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Background Carbapenemase-producing Enterobacteriaceae (CPE) are becoming of immediate concern for infection control policies. Prompt detection of CPE on health care setting admission is crucial to halt the spread of an outbreak. We report a cluster of 13 Klebsiella pneumoniae carbapenemase (KPC)-2–producing K pneumoniae cases in a tertiary care hospital.The objective of this study was to identify contributing factors originating the outbreak. Methods An outbreak investigation was conducted using descriptive epidemiology, observation of health care practices, and interviews of management staff. A root cause analysis was performed to identify patent and latent failures of infection control measures using the association of litigation and risk management method. Results The main patent failure was the delay in identifying KPC-2–producing K pneumoniae carriers. Contributing factors were work and environmental factors: understaffing, lack of predefined protocols, staff members' characteristics, and underlying patients' characteristics. Latent failures were as follows: no promotion of the national guidelines for prevention of CPE transmission, no clear procedure for the management of patients hospitalized abroad, no clear initiative for promoting a culture of quality in the hospital, biologic activity recently outsourced to a private laboratory, and poor communication among hospital members. Conclusion Clinical management should be better promoted to control hospital outbreaks and should include team work and safety culture. [ABSTRACT FROM AUTHOR]

Highlights: [•] Marteilia refringens was studied in bivalves: Mytilus galloprovincialis, Ruditapes decussatus and copepods Paracartia grani. [•] A non-involvement of clams in the parasite life cycle is suggested. [•] Mature sporangia were observed in mussels in spring and autumn. [•] Unusual parasite cells were observed in the digestive tract and gonad in young and adult copepodite stages of the copepod. [•] Positive PCR detections were observed in copepod eggs and all copepod developmental stages were retained by mussels. [ABSTRACT FROM AUTHOR]

Summary: Integra® dermal substitute is used as biomaterial after thermal injury. This artificial skin allows temporary coverage after burns excision, transformation of matrix in neo dermis and definitive engraftment. Infections are the most common complication of this technique. The objectives were to evaluate incidence of sepsis, to analyse microbiology and risk factors of developing Integra® infections. Patients with acute burns and indications of Integra® during five years were retrospectively enrolled. Demographic data, surgical procedures and microbiologic biopsies were collected. Fifty patients (40±15 years) were studied and 71surgical procedures using Integra® were performed. Burns were extended 45±21% total body surface area. Placement of Integra® was made 15±11days after burns and autografts 31±9days after placement of Integra®. Twenty-one patients had infected Integra® (42% of population). A total of 23Integra® infected sites were observed (15local and eight invasive). Diagnosis of infection was made after 13±5days using quantitative cultures. Other sites of infection were respiratory tract (46pneumonias) and others burned wounds (17infections outside of Integra®). Any risk factor was identified between burns who developed Integra® infections and the others. Incidence of infected Integra® was higher than in previous studies. Delayed application of Integra® after burns could explain higher incidence of infection. Pseudomonas aeruginosa and Staphylococcus aureus were more frequently isolated than other pathogens. Standardized technique for wounds coverage with Integra® is necessary to reduce incidence of infections and improve functional results in burns patients. [Copyright &y& Elsevier]

Abstract: A sensitive and specific method has been developed to detect semi-quantitatively testosterone in horse hair samples. The method involved a washing step with sodium dodecylsulfate aqueous solution. The mane and tail hair samples (100mg) were dissolved in 1mL of sodium hydroxide for 15min at 95°C in the presence of d3-boldenone used as internal standard. The next three steps involved diethyl ether extraction and a solid phase extraction on Isolute C18 (EC) cartridges eluted with methanol. The residue was derivatized by adding 100μL of acetonitrile and 30μL of PFPA then incubating for 15min at 60°C. After evaporation, 30μL of hexane was added and 2.5μL was injected into the column (a bonded phase fused silica capillary column DB5MS, 30m×0.25mm i.d.×0.25μm film thickness) of a Trace GC chromatograph. In order to improve the sensitivity of the method, damping gas flow has been optimized. Testosterone was identified in MS2 full scan mode on the Polaris Q instrument. The assay was capable of detecting less than 1pgmg−1. The recovery was close to 90%. The analysis of tail and mane samples collected from a gelding horse having received a single dose of testosterone propionate (1mgkg−1) showed the presence of testosterone in the range of 1–6pgmg−1 in hair collected during 5 months after administration. [Copyright &y& Elsevier]

