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991.
992.
Charlotte Durtette Eric Hachulla Matthieu Resche-Rigon Thomas Papo Thierry Zénone Bertrand Lioger Christophe Deligny Marc Lambert Cédric Landron Jacques Pouchot Jean Emmanuel Kahn Christian Lavigne Benoit De Wazieres Robin Dhote Guillaume Gondran Edouard Pertuiset Thomas Quemeneur Mohamed Hamidou Arsène Mekinian 《Autoimmunity reviews》2017,16(12):1219-1223
Background
Cogan syndrome is mainly treated with steroids. We aimed to determine the place of DMARDs and biologic-targeted treatments.Patients and methods
We conducted a French nationwide retrospective study of patients with Cogan syndrome (n = 40) and a literature review of cases (n = 22) and analyzed the efficacy of disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor α (TNF-α) antagonists.Results
We included 62 patients (31 females) (median age 37 years [range 2–76]. At diagnosis, 61 patients (98%) had vestibulo-auditory symptoms, particularly bilateral hearing loss in 41% and deafness in 31%. Ocular signs were present in 57 patients (92%), with interstitial keratitis in 31 (51%). The first-line treatment consisted of steroids alone (n = 43; 70%) or associated with other immunosuppressive drugs (n = 18; 30%). Overall, 13/43 (30%) and 4/18 (22%) patients with steroids alone and with associated immunosuppressive drugs, respectively (p = 0.8), showed vestibulo-auditory response; 32/39 (82%) and 15/19 (79%) ocular response; and 23/28 (82%) and 10/14 (71%) general response. Overall 61 patients had used a total of 126 lines of treatment, consisting of steroids alone (n = 51 lines), steroids with DMARDs (n = 65) and infliximab (n = 10). Vestibulo-auditory response was significantly more frequent with infliximab than DMARDs or steroids alone (80% vs 39% and 35%, respectively), whereas ocular, systemic and acute-phase reactant response rates were similar. Infliximab was the only significant predictor of vestibulo-auditory improvement (odds ratio 20.7 [95% confidence interval 1.65; 260], p = 0.019).Conclusion
Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome, but prospective studies are necessary. 相似文献993.
Luciano G Martelotto Maria R De Filippo Charlotte KY Ng Rachael Natrajan Laetitia Fuhrmann Joanna Cyrta Salvatore Piscuoglio Huei‐Chi Wen Raymond S Lim Ronglai Shen Anne M Schultheis Y Hannah Wen Marcia Edelweiss Odette Mariani Göran Stenman Timothy A Chan Pierre‐Emmanuel Colombo Larry Norton Britta Weigelt 《The Journal of pathology》2015,237(2):179-189
994.
Ho Tsong Fang R Khatissian E Monceaux V Cumont MC Beq S Ameisen JC Aubertin AM Israël N Estaquier J Hurtrel B 《AIDS (London, England)》2005,19(7):663-673
BACKGROUND: An attenuated immunodeficiency virus has been long considered innocuous. Nevertheless, converging data suggest that low levels of viral replication can still provoke AIDS. Pathogenesis of these attenuated infections is not understood. OBJECTIVES: To determine the pathogenicity of a long-term attenuated infection and to delineate T-cell dynamics during such an infection. METHODS: This is a cross-sectional study of 12 rhesus macaques infected with SIV Delta nef for 8 years. We evaluated apoptosis (annexin V), activation (HLA-DR, Ki67), and newly generated T cells (TCR excision circle: TREC). RESULTS: Infection with SIV Delta nef induced pathological CD4 T-cell depletion after 8 years of infection. Virus replication and CD8 T-cell activation positively correlated with the rate of disease progression. The frequency of TREC within CD8+CD45RA+ cells increased in SIV Delta nef-infected animals compared to age-matched non-infected controls. Moreover, in the cohort of infected animals, TREC+CD45RA+CD4+ T-cell counts correlated strongly with non-progression to AIDS. The animal with the lowest rate of disease progression exhibited a 115-fold increase in TREC+CD45RA+CD4+ T-cell counts compared to age-matched non-infected controls. In contrast, the animal showing the fastest rate of progression to AIDS displayed 600-fold lower TREC+CD45RA+CD4+ T-cell counts compared to age-matched non-infected controls. CONCLUSIONS: Our results suggest that the thymus plays a major role in the pathogenesis of an attenuated SIV infection and that a sustained thymic output could maintain CD4 T-cell homeostasis in the context of low viral loads. 相似文献
995.
