LIVIVO - Search results -

Search results

Result 1 - 10 of total 11

Search options

Article ; Online: Vivre avec... La maladie de Rendu-Osler.

Guilhem, Alexandre

2023 Volume 73, Issue 5, Page(s) 545–547

Title translation	Living with… Rendu-Osler disease.
MeSH term(s)	Humans ; Telangiectasia, Hereditary Hemorrhagic
Language	French
Publishing date	2023-06-13
Publishing country	France
Document type	Journal Article
ZDB-ID	205365-2
ISSN	2101-017X ; 0035-2640
ISSN (online)	2101-017X
ISSN	0035-2640
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Ua VI Zs.433: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Hereditary hemorrhagic telangiectasia: from signaling insights to therapeutic advances.

Al Tabosh, Tala / Al Tarrass, Mohammad / Tourvieilhe, Laura / Guilhem, Alexandre / Dupuis-Girod, Sophie / Bailly, Sabine

The Journal of clinical investigation

2024 Volume 134, Issue 4

Abstract: Hereditary hemorrhagic telangiectsia (HHT) is an inherited vascular disorder with highly variable expressivity, affecting up to 1 in 5,000 individuals. This disease is characterized by small arteriovenous malformations (AVMs) in mucocutaneous areas ( ... ...

Abstract	Hereditary hemorrhagic telangiectsia (HHT) is an inherited vascular disorder with highly variable expressivity, affecting up to 1 in 5,000 individuals. This disease is characterized by small arteriovenous malformations (AVMs) in mucocutaneous areas (telangiectases) and larger visceral AVMs in the lungs, liver, and brain. HHT is caused by loss-of-function mutations in the BMP9-10/ENG/ALK1/SMAD4 signaling pathway. This Review presents up-to-date insights on this mutated signaling pathway and its crosstalk with proangiogenic pathways, in particular the VEGF pathway, that has allowed the repurposing of new drugs for HHT treatment. However, despite the substantial benefits of these new treatments in terms of alleviating symptom severity, this not-so-uncommon bleeding disorder still currently lacks any FDA- or European Medicines Agency-approved (EMA-approved) therapies.
MeSH term(s)	Humans ; Telangiectasia, Hereditary Hemorrhagic/drug therapy ; Telangiectasia, Hereditary Hemorrhagic/genetics ; Arteriovenous Malformations/metabolism ; Signal Transduction/genetics
Language	English
Publishing date	2024-02-15
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	3067-3
ISSN	1558-8238 ; 0021-9738
ISSN (online)	1558-8238
ISSN	0021-9738
DOI	10.1172/JCI176379
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Ua VI Zs.184: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Altered expressions of CXCR4 and CD26 on T-helper lymphocytes in hereditary hemorrhagic telangiectasia.

Guilhem, Alexandre / Portalès, Pierre / Dupuis-Girod, Sophie / Rivière, Sophie / Vincent, Thierry

Orphanet journal of rare diseases

2021 Volume 16, Issue 1, Page(s) 511

Abstract: Background: Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disease characterized by a deregulated neo-angiogenesis. Besides a mainly vascular phenotype (muco-cutaneous telangiectases, arteriovenous malformations), a specific risk of ... ...