Abstract: The response of mosquito larvae to plant toxins found in their breeding sites was investigated by using Aedes aegypti larvae and toxic arborescent leaf litter as experimental models. The relation between larval tolerance to toxic leaf litter and cytochrome P450 monooxygenases (P450s) was examined at the toxicological, biochemical and molecular levels. Larvae pre-exposed to toxic leaf litter show a higher tolerance to those xenobiotics together with a strong increase in P450 activity levels. This enzymatic response is both time- and dose-dependent. The use of degenerate primers from various P450 genes (CYPs) allowed us to isolate 16 new CYP genes belonging to CYP4, CYP6 and CYP9 families. Expression studies revealed a 2.3-fold over-expression of 1 CYP gene (CYP6AL1) after larval pre-exposure to toxic leaf litter, this gene being expressed at a high level in late larval and pupal stages and in fat bodies and midgut. The CYP6AL1 protein has a high level of identity with other insect''s CYPs involved in xenobiotic detoxification. The role of CYP genes in tolerance to natural xenobiotics and the importance of such adaptive responses in the capacity of mosquitoes to colonize new habitats and to develop insecticide resistance mechanisms are discussed. [Copyright &y& Elsevier]

We show that if a knot exterior satisfies certain conditions, then it has finite cyclic coverings with arbitrarily large numbers of nontrivial algebraic components in their SL(2,C) character varieties (Theorem A). As an example, these conditions hold for hyperbolic punctured torus bundles over the circle (Theorem B). We investigate in more detail the finite cyclic covers of the figure-eight knot exterior and show that for every integer m there exists a finite covering such that its SL(2,C) character variety contains curve components which have associated boundary slopes whose distance is larger than m (Theorem C). Lastly, we show that given an integer m then there exists a hyperbolic knot exterior in the 3-shpere which has a finite cyclic covering such that its SL(2,C) character variety contains more than m norm curve components each of which contains the character of a discrete faithful presentation of the fundamental group of the covering space (Theorem D). [Copyright &y& Elsevier]

B Conclusion: b In conclusion, the IVM decreased the Dengue vectors in schools in Cambodia. B Background: b In Cambodia, recent results showed very high resistance of Aedes aegypti to temephos, the only larvicide used in Cambodia, and two adulticides (permethrin used for net treatment, and deltamethrin for insecticide fumigation). [Extracted from the article]

Abstract: Aim of the study: This retrospective study was conducted in order to characterize mechanisms of resistance to β-lactams and clonality of 24 isolates of Pseudomonas aeruginosa. These strains were isolated from patients hospitalized in Rouen University Hospital between August 2004 and December 2006 and were resistant to cefepime and/or ceftazidime (PMR) by a mechanism not only related to overproduction of the cephalosporinase. Patients and methods: Clinical strains of PMR were characterized by conventional biochemical methods, antibiotic susceptibility testing by disk diffusion in agar with or without cloxacillin and RAPD for genetic comparison. Identification of β-lactamase was performed by PCR amplification followed by sequencing of bla genes. Results: All strains produced the extended-spectrum β-lactamase (ESBL) TEM-116. Epidemiological study identified eight unrelated strains, eight related strains originating from a single unit, three related strains isolated in different wards and five related strains coproducing TEM-116 and SHV-2a but isolated in different units. Detection of ESBL in these strains was difficult due to a low level of ESBL production. Conclusion: This is the first report of TEM-116 in France in 24 strains of P. aeruginosa and its association with SHV-2a in five cases. SHV-2a has been described in P. aeruginosa in France but not TEM-116 which was recently reported in this species, in China and in Netherlands (in a strain coproducing SHV-12). ESBL detection in PMR remains indispensable since these strains can cause therapeutic failures. [Copyright &y& Elsevier]

