Annales de la Faculté des lettres de Bordeaux .
Université de Toulouse. Faculté des lettres.;Université de Bordeaux. Facult...
Check for Full Text
Annales.
Université de Bordeaux. Faculté des lettres et sciences humaines.;Universit...
Annales.
Université de Bordeaux. Faculté des lettres et sciences humaines.;Universit...
Repertoire analytique de littérature française : RALF.
R.A.L.F
RALF
Book
|
Repertoire analytique de littérature française : RALF.; 01/01/1971
Please log in to see more details
R.A.L.F
RALF
Université de Bordeaux. Faculté des lettres et sciences humaines. Section d...
Book
|
Repertoire analytique de littérature française : RALF.; 01/01/1971
Please log in to see more details
Actes et mémoires du ... Congrès international de langue et littérature d'oc.
Actes et mémoires.
Book
|
Actes et mémoires du ... Congrès international de langue et littérature d'oc.; 01/01/1961
Please log in to see more details
Actes et mémoires.
Congrès international de langue et littérature d'oc.;Université de Bordeaux...
Book
|
Actes et mémoires du ... Congrès international de langue et littérature d'oc.; 01/01/1961
Please log in to see more details
Revue des universités du Midi.
Université de Bordeaux. Faculté des lettres et sciences humaines.;Universit...
Catalogue méthodique des moulages des oeuvres de sculpture grecque
Book
|
Catalogue méthodique des moulages des oeuvres de sculpture grecque; 01/01/1892
Please log in to see more details
Université de Bordeaux. Faculté des lettres et sciences humaines. Musée arc...
Book
|
Catalogue méthodique des moulages des oeuvres de sculpture grecque; 01/01/1892
Please log in to see more details
Bulletin italien.
Université de Bordeaux. Faculté des lettres.;Université de Bordeaux. Facult...
Revue des lettres françaises et étrangères.
Université de Bordeaux. Faculté des lettres et sciences humaines.;Universit...
Annales de la Faculte des lettres de Bordeaux
Université de Bordeaux. Faculté des lettres.;Université de Bordeaux. Facult...
Bulletin italien.
Université de Bordeaux. Faculté des lettres et sciences humaines.;Univers...
Revue des lettres françaises et étrangères.
Université de Bordeaux. Faculté des lettres et sciences humaines.;Universit...
Revue des universités du Midi.
Université de Bordeaux. Faculté des lettres et sciences humaines.;Universit...
Annales de la Faculte des lettres de Bordeaux
Université de Bordeaux. Faculté des lettres.;Université de Bordeaux. Facult...
Reuniting philosophy and science to advance cancer research.
Academic Journal
|
Publisher: Cambridge University Press Country of Publication: England NLM ID: 0414576 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1469-185X (Electronic) Linking ISSN: 00063231 NLM ISO Abbreviation: Biol Rev Camb Philos Soc Subsets: MEDLINE
Please log in to see more details
Pradeu T;Daignan-Fornier B;Ewald A;Germain PL;Okasha S;Plutynski A;Benzekry...
Academic Journal
|
Publisher: Cambridge University Press Country of Publication: England NLM ID: 0414576 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1469-185X (Electronic) Linking ISSN: 00063231 NLM ISO Abbreviation: Biol Rev Camb Philos Soc Subsets: MEDLINE
Please log in to see more details
Cancers rely on multiple, heterogeneous processes at different scales, pertaining to m...
more
Reuniting philosophy and science to advance cancer research.
Publisher: Cambridge University Press Country of Publication: England NLM ID: 0414576 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1469-185X (Electronic) Linking ISSN: 00063231 NLM ISO Abbreviation: Biol Rev Camb Philos Soc Subsets: MEDLINE
Cancers rely on multiple, heterogeneous processes at different scales, pertaining to many biomedical fields. Therefore, understanding cancer is necessarily an interdisciplinary task that requires placing specialised experimental and clinical research into a broader conceptual, theoretical, and methodological framework. Without such a framework, oncology will collect piecemeal results, with scant dialogue between the different scientific communities studying cancer. We argue that one important way forward in service of a more successful dialogue is through greater integration of applied sciences (experimental and clinical) with conceptual and theoretical approaches, informed by philosophical methods. By way of illustration, we explore six central themes: (i) the role of mutations in cancer; (ii) the clonal evolution of cancer cells; (iii) the relationship between cancer and multicellularity; (iv) the tumour microenvironment; (v) the immune system; and (vi) stem cells. In each case, we examine open questions in the scientific literature through a philosophical methodology and show the benefit of such a synergy for the scientific and medical understanding of cancer.