Abstract	Background: Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disease characterized by a deregulated neo-angiogenesis. Besides a mainly vascular phenotype (muco-cutaneous telangiectases, arteriovenous malformations), a specific risk of infection is suggested by case series of severe and atypical infections as well as by reports of decreased T and natural killer (NK) lymphocyte counts. As some evidence supports a dysregulation of the CXCR4/CXCL12 chemotactic axis of HHT endothelial cells, we hypothesized that a similar phenomenon could occur on lymphocytes. Methods: Eighteen HHT patients with history of severe infection (HSI) were matched in age and sex with 18 HHT without HSI and 18 healthy control subjects (HC). We assessed the cell count and the surface expression of CXCR4 and CD26 (CXCL12 inactivating peptidase) of circulating T-helper and T-cytotoxic lymphocytes (including naive, memory and activated subsets) and NK cells. Results: The overall HHT group of 36 patients exhibited a reduction of circulating T-helper lymphocytes compared to HC (median: 517 vs. 1026 cells/mm Conclusions: Our findings support a dysregulation of the CXCL12/CXCR4 chemotaxis of T-helper lymphocytes in HHT patients, potentially linked to their T-helper lymphopenia and susceptibility to infection.
MeSH term(s)	Dipeptidyl Peptidase 4/metabolism ; Endothelial Cells ; Humans ; Killer Cells, Natural/metabolism ; Receptors, CXCR4 ; T-Lymphocytes, Helper-Inducer/metabolism ; Telangiectasia, Hereditary Hemorrhagic/epidemiology
Chemical Substances	CXCR4 protein, human ; Receptors, CXCR4 ; DPP4 protein, human (EC 3.4.14.5) ; Dipeptidyl Peptidase 4 (EC 3.4.14.5)
Language	English
Publishing date	2021-12-14
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2225857-7
ISSN	1750-1172 ; 1750-1172
ISSN (online)	1750-1172
ISSN	1750-1172
DOI	10.1186/s13023-021-02139-y
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article ; Online: Pro-angiogenic changes of T-helper lymphocytes in hereditary hemorrhagic telangiectasia.

Guilhem, Alexandre / Ciudad, Marion / Aubriot-Lorton, Marie-Hélène / Greigert, Hélène / Cladière, Claudie / Leguy-Seguin, Vanessa / Audia, Sylvain / Samson, Maxime / Bonnotte, Bernard

Frontiers in immunology

2023 Volume 14, Page(s) 1321182

Abstract: Hereditary hemorrhagic telangiectasia (HHT) is a rare inherited disease due to heterozygous loss-of-function mutations on the BMP9/10 pathway ( ...

Abstract	Hereditary hemorrhagic telangiectasia (HHT) is a rare inherited disease due to heterozygous loss-of-function mutations on the BMP9/10 pathway (
MeSH term(s)	Humans ; Telangiectasia, Hereditary Hemorrhagic/genetics ; Epistaxis/complications ; Endothelial Cells ; Vascular Endothelial Growth Factor A ; Telangiectasis/complications ; T-Lymphocytes, Helper-Inducer ; Activin Receptors, Type II
Chemical Substances	Vascular Endothelial Growth Factor A ; ACVRL1 protein, human (EC 2.7.11.30) ; Activin Receptors, Type II (EC 2.7.11.30)
Language	English
Publishing date	2023-12-08
Publishing country	Switzerland
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2023.1321182
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article ; Online: Seven cases of hereditary haemorrhagic telangiectasia-like hepatic vascular abnormalities associated with

Guilhem, Alexandre / Dupuis-Girod, Sophie / Espitia, Olivier / Rivière, Sophie / Seguier, Julie / Kerjouan, Mallorie / Lavigne, Christian / Maillard, Hélène / Magro, Pascal / Alric, Laurent / Lipsker, Dan / Parrot, Antoine / Leguy, Vanessa / Vanlemmens, Claire / Guibaud, Laurent / Vikkula, Miikka / Eyries, Melanie / Valette, Pierre-Jean / Giraud, Sophie

Journal of medical genetics

2023 Volume 60, Issue 9, Page(s) 905–909

Abstract: Background: EPHB4: Methods: Members of the French HHT network reported their cases of : Results: Among 21 patients with : Conclusion: ... ...

Abstract	Background: EPHB4 Methods: Members of the French HHT network reported their cases of Results: Among 21 patients with Conclusion: EPHB4
MeSH term(s)	Male ; Humans ; Female ; Telangiectasia, Hereditary Hemorrhagic/complications ; Telangiectasia, Hereditary Hemorrhagic/diagnosis ; Telangiectasia, Hereditary Hemorrhagic/genetics ; Epistaxis/complications ; Liver ; Intracranial Arteriovenous Malformations ; Mutation
Language	English
Publishing date	2023-02-22
Publishing country	England
Document type	Journal Article
ZDB-ID	220881-7
ISSN	1468-6244 ; 0022-2593
ISSN (online)	1468-6244
ISSN	0022-2593
DOI	10.1136/jmg-2022-109107
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 177: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: T-cell immune response predicts the risk of critical SARS-Cov2 infection in hospitalized COVID-19 patients.