• The issue of this article is of interest to prescribing physicians, infectious disease physicians, bacteriologists, and hygienists. Recalling the guidelines on blood culture collection to limit the number of unnecessary blood cultures reduces the number of venipunctures in patients, facilitates the interpretation of results, and has an economic impact. The originality of the intervention was the presence of a striking look on the poster to draw the reader's attention, that has more impact than a poster with only guidelines without image. At the methodological level, randomization makes it possible to ensure the initial comparability of groups. The before-after design, in the presence of a control group, makes it possible to evaluate the effect of the intervention. To reduce the number of blood culture samples collected. We performed a cluster randomized controlled trial in adult acute care, and subacute care and rehabilitation wards in a university hospital in France. A poster associating an image of eyes looking at the reader with a summary of blood culture sampling guidelines was displayed in hospital wards in the intervention group. The incidence rate of blood cultures per 1000 days during pre- and post-intervention periods was calculated. Thirty-one wards participated in the study. The median difference in blood cultures/1000 days between periods was −1.863 [−11.941; 1.007] in the intervention group and −5.824 [−14.763; −2.217] in the control group (P = 0.27). The intervention did not show the expected effect, possibly due to the choice of blood cultures as a target of good practice, but also to confounding factors such as the stringent policy of decreasing unnecessary costly testing. [ABSTRACT FROM AUTHOR]

The clinical course of a case of infant botulism was characterized by several relapses despite therapy with amoxicillin and metronidazole. Botulism was confirmed by identification of botulinum toxin and Clostridium botulinum in stools. A C. botulinum A2 strain resistant to penicillins and with heterogeneous resistance to metronidazole was isolated from stool samples up to 110 days after onset. Antibiotic susceptibility was tested by disc agar diffusion and MICs were determined by Etest. Whole genome sequencing allowed detection of a gene cluster composed of bla CBP for a novel penicillinase, blaI for a regulator, and blaR1 for a membrane-bound penicillin receptor in the chromosome of the C. botulinum isolate. The purified recombinant penicillinase was assayed. Resistance to β-lactams was in agreement with the kinetic parameters of the enzyme. In addition, the β-lactamase gene cluster was found in three C. botulinum genomes in databanks and in two of 62 genomes of our collection, all the strains belonging to group I C. botulinum . This is the first report of a C. botulinum isolate resistant to penicillins. This stresses the importance of antibiotic susceptibility testing for adequate therapy of botulism. [ABSTRACT FROM AUTHOR]

Abstract: Objective: We studied antibiotic resistance in pediatric UTIs and we evaluated the impact of antibiotic exposure in the previous 12 months, very little French data being available for this population. Methods: We conducted a multicenter prospective study including children consulting for, or admitted in 2 hospitals. Prior antibiotic exposure was documented from their health record. Results: One hundred and ten patients (73 girls), 11 days to 12 years of age, were included in 10 months. Ninety-six percent presented with pyelonephritis, associated to uropathy for 25%. Escherichia coli was predominant (78%), followed by Proteus spp. and Enterococcus spp. The antibiotic resistance rate of E. coli was high and close to that reported for adults with complicated UTIs: amoxicillin 60%, amoxicillin-clavulanate 35%, cefotaxim 5%, trimethoprim-sulfametoxazole 26%, nalidixic acid 9%, ciprofloxacin 7%, gentamycin 1%, nitrofurantoin and fosfomycin 0%. The antibiotic exposure in the previous 12 months involved 62 children (56%) most frequently with β-lactams (89%) for a respiratory tract infection (56%). A clear relationship between exposure and resistance was observed for amoxicillin (71% vs. 46%), first generation (65% vs. 46%) and third generation (9% vs. 3%) cephalosporins, or trimethoprim-sulfamethoxazole (36% vs. 15%). However, antibiotic exposure could not account alone for the results, as suggested by the 7% of ciprofloxacin resistance, observed without any identified previous treatment. Conclusion: Bacterial species and antibiotic resistance level in children are similar to those reported for adults. Antibiotic exposure in the previous 12 months increases the risk of resistance but other factors are involved (previous antibiotic therapies and fecal-oral or mother-to-child transmission). [Copyright &y& Elsevier]