(© 2023 The Authors. Biological Reviews published by John Wiley & Sons Ltd on behalf of Cambridge Philosophical Society.)
(© 2023 The Authors. Biological Reviews published by John Wiley & Sons Ltd on behalf of Cambridge Philosophical Society.)
Subject terms:
Research - Interdisciplinary Studies - Philosophy - NeoplasmsContent provider:
MEDLINE
Randomized Trial of Vaccines for Zaire Ebola Virus Disease.
Academic Journal
|
Publisher: Massachusetts Medical Society Country of Publication: United States NLM ID: 0255562 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1533-4406 (Electronic) Linking ISSN: 00284793 NLM ISO Abbreviation: N Engl J Med Subsets: MEDLINE
Please log in to see more details
Kieh M;Richert L;Beavogui AH;Grund B;Leigh B;D'Ortenzio E;Doumbia S;Lhomme ...
Academic Journal
|
Publisher: Massachusetts Medical Society Country of Publication: United States NLM ID: 0255562 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1533-4406 (Electronic) Linking ISSN: 00284793 NLM ISO Abbreviation: N Engl J Med Subsets: MEDLINE
Please log in to see more details
Background: Questions remain concerning the rapidity of immune responses and the durab...
more
Randomized Trial of Vaccines for Zaire Ebola Virus Disease.
Publisher: Massachusetts Medical Society Country of Publication: United States NLM ID: 0255562 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1533-4406 (Electronic) Linking ISSN: 00284793 NLM ISO Abbreviation: N Engl J Med Subsets: MEDLINE
Background: Questions remain concerning the rapidity of immune responses and the durability and safety of vaccines used to prevent Zaire Ebola virus disease.
Methods: We conducted two randomized, placebo-controlled trials - one involving adults and one involving children - to evaluate the safety and immune responses of three vaccine regimens against Zaire Ebola virus disease: Ad26.ZEBOV followed by MVA-BN-Filo 56 days later (the Ad26-MVA group), rVSVΔG-ZEBOV-GP followed by placebo 56 days later (the rVSV group), and rVSVΔG-ZEBOV-GP followed by rVSVΔG-ZEBOV-GP 56 days later (the rVSV-booster group). The primary end point was antibody response at 12 months, defined as having both a 12-month antibody concentration of at least 200 enzyme-linked immunosorbent assay units (EU) per milliliter and an increase from baseline in the antibody concentration by at least a factor of 4.
Results: A total of 1400 adults and 1401 children underwent randomization. Among both adults and children, the incidence of injection-site reactions and symptoms (e.g., feverishness and headache) was higher in the week after receipt of the primary and second or booster vaccinations than after receipt of placebo but not at later time points. These events were largely low-grade. At month 12, a total of 41% of adults (titer, 401 EU per milliliter) and 78% of children (titer, 828 EU per milliliter) had a response in the Ad26-MVA group; 76% (titer, 992 EU per milliliter) and 87% (titer, 1415 EU per milliliter), respectively, had a response in the rVSV group; 81% (titer, 1037 EU per milliliter) and 93% (titer, 1745 EU per milliliter), respectively, had a response in the rVSV-booster group; and 3% (titer, 93 EU per milliliter) and 4% (titer, 67 EU per milliliter), respectively, had a response in the placebo group (P<0.001 for all comparisons of vaccine with placebo). In both adults and children, antibody responses with vaccine differed from those with placebo beginning on day 14.
