intensive Care Units (PICU) between January 2007 and December 2018. Control subjects were children, consecutively admitted with any form of HUS without coexistent myocarditis, at a single PICU in Lyon, France, during the same time period.ResultsA total of 20 cases of myocarditis were reported among 8 PICUs, with a mean age of 34.3 ± 31.9 months; 66 controls were identified. There were no differences between the two groups concerning the season and the typical, Shiga toxin-producing Escherichia coli (STEC-HUS), or atypical HUS (aHUS). Maximal leukocyte count was higher in the myocarditis group (29.1 ± 16.3G/L versus 21.0 ± 9.9G/L, p = 0.04). The median time between admission and first cardiac symptoms was of 3 days (range 0–19 days), and 4 patients displayed myocarditis at admission. The fatality rate in the myocarditis group was higher than in the control group (40.0% versus 1.5%, p < 0.001). Thirteen (65%) children from the myocarditis group received platelet transfusion compared to 19 (29%) in the control group (p = 0.03).ConclusionOur study confirms that myocarditis is potentially lethal and identifies higher leukocyte count and platelet transfusion as possible risk factors of myocarditis