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L'effet des antipaludeens de synthese sur les infections virales est etudie depuis plusieurs annees, y compris l'hypothese d'un effet sur le SARS [1]. L'efficacite in vitro de la chloroquine contre le SARS-CoV-2 a ete decrite [2] , ainsi que la superiorite potentielle de l'hydroxycholoroquine. De nombreuses etudes in vivo ont ete menees. Il existe peu de donnees sur l'impact d'un traitement au long cours par hydroxychloroquine (HCQ) sur l'infection a SARS CoV-2 et les tests diagnostiques. Nous avons pour cela analyse les donnees des patients atteints de COVID-19 et suivis pour un rhumatisme inflammatoire chronique et/ou une maladie auto-immune systemique (iRMD-COVID-19) selon la prise ou non d'hydroxychloroquine comme DMARD. Les patients ont ete inclus a partir de la cohorte francaise iRMD-COVID-19 [3]. Les donnees cliniques, diagnostiques et d'evolution de l'infection a SARS CoV-2 des patients traites au prealable par HCQ ont ete comparees a celle des patients n'ayant pas de traitement par HCQ au moment de l'infection. Les criteres d'appariement etaient l'âge, le sexe, les comorbidites, un traitement immunosuppresseur, et l'utilisation de la PCR nasale. Parmi les 871 patients, 82 patients etaient traites par HCQ. Soixante et onze cas traites par HCQ ont pu etre apparies et compares a 191 temoins. Le taux de PCR nasale positive etait de 85 % dans le groupe HCQ contre 81 % dans le groupe controle (absolute standardized difference = 6,0 %). Il n'y avait pas de difference significative entre les cas et les controles concernant les signes cliniques, le taux d'hospitalisation (33,8 % vs 27,7 % ;OR = 1,75 (0,86-3,56) ;p = 0,12), le taux d'admission en soins intensifs (11,3 % vs 9,4 % ;OR= 1,94 (0,69-5,41) ;p = 0,21) et le taux de deces (5,9 % vs 6,6 % ;OR = 1,10 (0,30-4,04) ;p = 0,89). Chez les patients atteints de COVID-19 et suivis pour un rhumatisme inflammatoire chronique et/ou une maladie auto-immune systemique de la cohorte French RMD Covid 19, la prise d'HCQ comme DMARD n'a pas modifie le taux de positivite de la PCR nasale, n'a pas modifie la presentation clinique et n'a pas prevenu la survenue de forme severe de l'infection a SARS CoV-2 comparativement aux patients sans HCQ. (French) [ABSTRACT FROM AUTHOR] Copyright of Revue de Medecine Interne is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Resume Le rotavirus est la cause la plus frequente de gastro-enterite (GEA) necessitant une hospitalisation chez l’enfant. C’est un virus tres resistant et contagieux a l’origine de nombreuses GEA nosocomiales (GEAN). En France, le vaccin contre le rotavirus est disponible depuis 2006, mais il n’est pas recommande. Le but de cette etude retrospective etait de decrire les GEAN a rotavirus et d’evaluer leur incidence chez les enfants hospitalises dans le service de pediatrie generale de l’hopital Robert-Debre (Paris) entre le 1 er janvier 2009 et le 31 decembre 2013. Nous avons evalue si ces enfants auraient pu beneficier de la vaccination contre le rotavirus. Resultats Au total, 136 enfants ont presente une GEAN a rotavirus, soit une incidence de 2,5 cas pour 1000 jours d’hospitalisation. Cette incidence est restee stable entre 2009 et 2013 malgre le renforcement des mesures d’hygiene. L’âge moyen des enfants etait de 7 mois (entre 0,5 et 111 mois). Les GEAN a rotavirus etaient survenues chez des enfants hospitalises le plus souvent pour une pathologie respiratoire aigue (65 %) et necessitant une hospitalisation prolongee (mediane = 18 jours). Un quart des enfants etaient nes prematures (25 %). L’hydratation a ete orale pour 80 enfants (59 %), par perfusion intraveineuse pour 18 (13 %) et par voie intra-osseuse pour 1 enfant. La moitie des patients etait âgee de moins de 5 mois et aurait pu beneficier de la protection fournie par la vaccination. Conclusion Les GEAN rotavirus sont frequentes. La vaccination des nourrissons contre le rotavirus permettrait de diminuer les hospitalisations pour gastro-enterite communautaire a rotavirus et, indirectement, de proteger les enfants porteurs de pathologies sous-jacentes trop jeunes pour etre vaccines.