Samson, Maxime / Nicolas, Barbara / Ciudad, Marion / Greigert, Hélène / Guilhem, Alexandre / Cladiere, Claudie / Straub, Cécile / Blot, Mathieu / Piroth, Lionel / Rogier, Thomas / Devilliers, Hervé / Manckoundia, Patrick / Ghesquiere, Thibault / Francois, Stéphanie / Lakomy, Daniela / Audia, Sylvain / Bonnotte, Bernard

European journal of internal medicine

2022 Volume 102, Page(s) 104–109

Abstract: Introduction: This study aimed to identify markers of disease worsening in patients hospitalized for SARS-Cov2 infection.: Patients and methods: Patients hospitalized for severe recent-onset (<1 week) SARS-Cov2 infection were prospectively included. ... ...

Abstract	Introduction: This study aimed to identify markers of disease worsening in patients hospitalized for SARS-Cov2 infection. Patients and methods: Patients hospitalized for severe recent-onset (<1 week) SARS-Cov2 infection were prospectively included. The percentage of T-cell subsets and plasma IL-6 at admission (before any steroid therapy) were compared between patients who progressed to a critical infection and those who did not. Results: Thirty-seven patients (18 men, 19 women) were included; 11 (30%) progressed to critical infection. At admission, the critical infection patients were older (P = 0.021), had higher creatinine levels (P = 0.003), and decreased percentages of circulating B cells (P = 0.04), T cells (P = 0.009), and CD4+ T cells (P = 0.004) than those with a favorable course. Among T cell subsets, there was no significant difference between the two groups except for the percentage of Th17 cells, which was two-fold higher in patients who progressed to critical infection (P = 0.028). Plasma IL-6 at admission was also higher in this group (P = 0.018). In multivariate analysis, the percentage of circulating Th17 cells at admission was the only variable associated with higher risk of progression to critical SARS-Cov2 infection (P = 0.021). Conclusion: This study suggests that an elevated percentage of Th17 cells in patients hospitalized for SARS-Cov2 infection is associated with an increased risk of progression to critical disease. If these data are confirmed in a larger study, this marker could be used to better target the population of patients in whom tocilizumab could decrease the risk of progression to critical COVID-19.
MeSH term(s)	COVID-19 ; Female ; Humans ; Immunity ; Interleukin-6 ; Male ; RNA, Viral ; SARS-CoV-2 ; T-Lymphocytes
Chemical Substances	Interleukin-6 ; RNA, Viral
Language	English
Publishing date	2022-06-03
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	1038679-8
ISSN	1879-0828 ; 0953-6205
ISSN (online)	1879-0828
ISSN	0953-6205
DOI	10.1016/j.ejim.2022.06.001
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Zs.A 2608: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article ; Online: Antiplatelet and anticoagulant therapies in hereditary hemorrhagic telangiectasia: A large French cohort study (RETROPLACOTEL).

Grobost, Vincent / Hammi, Sami / Pereira, Bruno / Guilhem, Alexandre / Duffau, Pierre / Seguier, Julie / Parrot, Antoine / Gautier, Giovanni / Alric, Laurent / Kerjouan, Mallorie / Le Guillou, Xavier / Simon, Delphine / Chaussavoine, Laurent / Rondeau-Lutz, Murielle / Leguy-Seguin, Vanessa / Delagrange, Laura / Lavigne, Christian / Maillard, Hélène / Dupuis-Girod, Sophie

Thrombosis research

2023 Volume 229, Page(s) 107–113

Abstract: Background: It is unclear whether hereditary hemorrhagic telangiectasia (HHT) patients can tolerate antithrombotic therapies (AT) including antiplatelet (AP) and/or anticoagulant (AC) agents.: Objectives: Primary endpoint was tolerance to AT in HHT. ... ...