Primary breast lymphoma is a difficult diagnosis because it is very rare. It can be found during pregnancy. Its diagnosis is often delayed. Treatment combines radiotherapy and chemotherapy. Prognosis is generally bad. Therefore, it is most important to examine breasts during pregnancy. [Copyright &y& Elsevier]

La prise en charge d'une détresse respiratoire aiguë dans un contexte de pneumopathie interstitielle diffuse (PID) en secteur de réanimation est complexe de par l'hétérogénéité des étiologies et un arsenal thérapeutique limité. Nous présentons le cas d'une patiente de 50 ans présentant une acutisation d'une PID associée à une connectivite de chevauchement sclérodermie systémique (ScS)/polyarthrite rhumatoïde (PR) compliquée de syndrome de détresse respiratoire aiguë (SDRA) et dont la prise en charge a permis de démasquer une PID indépendante de la connectivite. Le diagnostic du syndrome de chevauchement ScS/PR était connu depuis 3 mois. Les critères de classification ACR/EULAR 2013 et 2009 étaient vérifiés pour la ScS et la PR, respectivement. Les ACPA étaient positifs ; les anticorps spécifiques aux myosites négatifs. La patiente recevait de la prednisone à 15 mg/j avec du cyclophosphamide (CYC) 600 mg/m2 IVL mensuel pour une pneumopathie interstitielle non spécifique fibrosante (PINS), en aggravation clinique et fonctionnelle. La CVF et la DLCO étaient respectivement de 52 %th et 20 %th, au diagnostic. L'état respiratoire s'était brutalement dégradé avec l'apparition d'une dyspnée hypoxémiante rapidement progressive vers un SDRA sévère compliqué de choc sans documentation d'une cause infectieuse. L'antibiothérapie était élargie à l'introduction du vasopresseur et les mesures de ventilation mécanique optimisées par le recours au décubitus ventral. Devant la gravité du tableau respiratoire, la patiente a été traitée - dans l'hypothèse d'une acutisation de la PID associée à la connectivite - par une première dose de rituximab à 375 mg/m2 associée à 1000 mg/j de méthylprednisone pendant 3 jours. L'origine toxique, attribuable à la CYC, n'était pas retenue au vu de la chronologie des manifestations cliniques. L'association de ces traitements avait permis l'amélioration clinique spectaculaire en 24 heures avec une extubation de la patiente 48 heures plus tard. L'acutisation de la PINS a été remise en cause par la corticosensibilité du SDRA et la rapidité de la réponse clinique. Le diagnostic de pneumopathie d'hypersensibilité (PHS) suraiguë est finalement privilégié devant une exposition à des animaux et produits de ferme, un aspect scanographique compatible avec une PHS, et un lavage bronchoalvéolaire montrant une hypercellularité avec une augmentation du nombre de polynucléaires neutrophiles et éosinophiles. Le sérodiagnostic des pneumopathies d'hypersensibilité trouvera des précipitines de plusieurs antigènes (A. versicolor , P. chrysogenum , C. sphaerospermum , S. chartarum). La PHS représente entre 4 et 15 % des PID et est une cause inhabituelle de SDRA immunoallergique secondaire à l'inhalation d'allergène. Les PHS aiguës prennent l'aspect d'une infection et plus rarement de SDRA. Dans le présent cas, cette entité venait compliquer une PID associée à une connectivite de chevauchement qui, en soi, constitue un facteur de risque de SDRA. Des présentations telles que celle de notre patiente permettent de souligner l'importance de l'anamnèse pour préciser l'habitus et le curriculum laboris souvent décisives dans la démarche diagnostique. La prise en charge réanimatoire est compliquée par les limites imposées par l'information disponible dans un contexte d'urgence, une présentation de PHS différant des formes classiques, et le terrain d'immunodépression induit par des traitements. Les examens complémentaires sont rarement d'aide au diagnostic urgent et ne sont informatifs qu'à distance de l'épisode aigu. Le scanner thoracique montrant classiquement des opacités en verre dépoli associées à des micronodules centrolobulaires diffus peut être difficile d'interprétation lorsqu'il existe des stigmates de SDRA s'ajoutant à une PID d'une autre origine. Le recours à la biopsie pulmonaire n'a pas d'intérêt dans le cadre d'une PHS aiguë. L'absence de thérapeutique spécifique dans le cadre de PID en exacerbation aiguë doit amener à considérer le recours à une corticothérapie de sauvetage. Cela impose d'éliminer et de traiter les potentielles infections opportunistes et/ou surinfections pulmonaires. Malgré une corticosensibilité caractéristique de la PHS, la mortalité reste élevée. La PHS aiguë est une cause exceptionnelle de SDRA dont la corticosensibilité devrait constituer un argument pour sa recherche dans des contextes de PID subaiguës préalablement connues. Une exacerbation aiguë de PID rapidement régressive à l'initiation d'une corticothérapie doit ainsi amener à la rechercher. [ABSTRACT FROM AUTHOR]