Background The occurrence of a TMA during SLE is a rare event that complicates 1% to 4% of lupus. The full spectrum of TMA can be encountered in the course of SLE: acquired thrombotic thrombocytopenic purpura (PTT), TMA associated with glomerulonephritis (TMA GN), atypical hemolytic uremic syndrome (aHUS), microangiopathic antiphospholipid syndrome (MAPS; defined or probable catastrophic antiphospholipid syndrome) or HELLP syndrome during pregnancy. Objectives To describe the clinical phenotype of TMA in lupus, to specify the prognosis and to identify factors allowing early classification and management of the different subtypes. Methods We performed a French multicentric retrospective study from January 1987 to December 2017 in units of internal medicine, nephrology and ICU. Inclusion criteria associated SLE defined by the ACR and/or SLICC criteria and a TMA defined either by the presence of microangiopathic anaemia and peripheral thrombocytopenia, either by histological signs of TMA. Results 68 events of TMA occurred in 60 SLE patients; median age was 24 years; 56 women (F/M ratio: 14). Diagnosis of SLE was assessed at a paediatric age in 24 (35.8%) patients. Clinical manifestations of SLE were: acute cutaneous lupus (n=48, 71%), arthritis (n=36, 53%), pleuritis (n=13, 19%), pericarditis (n=25, 37%), renal involvement (n=39, 57% including 20 class IV glomerulonephritis) and auto-immune cytopenia (n=22, 32%). Triggering factors of TMA were: lupus flare (n=38), infections (n=12), pregnancy and peripartum (n=3), or therapeutic rupture (n=4). Clinical and biological features at diagnosis of each subtypes of TMA are presented in table 1. A platelet count lower than 28 000/mm3 was predictive of TTP diagnosis (Se=90,5%, Sp=88,5%), likewise a creatinine plasma level greater than 100 µmol/L was predictive of TMA GN (Se=88,5%, Sp=85%). The treatment of TMA included (alone or in combination): corticosteroids in all cases, plasma exchange (n=52), cyclophosphamide (n=28), Rituximab (n=15), Eculizumab (n=2), antiplatelet agent (n=36), and/or effective anticoagulation (n=26). The median duration of follow-up was 150 months. Among TMA GN patients, the final median GFR was 58 mL/min (range: 0–120) with 5 individuals on chronic dialysis and 1 kidney transplant. Among TTP patients, one died from TMA, whereas the final median GFR of survivors (n=59) was 97 mL/min (range: 64–150), without any patient requiring dialysis. Table 1 Comparisons of clinical and laboratory data in different subtypes of TMA in SLE Conclusions SLE-associated TMA is a heterogeneous syndrome. TTP with a decreased ADAMTS13 activity and a low platelets level ( 100 µmol/L) have a poor renal prognosis. Early subtypes classification is mandatory for the clinician to provide prompt and appropriate management of this life-threatening complication. Disclosure of Interest None declared

La Presse Medicale - In Press.Proof corrected by the author Available online since jeudi 5 fevrier 2015

BackgroundMycophenolate mofetil (MMF) is increasingly used in children with systemic lupus erythematosus (SLE). Monitoring of its active metabolite, mycophenolic acid (MPA), is performed for individual dosage adjustment of MMF and is based on determination of area under the plasma concentration-time curve (AUC12h) of MPA. The objective of this monocentric study is to describe the pharmacokinetics (PK) of MPA in paediatric patients with SLE or lupus nephritis.MethodsPatients with lupus treated by MMF between January 2009 and July 2018 were included. MPA plasma concentrations (T0, T0.5h, T1h, T2h, T3h, T8h and T12h) were determined by EMIT (enzyme-multiplied immunotechnique) and MPA AUC12h calculated according to trapezoid rule.ResultsTwenty-two patients were diagnosed with lupus at 11.5 ± 2.9 years. Clinical presentation was SLE (n=18) or isolated lupus nephritis (n=4). Treatments prior to MMF were steroids and/or immunosuppressants (endoxan, rituximab). Age at initiation of MMF (Cellcept®n=20, Myfortic®n=2) was 12.9 ± 2.6 years.PK of MPA was performed after 8.2 ± 14.8 months of treatment, under MMF dose of 840 ± 218 mg (577 ± 98 mg/m2). A large interindividual variability in MPA concentration-time profiles was observed with the following mean parameters: Cmax=4.60 ± 11.70 µg/mL, tmax=80 ± 77 min, trough plasma concentration (C0) = 2.30 ± 1.60 µg/mL and AUC0-12h=46.29 ± 17.39 µg*h/mL.ConclusionData on the PK of MPA in lupus children are limited. Our results show the high interindividual variability in MPA exposure after oral administration of MMF. Monitoring of exposure based on AUC in combination with immunological disease parameters will allow to individualise treatment to optimiseDisclosure(s)Nothing to disclose

A case of fetal congenital cardiac diverticulum, diagnosed at 36 weeks of gestation. The fetus presented with a large cardiac effusion one day before his birth, and an active pericardial bleeding with tamponade and severe bradycardia right before the urgent C-section. Drainage of the effusion done right after the delivery, with good neonatal outcome.

