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991.
Guillaume?Bonnet Cindy?Batisse Marion?Bessadet Emmanuel?NicolasEmail authorView authors OrcID profile Jean-Luc?Veyrune 《BMC oral health》2017,17(1):155
Background
Historically, the complete removable denture is the last prosthetic procedure to switch to digital techniques whose advantages are mainly observed in the laboratory stages; however, it is not possible to measure the depressibility of the oral mucosa using optical cameras, thus conventional impression techniques are still necessary. This article describes the clinical and laboratory procedure and practitioners appraisal of the first fifteen digitally designed complete removable dental prostheses.Methods
Several systems are now available including the Wieland® Digital Denture® which offers a complete procedure. This system is composed of a five axis-milling machine combined with a laboratory scanner and a design software application. Fifteen rehabilitations were carried out using the Wieland® system.Results
The practitioner’s role is simplified by intraoral recording with a central point and a reduced number of sessions. The prosthesis laboratory requires considerable investment in learning and equipment, making it possible to obtain ideal mounting assemblies in accordance with the occluso-prosthetic concept of bilateral balanced occlusion. The absence of polymerization and therefore of base deformation risks reduce the equilibration step. Finally, the creation of templates as an alternative to the assembly of teeth on wax makes it possible to functionally validate (masticatory and phonatory) the future dentures. However, this procedure still presented some limitations in terms of scanning and software scope of applications.Conclusion
Digital denture design software is relatively efficient and helps to standardize clinical results. However, to this date, improvements of the software are still required for a routine use.992.
Axonal Neuropathies due to Mutations in Small Heat Shock Proteins: Clinical,Genetic, and Functional Insights into Novel Mutations 下载免费PDF全文
Andoni Echaniz‐Laguna Thomas Geuens Philippe Petiot Yann Péréon Elias Adriaenssens Mansour Haidar Simona Capponi Thierry Maisonobe Emmanuel Fournier Odile Dubourg Bertrand Degos François Salachas Timothée Lenglet Bruno Eymard Emilien Delmont Jean Pouget Raul Juntas Morales Cyril Goizet Philippe Latour Vincent Timmerman Tanya Stojkovic 《Human mutation》2017,38(5):556-568
In this study, we describe the phenotypic spectrum of distal hereditary motor neuropathy caused by mutations in the small heat shock proteins HSPB1 and HSPB8 and investigate the functional consequences of newly discovered variants. Among 510 unrelated patients with distal motor neuropathy, we identified mutations in HSPB1 (28 index patients/510; 5.5%) and HSPB8 (four index patients/510; 0.8%) genes. Patients have slowly progressive distal (100%) and proximal (13%) weakness in lower limbs (100%), mild lower limbs sensory involvement (31%), foot deformities (73%), progressive distal upper limb weakness (29%), mildly raised serum creatine kinase levels (100%), and central nervous system involvement (9%). We identified 12 HSPB1 and four HSPB8 mutations, including five and three not previously reported. Transmission was either dominant (78%), recessive (3%), or de novo (19%). Three missense mutations in HSPB1 (Pro7Ser, Gly53Asp, and Gln128Arg) cause hyperphosphorylation of neurofilaments, whereas the C‐terminal mutant Ser187Leu triggers protein aggregation. Two frameshift mutations (Leu58fs and Ala61fs) create a premature stop codon leading to proteasomal degradation. Two mutations in HSPB8 (Lys141Met/Asn) exhibited increased binding to Bag3. We demonstrate that HSPB1 and HSPB8 mutations are a major cause of inherited motor axonal neuropathy. Mutations lead to diverse functional outcomes further demonstrating the pleotropic character of small heat shock proteins. 相似文献
993.
994.
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995.
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
996.
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
997.
998.
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
999.
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
1000.
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