Conclusions: No safety concerns were identified in this trial. With all three vaccine regimens, immune responses were seen from day 14 through month 12. (Funded by the National Institutes of Health and others; PREVAC ClinicalTrials.gov number, NCT02876328; EudraCT numbers, 2017-001798-18 and 2017-001798-18/3rd; and Pan African Clinical Trials Registry number, PACTR201712002760250.).
(Copyright © 2022 Massachusetts Medical Society.)
Methods: We conducted two randomized, placebo-controlled trials - one involving adults and one involving children - to evaluate the safety and immune responses of three vaccine regimens against Zaire Ebola virus disease: Ad26.ZEBOV followed by MVA-BN-Filo 56 days later (the Ad26-MVA group), rVSVΔG-ZEBOV-GP followed by placebo 56 days later (the rVSV group), and rVSVΔG-ZEBOV-GP followed by rVSVΔG-ZEBOV-GP 56 days later (the rVSV-booster group). The primary end point was antibody response at 12 months, defined as having both a 12-month antibody concentration of at least 200 enzyme-linked immunosorbent assay units (EU) per milliliter and an increase from baseline in the antibody concentration by at least a factor of 4.
Results: A total of 1400 adults and 1401 children underwent randomization. Among both adults and children, the incidence of injection-site reactions and symptoms (e.g., feverishness and headache) was higher in the week after receipt of the primary and second or booster vaccinations than after receipt of placebo but not at later time points. These events were largely low-grade. At month 12, a total of 41% of adults (titer, 401 EU per milliliter) and 78% of children (titer, 828 EU per milliliter) had a response in the Ad26-MVA group; 76% (titer, 992 EU per milliliter) and 87% (titer, 1415 EU per milliliter), respectively, had a response in the rVSV group; 81% (titer, 1037 EU per milliliter) and 93% (titer, 1745 EU per milliliter), respectively, had a response in the rVSV-booster group; and 3% (titer, 93 EU per milliliter) and 4% (titer, 67 EU per milliliter), respectively, had a response in the placebo group (P<0.001 for all comparisons of vaccine with placebo). In both adults and children, antibody responses with vaccine differed from those with placebo beginning on day 14.
Conclusions: No safety concerns were identified in this trial. With all three vaccine regimens, immune responses were seen from day 14 through month 12. (Funded by the National Institutes of Health and others; PREVAC ClinicalTrials.gov number, NCT02876328; EudraCT numbers, 2017-001798-18 and 2017-001798-18/3rd; and Pan African Clinical Trials Registry number, PACTR201712002760250.).
(Copyright © 2022 Massachusetts Medical Society.)
Subject terms:
Adult - Child - Humans - Antibodies, Viral - Democratic Republic of the Congo - Ebola Vaccines therapeutic use - Ebolavirus - Hemorrhagic Fever, Ebola prevention & controlContent provider:
MEDLINE
A late Middle Pleistocene Middle Stone Age sequence identified at Wadi Lazalim in southern Tunisia.
Academic Journal
|
Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE
Please log in to see more details
Cancellieri E;Bel Hadj Brahim H;Ben Nasr J;Ben Fraj T;Boussoffara R;Di Matt...
Academic Journal
|
Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE
Please log in to see more details
The late Middle Pleistocene, starting at around 300 ka, witnessed large-scale biologic...
more
A late Middle Pleistocene Middle Stone Age sequence identified at Wadi Lazalim in southern Tunisia.
Publisher: Nature Publishing Group Country of Publication: England NLM ID: 101563288 Publication Model: Electronic Cited Medium: Internet ISSN: 2045-2322 (Electronic) Linking ISSN: 20452322 NLM ISO Abbreviation: Sci Rep Subsets: MEDLINE
The late Middle Pleistocene, starting at around 300 ka, witnessed large-scale biological and cultural dynamics in hominin evolution across Africa including the onset of the Middle Stone Age that is closely associated with the evolution of our species-Homo sapiens. However, archaeological and geochronological data of its earliest appearance are scarce. Here we report on the late Middle Pleistocene sequence of Wadi Lazalim, in the Sahara of Southern Tunisia, which has yielded evidence for human occupations bracketed between ca. 300-130 ka. Wadi Lazalim contributes valuable information on the spread of early MSA technocomplexes across North Africa, that likely were an expression of large-scale diffusion processes.