The fission probability distributions of 232,233,234Pa and 231Th have been measured up to an excitation energy of 15 MeV, using the transfer reactions 232Th(3He,t)232Pa, 232Th(3He,d)233Pa, 232Th(3He,p)234Pa and 232Th(3He,4He)231Th. From these measurements, the neutron induced fission cross sections of 231Pa, 233Pa and 230Th have been determined from the product of the fission probabilities of 232Pa, 233Pa and 231Th respectively with the calculated compound nucleus formation cross sections in the 231Pa+n, 233Pa+n and 230Th+n reactions. The validity of the applied method has been successfully tested with the existing neutron induced fission cross sections of 230Th and 231Pa. Special emphasis is put on the 233Pa(n,f) reaction which is of importance for thorium fueled nuclear reactors. Based on a statistical model analysis of the neutron induced fission cross section as a function of neutron energy, it has been possible to determine the barrier parameters of the 234Pa fissioning nucleus. Cross sections for the compound nucleus inelastic scatttering 233Pa(n,n′) and radiative capture 233Pa(n,γ) reactions have also been calculated and compared with recent evaluations. [Copyright &y& Elsevier]

The European Committee on Antimicrobial Susceptibility Testing (EUCAST) recently warned about an area of technical uncertainty (ATU) of amoxicillin/clavulanate (AMX/C) disk susceptibility testing against members of the Enterobacterales. Thus, we aimed to compare the reliability of three routine methods and to evaluate the impact of the ATU. 286 Escherichia coli strains (including 159 AMX-resistant strains) were categorized for the two EUCAST AMX/C breakpoints by disk diffusion (Bio-Rad), the Phoenix automated system (Becton Dickinson) and the Etest (AES) compared to the broth microdilution reference method. By microdilution, 84.2% of strains were AMX/C-susceptible using the urinary breakpoint (MIC ≤32 mg/L) and 62.2% using the systemic breakpoint (MIC ≤8 mg/L), with 63.6% of MICs between 4 and 16 mg/L. For the systemic breakpoint, category agreement (CA) and very major error (VME) were unacceptable for the Etest (71.7% and 27.3%), disk (73.1% and 23.4% at 19-mm cut-off) and to a lesser extent for the Phoenix system (83.6% and 10.5%). For disks, an unacceptable VME rate was observed for diameters up to 22 mm, probably due to overcharged disks. For the Etest, VMEs were high at 6 mg/L (46/63) and 8 mg/L (22/29). For the urinary breakpoint, CA was more acceptable for disk (88.9%) and Etest (84.3%) but was unevaluable for Phoenix. AMX/C susceptibility testing of E. coli for systemic breakpoint was unreliable with the three routine methods, explained mainly by the high prevalence (~60%) of strains with microdilution MICs around the breakpoint (8 mg/L). Our data confirmed the EUCAST 19–20-mm ATU for disk and suggest introducing ATU for Etest MIC values of 6 and 8 mg/L. [ABSTRACT FROM AUTHOR]