Abstract	Background: It is unclear whether hereditary hemorrhagic telangiectasia (HHT) patients can tolerate antithrombotic therapies (AT) including antiplatelet (AP) and/or anticoagulant (AC) agents. Objectives: Primary endpoint was tolerance to AT in HHT. Secondary endpoints were to identify factors associated with major bleeding events (MBE) and premature discontinuation of AT. Methods: Retrospective multicenter study in French national HHT Registry patients exposed to AT. Results: We included 126 patients with 180 courses of AT. Median follow-up was 24 [11-52] months. Mean age was 65.6 ± 13.1 years. The first 3 months of AT exposure had an increased risk of hospitalization for hemorrhage (p < 0.001) and transfusions (p < 0.001). MBE (n = 63) occurred more frequently in the first 3 months of AT exposure (p < 0.001). Premature discontinuation of AT occurred in 61 cases. Rate of premature discontinuation was 29 % under both AP and AT therapy but significantly higher under dual AP therapy (n = 4/7, 57 % p = 0.008). Risk factors for MBE were: age ≥ 60 years (HR 2.34 [1.12;4.87], p = 0.023), prior hospitalization in the 3 months before starting AT for hemorrhage (HR 3.59 [1.93;6.66], p < 0.001) or transfusion (HR 3.15 [1.61;6.18], p = 0.001), previous history of gastro-intestinal bleeding (HR 2.71 [1.57;4.65], p < 0.001) or MBE (HR 4.62 [2.68;7.98], p < 0.001). Frequency of MBE did not differ between groups except for a higher risk in the dual AP group (HR 3.92 [1.37;11.22], p = 0.011). Conclusion: Tolerance of AC or AP therapy was similar in HHT population but not dual AP therapy. We identified risk factors for MBE occurrence or premature discontinuation under AT.
MeSH term(s)	Humans ; Middle Aged ; Aged ; Cohort Studies ; Telangiectasia, Hereditary Hemorrhagic/complications ; Telangiectasia, Hereditary Hemorrhagic/drug therapy ; Anticoagulants/therapeutic use ; Gastrointestinal Hemorrhage/chemically induced ; Retrospective Studies
Chemical Substances	Anticoagulants
Language	English
Publishing date	2023-07-06
Publishing country	United States
Document type	Multicenter Study ; Journal Article
ZDB-ID	121852-9
ISSN	1879-2472 ; 0049-3848
ISSN (online)	1879-2472
ISSN	0049-3848
DOI	10.1016/j.thromres.2023.07.001
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Zs.A 863: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article ; Online: Correction to: The dysregulated innate immune response in severe COVID-19 pneumonia that could drive poorer outcome.

Blot, Mathieu / Bour, Jean-Baptiste / Quenot, Jean Pierre / Bourredjem, Abderrahmane / Nguyen, Maxime / Guy, Julien / Monier, Serge / Georges, Marjolaine / Large, Audrey / Dargent, Auguste / Guilhem, Alexandre / Mouries-Martin, Suzanne / Barben, Jeremy / Bouhemad, Belaid / Charles, Pierre-Emmanuel / Chavanet, Pascal / Binquet, Christine / Piroth, Lionel

Journal of translational medicine

2021 Volume 19, Issue 1, Page(s) 100

Language	English
Publishing date	2021-03-08
Publishing country	England
Document type	Published Erratum
ISSN	1479-5876
ISSN (online)	1479-5876
DOI	10.1186/s12967-021-02746-0
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Inter-library loan at ZB MED

Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

Article ; Online: Efficacy and safety of dapsone as second line therapy for adult immune thrombocytopenia: A retrospective study of 42 patients.

Estève, Clémentine / Samson, Maxime / Guilhem, Alexandre / Nicolas, Barbara / Leguy-Seguin, Vanessa / Berthier, Sabine / Bonnotte, Bernard / Audia, Sylvain

PloS one

2017 Volume 12, Issue 10, Page(s) e0187296

Abstract: Dapsone is recommended as a second line therapy in immune thrombocytopenia (ITP), but is underused because of its potential side effects. The medical charts of 42 ITP patients treated with dapsone (100 mg/day) were retrospectively reviewed in order to ... ...