(© 2022. The Author(s).)
(© 2022. The Author(s).)
Subject terms:
Animals - Archaeology - Biological Evolution - Humans - Tunisia - Fossils - HominidaeContent provider:
MEDLINE
PDF
Full Text
These boots are made for burnin': Inferring the position of the corpse and the presence of leather footwears during cremation through isotope (δ13C, δ18O) and infrared (FTIR) analyses of experimentally burnt skeletal remains.
Academic Journal
|
Publisher: Public Library of Science Country of Publication: United States NLM ID: 101285081 Publication Model: eCollection Cited Medium: Internet ISSN: 1932-6203 (Electronic) Linking ISSN: 19326203 NLM ISO Abbreviation: PLoS One Subsets: MEDLINE
Please log in to see more details
Salesse K;Stamataki E;Kontopoulos I;Verly G;Annaert R;Boudin M;Capuzzo G;Cl...
Academic Journal
|
Publisher: Public Library of Science Country of Publication: United States NLM ID: 101285081 Publication Model: eCollection Cited Medium: Internet ISSN: 1932-6203 (Electronic) Linking ISSN: 19326203 NLM ISO Abbreviation: PLoS One Subsets: MEDLINE
Please log in to see more details
Cremation is a complex mortuary practice, involving a number of activities of the livi...
more
These boots are made for burnin': Inferring the position of the corpse and the presence of leather footwears during cremation through isotope (δ13C, δ18O) and infrared (FTIR) analyses of experimentally burnt skeletal remains.
Publisher: Public Library of Science Country of Publication: United States NLM ID: 101285081 Publication Model: eCollection Cited Medium: Internet ISSN: 1932-6203 (Electronic) Linking ISSN: 19326203 NLM ISO Abbreviation: PLoS One Subsets: MEDLINE
Cremation is a complex mortuary practice, involving a number of activities of the living towards the dead before, during, and after the destruction of the bodily soft tissues by fire. The limiting information concerning these behavioral patterns obtained from the pyre remains and/or cremation deposits prevents the reconstruction of the handling of the corpse during the burning process. This pioneering study tries to determine the initial positioning of the corpse in the pyre and assess whether the deceased was wearing closed leather shoes during cremation through isotopic (δ13C, δ18O) and infrared (ATR-FTIR) analyses of experimentally burnt pig remains, used as a proxy for humans. The results obtained show that both the position of feet on or within the pyre and the presence of footwears may moderately-to-highly influence the oxygen isotope ratios of bone apatite carbonates and the cyanamide content of calcined bone in certain situations. By forming a protective layer, shoes appear to temporarily delay the burning of the underlying pig tissues and to increase the heat-shielding effect of the soft tissues protecting the bone mineral fraction. In such case, bioapatite bone carbonates exchange oxygen with a relatively more 18O-depleted atmosphere (due to the influence of lignin-derived oxygen rather than cellulose-derived oxygen), resulting in more pronounced decrease in the δ18Ocarb values during burning of the shoed feet vs. unshoed feet. The shift observed here was as high as 2.5‰. A concomitant isotopic effect of the initial location of the feet in the pyres was also observed, resulting in a top-to-bottom decrease difference in the δ18Ocarb values of shoed feet of about 1.4‰ between each deposition level tested. Finally, the presence of cyanamide (CN/P ≥ 0.02) seems to be indicative of closed footwear since the latter creates favorable conditions for its incorporation into bone apatite.
Competing Interests: The authors have declared that no competing interests exist.
Competing Interests: The authors have declared that no competing interests exist.
Subject terms:
Animals - Body Remains chemistry - Bone and Bones chemistry - Cadaver - Carbon Isotopes analysis - Humans - Oxygen Isotopes analysis - Shoes - Spectroscopy, Fourier Transform Infrared - Swine - CremationContent provider:
MEDLINE
[The university health service: Role of a mental health nurse].