996.
John E. J. RaskoKathryn N. North†Emmanuel J. Favaloro‡Linda Grispo‡Michael C. Berndt§ 《British journal of haematology》1995,89(3):582-588
Haemostatis has not previously been studied in patients with neurofibromatosis 1 (NF-1), despite case reports of an association with von Willebrand disease and reported excessive bleeding in those undergoing surgery for neurofibromas. Platelets from NF-1 patients ( n = 28) were tested for aggregation and ATP release with agonists including ADP, arachidonic acid, thrombin and collagen. Mepacrine staining of platelets and three different assays for von Willebrand factor (VWF) were also performed.
In response to collagen as the platelet agonist, tested at both 2 and 1 μg/ml, NF-1 patients had an attenuated rate of aggregation ( P < 0.007), aggregation lag phase ( P < 0.005) and ATP release ( P < 0.045), as well as requiring higher collagen concentrations to attain threshold aggregation response ( P = 0.041). Normal platelets resuspended in selected NF-1 plasma exhibited significantly reduced platelet aggregation and release compared to controls, which was not corrected by mixing 1:1 with normal plasma. Collagen binding activity was reduced in NF-1 patients compared with controls (127% v 161%, P = 0.05).
As a group, patients with NF-1 display defective platelet function characterized by in vitro evidence of impaired responsiveness to collagen. It is suggested that a plasma factor, present in a significant proportion of NF-1 patients, may interfere with the ability of collagen to interact with other proteins such as von Willebrand factor and the platelet collagen receptor. 相似文献
In response to collagen as the platelet agonist, tested at both 2 and 1 μg/ml, NF-1 patients had an attenuated rate of aggregation ( P < 0.007), aggregation lag phase ( P < 0.005) and ATP release ( P < 0.045), as well as requiring higher collagen concentrations to attain threshold aggregation response ( P = 0.041). Normal platelets resuspended in selected NF-1 plasma exhibited significantly reduced platelet aggregation and release compared to controls, which was not corrected by mixing 1:1 with normal plasma. Collagen binding activity was reduced in NF-1 patients compared with controls (127% v 161%, P = 0.05).
As a group, patients with NF-1 display defective platelet function characterized by in vitro evidence of impaired responsiveness to collagen. It is suggested that a plasma factor, present in a significant proportion of NF-1 patients, may interfere with the ability of collagen to interact with other proteins such as von Willebrand factor and the platelet collagen receptor. 相似文献
997.
Lymphotoxin‐β receptor in microenvironmental cells promotes the development of T‐cell acute lymphoblastic leukaemia with cortical/mature immunophenotype 下载免费PDF全文
Mónica T. Fernandes Marinella N. Ghezzo André B. Silveira Ravi K. Kalathur Vanda Póvoa Ana R. Ribeiro Sílvia R. Brandalise Emmanuel Dejardin Nuno L. Alves Jacques Ghysdael João T. Barata José Andres Yunes Nuno R. dos Santos 《British journal of haematology》2015,171(5):736-751
Lymphotoxin‐mediated activation of the lymphotoxin‐β receptor (LTβR; LTBR) has been implicated in cancer, but its role in T‐cell acute lymphoblastic leukaemia (T‐ALL) has remained elusive. Here we show that the genes encoding lymphotoxin (LT)‐α and LTβ (LTA, LTB) are expressed in T‐ALL patient samples, mostly of the TAL/LMO molecular subtype, and in the TEL‐JAK2 transgenic mouse model of cortical/mature T‐ALL (Lta, Ltb). In these mice, expression of Lta and Ltb is elevated in early stage T‐ALL. Surface LTα1β2 protein is expressed in primary mouse T‐ALL cells, but only in the absence of microenvironmental LTβR interaction. Indeed, surface LT expression is suppressed in leukaemic cells contacting Ltbr‐expressing but not Ltbr‐deficient stromal cells, both in vitro and in vivo, thus indicating that dynamic surface LT expression in leukaemic cells depends on interaction with its receptor. Supporting the notion that LT signalling plays a role in T‐ALL, inactivation of Ltbr results in a significant delay in TEL‐JAK2‐induced leukaemia onset. Moreover, young asymptomatic TEL‐JAK2;Ltbr?/? mice present markedly less leukaemic thymocytes than age‐matched TEL‐JAK2;Ltbr+/+ mice and interference with LTβR function at this early stage delayed T‐ALL development. We conclude that LT expression by T‐ALL cells activates LTβR signalling in thymic stromal cells, thus promoting leukaemogenesis. 相似文献
998.