Abstract	Dapsone is recommended as a second line therapy in immune thrombocytopenia (ITP), but is underused because of its potential side effects. The medical charts of 42 ITP patients treated with dapsone (100 mg/day) were retrospectively reviewed in order to assess its efficacy and safety in daily clinical practice. The overall response rate was 54.8% (n = 22, with a complete response in 38.1%) with a median time to response of 29 days (24-41 days). Patients with complete response had shorter disease duration whereas no difference was observed between responders and non-responders regarding age, sex or previous treatments received. Importantly, after dapsone withdrawal, a sustained response was observed in 5 patients, representing 12% of the whole cohort. Twenty percent of patients (n = 8) relapsed on therapy after 8.1 (6.5-13.6) months. Side effects occurred in 31% (n = 13) of patients, and required dapsone withdrawal in 22% (n = 9) or dosage reduction in 10% (n = 4) of the cases. Side effects resolved in all but one case. Overall, these data support dapsone as an interesting second line therapy in ITP, with a good safety and efficacy profile at a low cost.
Language	English
Publishing date	2017
Publishing country	United States
Document type	Journal Article
ISSN	1932-6203
ISSN (online)	1932-6203
DOI	10.1371/journal.pone.0187296
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Inter-library loan at ZB MED

Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

Article ; Online: The dysregulated innate immune response in severe COVID-19 pneumonia that could drive poorer outcome.

Journal of translational medicine

2020 Volume 18, Issue 1, Page(s) 457

Abstract: Background: Although immune modulation is a promising therapeutic avenue in coronavirus disease 2019 (COVID-19), the most relevant targets remain to be found. COVID-19 has peculiar characteristics and outcomes, suggesting a unique immunopathogenesis.: ...

Abstract	Background: Although immune modulation is a promising therapeutic avenue in coronavirus disease 2019 (COVID-19), the most relevant targets remain to be found. COVID-19 has peculiar characteristics and outcomes, suggesting a unique immunopathogenesis. Methods: Thirty-six immunocompetent non-COVID-19 and 27 COVID-19 patients with severe pneumonia were prospectively enrolled in a single center, most requiring intensive care. Clinical and biological characteristics (including T cell phenotype and function and plasma concentrations of 30 cytokines) and outcomes were compared. Results: At similar baseline respiratory severity, COVID-19 patients required mechanical ventilation for significantly longer than non-COVID-19 patients (15 [7-22] vs. 4 (0-15) days; p = 0.0049). COVID-19 patients had lower levels of most classical inflammatory cytokines (G-CSF, CCL20, IL-1β, IL-2, IL-6, IL-8, IL-15, TNF-α, TGF-β), but higher plasma concentrations of CXCL10, GM-CSF and CCL5, compared to non-COVID-19 patients. COVID-19 patients displayed similar T-cell exhaustion to non-COVID-19 patients, but with a more unbalanced inflammatory/anti-inflammatory cytokine response (IL-6/IL-10 and TNF-α/IL-10 ratios). Principal component analysis identified two main patterns, with a clear distinction between non-COVID-19 and COVID-19 patients. Multivariate regression analysis confirmed that GM-CSF, CXCL10 and IL-10 levels were independently associated with the duration of mechanical ventilation. Conclusion: We identified a unique cytokine response, with higher plasma GM-CSF and CXCL10 in COVID-19 patients that were independently associated with the longer duration of mechanical ventilation. These cytokines could represent the dysregulated immune response in severe COVID-19, as well as promising therapeutic targets. ClinicalTrials.gov: NCT03505281.
MeSH term(s)	Aged ; Aged, 80 and over ; COVID-19/diagnosis ; COVID-19/immunology ; COVID-19/mortality ; COVID-19/therapy ; Critical Care ; Female ; France/epidemiology ; Humans ; Immunity, Innate/physiology ; Immunophenotyping ; Lymphocyte Activation/physiology ; Male ; Middle Aged ; Pneumonia, Viral/diagnosis ; Pneumonia, Viral/immunology ; Pneumonia, Viral/mortality ; Pneumonia, Viral/therapy ; Prognosis ; Respiration, Artificial ; SARS-CoV-2/physiology ; Severity of Illness Index
Language	English
Publishing date	2020-12-03
Publishing country	England
Document type	Controlled Clinical Trial ; Journal Article ; Research Support, Non-U.S. Gov't
ISSN	1479-5876
ISSN (online)	1479-5876
DOI	10.1186/s12967-020-02646-9
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Inter-library loan at ZB MED

Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

To top

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

Order via subito

More links

Kategorien

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

Order via subito

Inter-library loan at ZB MED

More links

Kategorien

Order via subito

Inter-library loan at ZB MED

More links

Kategorien

Order via subito

Inter-library loan at ZB MED