Academic Journal
|
Publisher: Elsevier Masson Country of Publication: France NLM ID: 1267175 Publication Model: Print-Electronic Cited Medium: Print ISSN: 1293-8505 (Print) Linking ISSN: 12938505 NLM ISO Abbreviation: Rev Infirm
Please log in to see more details
Pougnet R;Rahis AC;Grall B;Le Goff Y
Academic Journal
|
Publisher: Elsevier Masson Country of Publication: France NLM ID: 1267175 Publication Model: Print-Electronic Cited Medium: Print ISSN: 1293-8505 (Print) Linking ISSN: 12938505 NLM ISO Abbreviation: Rev Infirm
Please log in to see more details
University health services have been serving students for many years. During this very...
more
[The university health service: Role of a mental health nurse].
Publisher: Elsevier Masson Country of Publication: France NLM ID: 1267175 Publication Model: Print-Electronic Cited Medium: Print ISSN: 1293-8505 (Print) Linking ISSN: 12938505 NLM ISO Abbreviation: Rev Infirm
University health services have been serving students for many years. During this very particular health crisis, they are actively caring for the health of their population, including psychological health. Nursing skills are essential here.
(Copyright © 2021 Elsevier Masson SAS. All rights reserved.)
(Copyright © 2021 Elsevier Masson SAS. All rights reserved.)
Subject terms:
Humans - Mental Health - Students - Psychiatric Nursing - Student Health Services - Students, NursingContent provider:
MEDLINE
Descriptive analysis of the French NS-Park registry: Towards a nation-wide Parkinson's disease cohort?
Academic Journal
|
Publisher: Elsevier Science Country of Publication: England NLM ID: 9513583 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5126 (Electronic) Linking ISSN: 13538020 NLM ISO Abbreviation: Parkinsonism Relat Disord Subsets: MEDLINE
Please log in to see more details
Mariani LL;Doulazmi M;Chaigneau V;Brefel-Courbon C;Carrière N;Danaila T;Def...
Academic Journal
|
Publisher: Elsevier Science Country of Publication: England NLM ID: 9513583 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5126 (Electronic) Linking ISSN: 13538020 NLM ISO Abbreviation: Parkinsonism Relat Disord Subsets: MEDLINE
Please log in to see more details
Introduction: Parkinson's disease (PD) is the second most common neurodegenerative dis...
more
Descriptive analysis of the French NS-Park registry: Towards a nation-wide Parkinson's disease cohort?
Publisher: Elsevier Science Country of Publication: England NLM ID: 9513583 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5126 (Electronic) Linking ISSN: 13538020 NLM ISO Abbreviation: Parkinsonism Relat Disord Subsets: MEDLINE
Introduction: Parkinson's disease (PD) is the second most common neurodegenerative disorder after Alzheimer's. The French clinical research network for PD (NS-Park) has created a national patient registry to i)report medical activity of Parkinson Expert Centers (PECs) to the Ministry of Health, ii)facilitate PD patients pre-screening for clinical trials, iii) provide a source for pharmaco-epidemiology studies.
Objective: Assess the French Parkinsonian population at a nation-wide level and discover new clinical characteristics.
Methods: In this feasibility study, PECs prospectively collected clinical data in a standardized manner. The population main clinical characteristics are described, focusing on motor and non-motor symptoms and treatments, assessing its representativeness. By using an unbiased clustering with multiple correspondence analysis (MCA), we also investigate potential relationships between multiple variables like symptoms and treatments, as clues for future studies.
Results: Between 2012 and 2016, among 11,157 included parkinsonian syndromes, 9454 (85%) had PD. MCA identified various profiles depending on disease duration. Occurrences of motor complications, axial signs, cognitive disorders and Levodopa use increase over time. Neurovegetative symptoms, psychiatric disorders, sleep disturbances and impulse control disorders (ICDs) seem stable over time. As expected, ICDs were associated to dopaminergic agonist use but other associations, such as ICDs and sleep disturbances for instance, or anxiety and depression, were found.