The GloPID-R (Global Research Collaboration for Infectious Disease Preparedness) chikungunya (CHIKV), o'nyong-nyong (ONNV) and Mayaro virus (MAYV) Working Group has been established to investigate natural history, epidemiology and clinical aspects of infection by these viruses. Here, we present a report dedicated to entomological aspects of CHIKV, ONNV and MAYV. Recent global expansion of chikungunya virus has been possible because CHIKV established a transmission cycle in urban settings using anthropophilic vectors such as Aedes albopictus and Aedes aegypti. MAYV and ONNV have a more limited geographic distribution, being confined to Africa (ONNV) and central-southern America (MAYV). ONNV is probably maintained through an enzootic cycle that has not been characterized yet, with Anopheles species as main vectors and humans as amplification hosts during epidemics. MAYV is transmitted by Haemagogus species in an enzootic cycle using non-human primates as the main amplification and maintenance hosts, and humans becoming sporadically infected when venturing in or nearby forest habitats. Here, we focused on the transmission cycle and natural vectors that sustain circulation of these viruses in their respective locations. The knowledge of the natural ecology of transmission and the capacity of different vectors to transmit these viruses is crucial to understand CHIKV emergence, and to assess the risk that MAYV and ONNV will expand on wide scale using anthropophilic mosquito species not normally considered primary vectors. Finally, the experts identified knowledge gaps and provided adapted recommendations, in order to address future entomological investigations in the right direction. • Global expansion of chikungunya virus is possible because it established a urban transmission cycle using Aedes vectors. • Mayaro virus uses Haemagogus species and non-human primates for transmission, with humans representing incidental hosts. • Anopheles species are main vectors of o'nyong-nyong virus but its enzootic cycle has not been characterized yet. • Transmission cycle and natural vectors of these viruses in respective locations are described. • It exists a risk of expansion of ONNV and MAYV on wide scale using anthropophilic mosquito species. [ABSTRACT FROM AUTHOR]