Saad J. Taj-Aldeen Blandine Rammaert Maria Gamaletsou Nikolaos V. Sipsas Valerie Zeller Emmanuel Roilides Dimitrios P. Kontoyiannis Andy O. Miller Vidmantas Petraitis Thomas J. Walsh Olivier Lortholary 《Medicine》2015,94(50)
Osteoarticular mycoses due to non-Aspergillus moulds are uncommon and challenging infections.A systematic literature review of non-Aspergillus osteoarticular mycoses was performed using PUBMED and EMBASE databases from 1970 to 2013.Among 145 patients were 111 adults (median age 48.5 [16–92 y]) and 34 pediatric patients (median age 7.5 [3–15 y]); 114 (79.7%) were male and 88 (61.9%) were immunocompromised. Osteomyelitis was due to direct inoculation in 54.5%. Trauma and puncture wounds were more frequent in children (73.5% vs 43.5%; P = 0.001). Prior surgery was more frequent in adults (27.7% vs 5.9%; P = 0.025). Vertebral (23.2%) and craniofacial osteomyelitis (13.1%) with neurological deficits predominated in adults. Lower limb osteomyelitis (47.7%) and knee arthritis (67.8%) were predominantly seen in children. Hyalohyphomycosis represented 64.8% of documented infections with Scedosporium apiospermum (33.1%) and Lomentospora prolificans (15.8%) as the most common causes. Combined antifungal therapy and surgery was used in 69% of cases with overall response in 85.8%. Median duration of therapy was 115 days (range 5–730). When voriconazole was used as single agent for treatment of hyalohyphomycosis and phaeohyphomycosis, an overall response rate was achieved in 94.1% of cases.Non-Aspergillus osteoarticular mycoses occur most frequently in children after injury and in adults after surgery. Accurate early diagnosis and long-course therapy (median 6 mo) with a combined medical-surgical approach may result in favorable outcome. 相似文献
999.
Hugo Botebol Emmanuel Lesuisse Robert ?uták Christophe Six Jean-Claude Lozano Philippe Schatt Valérie Vergé Amos Kirilovsky Joe Morrissey Thibaut Léger Jean-Michel Camadro Audrey Gueneugues Chris Bowler Stéphane Blain Fran?ois-Yves Bouget 《Proceedings of the National Academy of Sciences of the United States of America》2015,112(47):14652-14657
1000.
Hermann E Truyens C Alonso-Vega C Even J Rodriguez P Berthe A Gonzalez-Merino E Torrico F Carlier Y 《Blood》2002,100(6):2153-2158
Fetal/neonatal immune responses generally are considered to be immature and weaker than that of adults. We have studied the cord-blood T cells of newborns congenitally infected with Trypanosoma cruzi, the protozoan agent of Chagas disease. Our data demonstrate a predominant activation of CD8 T cells expressing activation markers and armed to mediate effector functions. The analysis of the T-cell receptor beta chain variable repertoire shows the oligoclonal expansion of these T lymphocytes, indicating that activation was driven by parasite antigens. Indeed, we have detected parasite-specific CD8 T cells secreting interferon-gamma after coincubation with live T cruzi. This response is enhanced in the presence of recombinant interleukin-15, which limits the T-cell spontaneous apoptosis. These findings point out that the fetal immune system is more competent than previously appreciated, since fetuses exposed to live pathogens are able to develop an adultlike immune CD8 T-cell response. 相似文献