Conclusions: Our results report one of the biggest PD registries ever reported and demonstrate the feasibility of implementing a nation-wide registry of PD patients in France, a potent tool for future longitudinal studies and clinical trials' population selection, and for pharmaco-epidemiology and cost-effectiveness studies.
(Copyright © 2019 Elsevier Ltd. All rights reserved.)
Objective: Assess the French Parkinsonian population at a nation-wide level and discover new clinical characteristics.
Methods: In this feasibility study, PECs prospectively collected clinical data in a standardized manner. The population main clinical characteristics are described, focusing on motor and non-motor symptoms and treatments, assessing its representativeness. By using an unbiased clustering with multiple correspondence analysis (MCA), we also investigate potential relationships between multiple variables like symptoms and treatments, as clues for future studies.
Results: Between 2012 and 2016, among 11,157 included parkinsonian syndromes, 9454 (85%) had PD. MCA identified various profiles depending on disease duration. Occurrences of motor complications, axial signs, cognitive disorders and Levodopa use increase over time. Neurovegetative symptoms, psychiatric disorders, sleep disturbances and impulse control disorders (ICDs) seem stable over time. As expected, ICDs were associated to dopaminergic agonist use but other associations, such as ICDs and sleep disturbances for instance, or anxiety and depression, were found.
Conclusions: Our results report one of the biggest PD registries ever reported and demonstrate the feasibility of implementing a nation-wide registry of PD patients in France, a potent tool for future longitudinal studies and clinical trials' population selection, and for pharmaco-epidemiology and cost-effectiveness studies.
(Copyright © 2019 Elsevier Ltd. All rights reserved.)
Subject terms:
Adult - Aged - Aged, 80 and over - Cohort Studies - Feasibility Studies - Female - France - Humans - Male - Middle Aged - Parkinson Disease physiopathology - Parkinsonian Disorders physiopathology - RegistriesContent provider:
MEDLINE
Distal extremities soft tissue sarcomas: Are they so different from other limb localizations?
Academic Journal
|
Publisher: Wiley-Liss Country of Publication: United States NLM ID: 0222643 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1096-9098 (Electronic) Linking ISSN: 00224790 NLM ISO Abbreviation: J Surg Oncol Subsets: MEDLINE
Please log in to see more details
Mattei JC;Brouste V;Terrier P;Bonvalot S;Lecesne A;Stoeckle E;Italiano A;Ra...
Academic Journal
|
Publisher: Wiley-Liss Country of Publication: United States NLM ID: 0222643 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1096-9098 (Electronic) Linking ISSN: 00224790 NLM ISO Abbreviation: J Surg Oncol Subsets: MEDLINE
Please log in to see more details
Background and Objectives: Soft tissue sarcoma localization in distal extremities (DES...
more
Distal extremities soft tissue sarcomas: Are they so different from other limb localizations?
Publisher: Wiley-Liss Country of Publication: United States NLM ID: 0222643 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1096-9098 (Electronic) Linking ISSN: 00224790 NLM ISO Abbreviation: J Surg Oncol Subsets: MEDLINE
Background and Objectives: Soft tissue sarcoma localization in distal extremities (DESTS) of the limbs (hand/fingers, and foot/toes) is unusual. The literature is scarce about their behavior and this study was designed to assess their epidemiological characteristics, outcomes, and prognosis compared to other limb localizations (OLSTS).
Methods: From 1980 to 2010, adult DESTS and OLSTS in 22 centers were included. Demographics, tumor type, treatment modalities, and latest follow-up status were collected. Primary endpoints were overall survival and local/metastatic recurrence incidences.
Results: Two hundred five DESTS and 3001 OLSTS were included. The patients were younger, with more female and smaller tumors in DESTS. There were more clear cell/epithelioid sarcomas, synovial sarcomas, and myxoid liposarcomas vs more dedifferentiated liposarcomas in OLSTS. DESTS tumors were less irradiated and more often amputated (24.3% vs 3.4%). The five-year survival rate was 78.2% compared to 68.6% in OLSTS and after multivariate analysis, STS localization did not impact survival or local/metastatic recurrence.