Introduction Le purpura thrombotique thrombocytopénique (PTT) est une microangiopathie thrombotique (MAT) grave responsable de défaillance multi-organe en l’absence de thérapeutique. Il peut être associé à des connectivites dont le syndrome Sjögren primitif (SSp). Nous rapportons le cas d’une patiente suivie pour un SSp et présentant une rechute précoce de PTT malgré un traitement optimal au diagnostic. Observation Une femme de 49 ans, sans antécédent particulier en dehors d’un SSp sans facteur d’évolutivité depuis 20 ans, s’est présentée aux urgences pour des troubles neurologiques, arthralgies et un syndrome hémorragique depuis 24 heures. On ne note pas d’épisode infectieux ou de nouveau traitement introduit. La fonction rénale n’est pas altérée et le dosage de la troponine Ic est normal. L’IRM cérébrale montre l’absence d’anomalie. Le diagnostic de MAT amène à une prise en charge en secteur de réanimation et à l’initiation d’échanges plasmatiques (EP) quotidiens avec 1,5 de volume plasmatique total substitué à chaque séance. On caractérisera le PTT par l’effondrement de l’activité ADAMTS13 et le dosage d’anticorps anti-ADAMTS13 supra-seuil (> 100 UI/mL). Le SSp répondait aux critères de classification et ne relevait jusqu’à l’épisode de MAT d’aucun traitement. On précise la positivité des facteurs antinucléaires avec la présence d’anti-SSA. La patiente reçoit une corticothérapie à 1 mg/kg/j et de l’hydroxychloroquine à 6,5 mg/kg/j y est associée. Au vu de l’association de SSp au PTT, un traitement par rituximab est administré à 375 mg/m 2 selon un schéma j1, j4, j8, j15 (PTT-RTX 1) sans intervalle libre depuis le début des EP. L’examen clinique se normalise à j2 de prise en charge. La thrombopénie et les LDH se corrigent à j4 chez une patiente restant asymptomatique. Les EP sont ainsi arrêtés sur les critères de réponse biologique classique. À j7 de prise en charge, la thrombopénie réapparaît et l’anémie s’aggrave avec la réapparition de schizocytes. Les EP quotidiens sont réinitiés et progressivement espacés (au total 16 EP) suite à une réponse biologique. La rémission est maintenue après un recul de 2 mois. Discussion Le SSp est la connectivite la plus fréquemment associée au PTT (SSp-PTT) après le lupus érythémateux systémique (LES). Très peu d’études se sont intéressées à caractériser les patients avec SSp et MAT [1,2] . Les quelques données disponibles montrent l’absence de manifestation extraglandulaire ou de facteurs de risque d’évolution vers une maladie lymphomateuse [1] . Si le risque de maladie auto-immune a bien été décrit et corrélé à la présence d’anti-SSA et anti-ADN natif au diagnostic de PTT, les données de la littérature sont insuffisantes pour caractériser la réponse thérapeutique dans les SSp-PTT. Certaines études ont évoqué un pronostic plus sombre dans les PTT satellites de LES [3] . Les stratégies thérapeutiques n’ont pas été étudiées et sont classiquement adaptées à la réponse thérapeutique initiale. L’hydroxychloroquine ne semble pas prévenir le PTT [1,3] . Il existe une tendance à l’augmentation du taux de réévolutivité précoce dans les SSp-PTT comparée au PTT idiopathique seul [2] . Le traitement par rituximab, ayant déjà prouvé son efficacité dans les PTT auto-immuns réfractaires par une action de déplétion des lymphocytes B auto-réactifs producteurs d’anticorps anti-ADAMTS13, semble justifié dans la prise en charge précoce des SSp-PTT. Conclusion Le SSp-PTT est un évènement rare dans le cadre d’un SSp et relève d’une thérapeutique précoce par rituximab au vu du potentiel évolutif potentiellement plus important que les formes de PTT non associées aux connectivites. De futures études sur les MAT permettront de mieux caractériser l’histoire naturelle du SSp-PTT. [ABSTRACT FROM AUTHOR]

Le syndrome de Barth est une maladie héréditaire due à des mutations du gène TAFAZZINE (TAZ/G4.5) situé en Xq28. Ce syndrome associe une cardiomyopathie dilatée, une neutropénie, un syndrome myopathique, un retard de croissance et des troubles cognitifs, qui font penser à une cytopathie mitochondriale. À 5 semaines de vie, M. est hospitalisé pour insuffisance cardiaque sévère, dápparition brutale suite à une cardiomyopathie dilatée. L’évolution est favorable sous IEC et digoxine, avec, dans les années suivantes, l’installation d’un retard psychomoteur avec hypotonie sans hyper-CKémie. Des infections ORL, pulmonaires et digestives fréquentes, contemporaines d’une neutropénie, sont présentes depuis la petite enfance. Une élévation de l’acide 3- méthylglutaconique sur la chromatographie des acides organiques urinaires évoque le diagnostic d’un syndrome de Barth. À l’âge de 9 ans, l’analyse du gène TAZ retrouve une délétion partielle. L’association, chez un garçon, d’une neutropénie, d’un syndrome myopathique et d’une cardiomyopathie dilatée sévère précoce et parfois «transitoire» doit faire penser au syndrome de Barth. La présence dúne acidurie 3-méthylglutaconique et d’une hypocholéstérolémie rend vraisemblable ce diagnostic que la biologie moléculaire confirmera. [ABSTRACT FROM AUTHOR]