We hereby report the third case of antenatal diagnosis of congenital cyst of the pancreas. It is a very rare lesion and could present as a differential diagnosis of any intra-abdominal cystic mass. Early treatment is easy and prevents complications such as ascites and pancreatitis in case of intra-pancreatic enteric duplication.

Summary: Purpose: To evaluate the efficacy of vigabatrin (VGB) in the treatment of infantile spasms (ISs) associated with Down syndrome (DS) and to assess the feasibility of early discontinuation to reduce the possible retinal toxicity. Methods: Five children with ISs with DS were treated with vigabatrin as first-line monotherapy in an open prospective study. The short-term response was evaluated, and VGB was continued in responders. The treatment was stopped after 6 months in children who were still spasm free. Results: Four children of five became spasm free with VGB, three of them responding within 1 week. This response was maintained during the 6 months of VGB treatment. After VGB discontinuation, and with a follow-up ranging from 2 to 4 years, none of the responders experienced spasm recurrence or other types of seizures. Conclusions: This study confirms the efficacy of VGB in ISs associated with DS. Moreover, it shows that the duration of VGB treatment can be reduced to 6 months without relapse of ISs. This short treatment might reduce the risk of developing visual field constriction.

In right congenital diaphragmatic hernia (RCDH), several clinical diagnostic pitfalls are possible and should be known to those caring for infants and children with this disorder. The records of the 18 patients at Hotel Dieu de France Hospital with a history of CDH between 1990 and 1999 were collected; those of the ten who had a RCDH were reviewed retrospectively. The mean age at diagnosis was 6 months; the male-to-female ratio was 2:3. The delay between the first symptom and the diagnosis ranged between 0 and 10.5 months (mean 4.5 months). An acute presentation was observed in four cases, consisting of respiratory distress in three; the 4th presented with gastric volvulus and intestinal obstruction. The presenting symptoms were mild in four cases; recurrent respiratory infections in three and failure to thrive in one. The diagnosis was incidental in two cases during the evaluation of respiratory symptoms attributed to an atrial septal defect. The radiologic findings provided by a chest radiograph (CxR) were sufficient to make an accurate diagnosis in eight cases and peritoneography was useful in one. In six cases, the presenting CxR had been misinterpreted as normal or acute lobar pneumonia. Pathologic findings at surgery consisted of lateral and posterior right diaphragmatic defects in nine cases; the defect was lateral and anterior in one. A hernia sac was found in seven cases; malrotation was present in three. Surgical correction was done by an abdominal approach in nine cases and a thoracic approach in one. The diaphragmatic defect was repaired by transverse closure in six cases, diaphragm plication in three and prosthetic closure in one. The postoperative outcome was uneventful in eight cases. Two patients died. Thus, RCDH seems to cause less severe symptoms than left-sided LCDH. It usually manifests beyond the neonatal period as respiratory or gastrointestinal symptoms. The diagnosis should be made easily by a CxR. The presence of a hernia sac correlated with a mild presentation. An abdominal surgical approach is preferred.

We hereby report the second case of antenatally diagnosed "neurenteric cyst" in the literature. The patient we describe presented as a neonatal respiratory distress syndrome (NRDS) which did not respond to a vigorous resuscitation. Anomaly of the antenatal ultrasonography and of the initial chest x-ray added to the non-response to therapy, led us to an urgent thoracic scan and to a lifesaving thoracotomy. We think that every posterior mediastinal cystic mass, with or without vertebral malformations, diagnosed antenatally, should raise the possibility of neurenteric cyst. The perinatal management of the newborn to be, will thus be simplified. This will lead to a better outcome.

Though conventional coronary angiography (CCA) has been the standard of reference for diagnosing coronary artery disease in the past decades, computed tomography angiography (CIA) has rapidly emerged, and is nowadays widely used in clinical practice. Here, we introduce a standardized evaluation framework to reliably evaluate and compare the performance of the algorithms devised to detect and quantify the coronary artery stenoses, and to segment the coronary artery lumen in CIA data. The objective of this evaluation framework is to demonstrate the feasibility of dedicated algorithms to: (I) (semi-)automatically detect and quantify stenosis on CIA, in comparison with quantitative coronary angiography (QCA) and CIA consensus reading, and (2) (semi-)automatically segment the coronary lumen on CIA, in comparison with expert's manual annotation. A database consisting of 48 multicenter multivendor cardiac CIA datasets with corresponding reference standards are described and made available. The algorithms from 11 research groups were quantitatively evaluated and compared. The results show that (1) some of the current stenosis detection/quantification algorithms may be used for triage or as a second-reader in clinical practice, and that (2) automatic lumen segmentation is possible with a precision similar to that obtained by experts. The framework is open for new submissions through the website, at http://coronary.bigr.nl/stenoses/. (C) 2013 Elsevier B.V. All rights reserved.