Conclusion: Though rare and smaller than other limb localizations, DESTS are to be considered as aggressive. Despite a higher amputation rate, the prognosis remains the same as in OLSTS. Limb sparing vs amputation should be carefully assessed in DESTS, especially if grade 3 or of a poor prognosis histological subtype.
(© 2019 Wiley Periodicals, Inc.)
Methods: From 1980 to 2010, adult DESTS and OLSTS in 22 centers were included. Demographics, tumor type, treatment modalities, and latest follow-up status were collected. Primary endpoints were overall survival and local/metastatic recurrence incidences.
Results: Two hundred five DESTS and 3001 OLSTS were included. The patients were younger, with more female and smaller tumors in DESTS. There were more clear cell/epithelioid sarcomas, synovial sarcomas, and myxoid liposarcomas vs more dedifferentiated liposarcomas in OLSTS. DESTS tumors were less irradiated and more often amputated (24.3% vs 3.4%). The five-year survival rate was 78.2% compared to 68.6% in OLSTS and after multivariate analysis, STS localization did not impact survival or local/metastatic recurrence.
Conclusion: Though rare and smaller than other limb localizations, DESTS are to be considered as aggressive. Despite a higher amputation rate, the prognosis remains the same as in OLSTS. Limb sparing vs amputation should be carefully assessed in DESTS, especially if grade 3 or of a poor prognosis histological subtype.
(© 2019 Wiley Periodicals, Inc.)
Subject terms:
Adult - Aged - Female - Humans - Incidence - Male - Middle Aged - Neoplasm Metastasis - Neoplasm Recurrence, Local epidemiology - Prognosis - Sarcoma mortality - Sarcoma pathology - Extremities - Sarcoma therapyContent provider:
MEDLINE
Efficacy of Exome-Targeted Capture Sequencing to Detect Mutations in Known Cerebellar Ataxia Genes.
Academic Journal
|
Publisher: American Medical Association Country of Publication: United States NLM ID: 101589536 Publication Model: Print Cited Medium: Internet ISSN: 2168-6157 (Electronic) Linking ISSN: 21686149 NLM ISO Abbreviation: JAMA Neurol Subsets: MEDLINE
Please log in to see more details
Coutelier M;Hammer MB;Stevanin G;Monin ML;Davoine CS;Mochel F;Labauge P;Ewe...
Academic Journal
|
Publisher: American Medical Association Country of Publication: United States NLM ID: 101589536 Publication Model: Print Cited Medium: Internet ISSN: 2168-6157 (Electronic) Linking ISSN: 21686149 NLM ISO Abbreviation: JAMA Neurol Subsets: MEDLINE
Please log in to see more details
Importance: Molecular diagnosis is difficult to achieve in disease groups with a highl...
more
Efficacy of Exome-Targeted Capture Sequencing to Detect Mutations in Known Cerebellar Ataxia Genes.
Publisher: American Medical Association Country of Publication: United States NLM ID: 101589536 Publication Model: Print Cited Medium: Internet ISSN: 2168-6157 (Electronic) Linking ISSN: 21686149 NLM ISO Abbreviation: JAMA Neurol Subsets: MEDLINE
Importance: Molecular diagnosis is difficult to achieve in disease groups with a highly heterogeneous genetic background, such as cerebellar ataxia (CA). In many patients, candidate gene sequencing or focused resequencing arrays do not allow investigators to reach a genetic conclusion.
Objectives: To assess the efficacy of exome-targeted capture sequencing to detect mutations in genes broadly linked to CA in a large cohort of undiagnosed patients and to investigate their prevalence.
Design, Setting, and Participants: Three hundred nineteen index patients with CA and without a history of dominant transmission were included in the this cohort study by the Spastic Paraplegia and Ataxia Network. Centralized storage was in the DNA and cell bank of the Brain and Spine Institute, Salpetriere Hospital, Paris, France. Patients were classified into 6 clinical groups, with the largest being those with spastic ataxia (ie, CA with pyramidal signs [n = 100]). Sequencing was performed from January 1, 2014, through December 31, 2016. Detected variants were classified as very probably or definitely causative, possibly causative, or of unknown significance based on genetic evidence and genotype-phenotype considerations.
Main Outcomes and Measures: Identification of variants in genes broadly linked to CA, classified in pathogenicity groups.
Results: The 319 included patients had equal sex distribution (160 female [50.2%] and 159 male patients [49.8%]; mean [SD] age at onset, 27.9 [18.6] years). The age at onset was younger than 25 years for 131 of 298 patients (44.0%) with complete clinical information. Consanguinity was present in 101 of 298 (33.9%). Very probable or definite diagnoses were achieved for 72 patients (22.6%), with an additional 19 (6.0%) harboring possibly pathogenic variants. The most frequently mutated genes were SPG7 (n = 14), SACS (n = 8), SETX (n = 7), SYNE1 (n = 6), and CACNA1A (n = 6). The highest diagnostic rate was obtained for patients with an autosomal recessive CA with oculomotor apraxia-like phenotype (6 of 17 [35.3%]) or spastic ataxia (35 of 100 [35.0%]) and patients with onset before 25 years of age (41 of 131 [31.3%]). Peculiar phenotypes were reported for patients carrying KCND3 or ERCC5 variants.
Conclusions and Relevance: Exome capture followed by targeted analysis allows the molecular diagnosis in patients with highly heterogeneous mendelian disorders, such as CA, without prior assumption of the inheritance mode or causative gene. Being commonly available without specific design need, this procedure allows testing of a broader range of genes, consequently describing less classic phenotype-genotype correlations, and post hoc reanalysis of data as new genes are implicated in the disease.
Objectives: To assess the efficacy of exome-targeted capture sequencing to detect mutations in genes broadly linked to CA in a large cohort of undiagnosed patients and to investigate their prevalence.
Design, Setting, and Participants: Three hundred nineteen index patients with CA and without a history of dominant transmission were included in the this cohort study by the Spastic Paraplegia and Ataxia Network. Centralized storage was in the DNA and cell bank of the Brain and Spine Institute, Salpetriere Hospital, Paris, France. Patients were classified into 6 clinical groups, with the largest being those with spastic ataxia (ie, CA with pyramidal signs [n = 100]). Sequencing was performed from January 1, 2014, through December 31, 2016. Detected variants were classified as very probably or definitely causative, possibly causative, or of unknown significance based on genetic evidence and genotype-phenotype considerations.
Main Outcomes and Measures: Identification of variants in genes broadly linked to CA, classified in pathogenicity groups.
Results: The 319 included patients had equal sex distribution (160 female [50.2%] and 159 male patients [49.8%]; mean [SD] age at onset, 27.9 [18.6] years). The age at onset was younger than 25 years for 131 of 298 patients (44.0%) with complete clinical information. Consanguinity was present in 101 of 298 (33.9%). Very probable or definite diagnoses were achieved for 72 patients (22.6%), with an additional 19 (6.0%) harboring possibly pathogenic variants. The most frequently mutated genes were SPG7 (n = 14), SACS (n = 8), SETX (n = 7), SYNE1 (n = 6), and CACNA1A (n = 6). The highest diagnostic rate was obtained for patients with an autosomal recessive CA with oculomotor apraxia-like phenotype (6 of 17 [35.3%]) or spastic ataxia (35 of 100 [35.0%]) and patients with onset before 25 years of age (41 of 131 [31.3%]). Peculiar phenotypes were reported for patients carrying KCND3 or ERCC5 variants.
Conclusions and Relevance: Exome capture followed by targeted analysis allows the molecular diagnosis in patients with highly heterogeneous mendelian disorders, such as CA, without prior assumption of the inheritance mode or causative gene. Being commonly available without specific design need, this procedure allows testing of a broader range of genes, consequently describing less classic phenotype-genotype correlations, and post hoc reanalysis of data as new genes are implicated in the disease.