L'epidemie actuelle de maladie a coronavirus 2019 (COVID-19) peut toucher tous les patients atteints de maladies auto-immunes et systemiques. Les patients souffrant d'un lupus systemique (LS) ont des facteurs de risque de developper des formes severes de COVID-19 en raison des comorbidites frequemment associees au LS (insuffisance renale chronique, syndrome metabolique notamment) et des traitements corticoides et immunosuppresseurs. Jusqu'a present, l'analyse des facteurs de risque de developper une COVID-19 severe au cours du LS a ete biaisee par le mode de recrutement des patients, puisque seuls les malades les plus symptomatiques ont ete inclus dans les etudes. Nous avons realise une etude observationnelle de la COVID-19 chez les patients suivis pour un LS en incluant les malades asymptomatiques ou paucisymptomatiques dans le but d'analyser les facteurs associes a une forme grave de cette maladie. Les cas etaient recrutes de 3 facons differentes : declarations spontanees au niveau local et au niveau national par les malades ou les medecins concernes ;declaration a la cohorte french RMD COVID-19 (etude ancillaire organisee sous l'egide de la filiere FAI2R) et depistage par serologie SARS-CoV-2 des malades avec lupus vus en consultation. Pour etre inclus, les patients devaient remplir les criteres de classification de lupus systemique de 1997 de l'ACR et/ou les criteres actualises de 2019 de l'EULAR/ACR et devaient avoir une infection a SARS-CoV-2 prouvee biologiquement par RT-PCR naso-pharyngee ou par serologie sanguine. Les malades asymptomatiques ou paucisymptomatiques etaient recrutes au cours des consultations de suivi habituelles du LS. 109 patients (dont 89 % de femmes) ont ete inclus entre le 1er mars 2020 et le 31 decembre 2020. L'âge median (Q1, Q3) des cas etait de 40 ans (33-56). Le score median de sequelle (SLICC damage index) etait de 0 (0-2). Les principales comorbidites etaient le surpoids (22 %), l'obesite (25 %), l'hypertension arterielle (22 %) et une maladie renale chronique (21 %). 7 % etaient fumeurs actifs. 42 % avaient un antecedent de nephropathie lupique. 87 % etaient traites par hydroxychloroquine (HCQ), 63 % par corticoide a la posologie moyenne de 8 mg/j (IC 95 % [6 ;10]) et 34 % par immunosuppresseur. Au moment de l'infection, 26 % rapportaient des signes d'activite clinique du LS, 51 % avaient une elevation des anticorps anti ADN double brin et 19 % une baisse du C3. Lors de la survenue de la COVID-19, L'HCQ, le corticoide et l'immunosuppresseur etaient maintenus respectivement dans 92 %, 93 % et 79 % des cas. 9 patients (8 %) ont eu une infection asymptomatique par le SARS-CoV-2. La COVID-19 s'est manifestee pour 40 patients (40 %) par une simple infection des voies aeriennes superieures, pour 31 (29 %) par une bronchite et pour 32 (30 %) par une pneumonie. 29 patients (26 %) ont ete hospitalises, 22 (21 %) ont necessite une oxygenotherapie, 12 (12 %) ont ete transferes en unite de soins intensifs (USI), 9 (8 %) ont developpe un syndrome de detresse respiratoire aigue et 4 (4 %) sont decedes. En analyses multivariees par regression logistique, les deux seuls facteurs associes au recours a une oxygenotherapie etaient l'âge (p = 0,047) et l'obesite (p = 0,02). Le seul facteur associe a une admission en USI etait l'âge (p = 0,01). Les corticoides, les immunosuppresseurs et l'antecedent de nephropathie lupique n'etaient pas independamment associes au risque de developper une forme necessitant le recours a l'oxygenotherapie ou une hospitalisation en USI. La consultation medicale de surveillance au cours du LS a permis d'inclure un grand nombre de malade paucisymptomatique ou asymptomatique habituellement non etudies dans ce type de travail. Cette serie fournit des elements rassurants quant aux facteurs de risque propres au LS de developper une forme grave de COVID-19. Ces facteurs de risque ne semblent en effet pas differents de ceux de la populat on generale, a savoir l'âge et l'obesite. Une maladie renale chronique, une nephropathie lupique, un traitement par corticoide et immunosuppresseur ne sont pas, dans cette cohorte, des facteurs independamment associes a une forme severe de la maladie. (French) [ABSTRACT FROM AUTHOR] Copyright of Revue de Medecine Interne is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)