LIVIVO - Search results -

Search results

Result 1 - 10 of total 11

Search options

Article ; Online: Multiple Myeloma With a "Normal" Erythrocyte Sedimentation Rate.

Thomas, Benoît / Revuz, Sabine

The American journal of the medical sciences

2020 Volume 361, Issue 6, Page(s) e53–e54

MeSH term(s)	Blood Sedimentation ; Erythrocytes/metabolism ; Fatal Outcome ; Humans ; Male ; Middle Aged ; Multiple Myeloma/blood ; Multiple Myeloma/diagnostic imaging
Language	English
Publishing date	2020-11-09
Publishing country	United States
Document type	Case Reports ; Letter
ZDB-ID	82078-7
ISSN	1538-2990 ; 0002-9629
ISSN (online)	1538-2990
ISSN	0002-9629
DOI	10.1016/j.amjms.2020.11.006
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Ua VI Zs.2/10: 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: Thrombotic Thrombocytopenic Purpura Without Schistocytes: Beware of Misdiagnosis.

Decker, Paul / Moulinet, Thomas / Revuz, Sabine / Perez, Pierre / Jaussaud, Roland

Neurology. Clinical practice

2019 Volume 11, Issue 5, Page(s) e798–e800

Language	English
Publishing date	2019-12-06
Publishing country	United States
Document type	Case Reports
ZDB-ID	2645818-4
ISSN	2163-0933 ; 2163-0402
ISSN (online)	2163-0933
ISSN	2163-0402
DOI	10.1212/CPJ.0000000000001067
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article ; Online: Immune Thrombocytopenic Purpura in Patients with COVID-19.

Revuz, Sabine / Vernier, Nathalie / Saadi, Leilah / Campagne, Julien / Poussing, Sophie / Maurier, François

European journal of case reports in internal medicine

2020 Volume 7, Issue 7, Page(s) 1751

Abstract: We described three COVID-19-infected patients with profound immune thrombocytopenia causing haemorrhagic mucocutaneous complications. We conclude that an immune mechanism was responsible as common causes were excluded. Since corticoids were considered ... ...

Abstract	We described three COVID-19-infected patients with profound immune thrombocytopenia causing haemorrhagic mucocutaneous complications. We conclude that an immune mechanism was responsible as common causes were excluded. Since corticoids were considered harmful in the circumstances, the patients were successfully treated with intravenous immunoglobulins without later relapse. Learning points: The severity of haemorrhagic syndrome is not correlated with the severity of COVID-19 infection.Thrombocytopenia in mild COVID-19 infection seems to have an autoimmune mechanism.Intravenous immunoglobulins (1 g/kg) should be the first line of treatment.
Keywords	covid19
Language	English
Publishing date	2020-06-09
Publishing country	Italy
Document type	Journal Article
ISSN	2284-2594
ISSN (online)	2284-2594
DOI	10.12890/2020_001751
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: Immune Thrombocytopenic Purpura in Patients with COVID-19

Sabine Revuz / Nathalie Vernier / Leilah Saadi / Julien Campagne / Sophie Poussing / François Maurier

European Journal of Case Reports in Internal Medicine (2020)

2020

Abstract	We described three COVID-19-infected patients with profound immune thrombocytopenia causing haemorrhagic mucocutaneous complications. We conclude that an immune mechanism was responsible as common causes were excluded. Since corticoids were considered harmful in the circumstances, the patients were successfully treated with intravenous immunoglobulins without later relapse.
Keywords	covid-19 ; sars-cov-2 ; thrombocytopenia ; intravenous immunoglobulins ; Medicine ; R ; covid19
Language	English
Publishing date	2020-06-01T00:00:00Z
Publisher	SMC MEDIA SRL
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

Full text online

Full text

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: Interstitial pneumonia with autoimmune features: Evaluation of connective tissue disease incidence during follow-up.

Decker, Paul / Sobanski, Vincent / Moulinet, Thomas / Launay, David / Hachulla, Eric / Valentin, Victor / Godbert, Benoit / Revuz, Sabine / Guillaumot, Anne / Gomez, Emmanuel / Chabot, François / Wémeau, Lidwine / Jaussaud, Roland

European journal of internal medicine

2021 Volume 97, Page(s) 62–68

Abstract: Objectives: Among interstitial pneumonia with autoimmune features (IPAF) patients, identifying those at risk to develop a connective tissue disease (CTD) during the disease course is a key issue. The aim of this study was to evaluate the incidence of ... ...

Abstract	Objectives: Among interstitial pneumonia with autoimmune features (IPAF) patients, identifying those at risk to develop a connective tissue disease (CTD) during the disease course is a key issue. The aim of this study was to evaluate the incidence of definite CTD diagnosis in IPAF patients during follow-up. Methods: We performed a multicentric cohort study of interstitial lung disease (ILD) from 2010 to 2017 in pneumology and immunology departments of tertiary care centers. Patients with a known cause of ILD (including established CTD) at diagnosis were excluded. Among patients with idiopathic ILD and at least three years of follow-up, two groups (IPAF and non-IPAF) were retrospectively analyzed at time of diagnosis. Results: A total of 249 patients with ILD were enrolled, including 70 IPAF and 179 non-IPAF patients. After a mean follow-up time of 77 ± 44 months, 18/70 IPAF patients (26%) had a CTD diagnosis - 9 antisynthetase syndrome, 8 systemic sclerosis and 1 overlap myositis - compared with 4/179 non-IPAF patients (2%). IPAF patients were at higher risk of CTD occurrence at 3 years of follow-up compared to non-IPAF patients (HR 10.1, 95% CI 3.1-33.1, p < 0. 01). IPAF patients progressing to CTD tended to be younger, more often female and have more frequently puffy fingers, capillaroscopy abnormalities and antisynthetase antibodies at diagnosis. Conclusions: We found that a significant proportion of IPAF patients had associated CTD diagnosis during follow-up. Prospective studies are needed to confirm baseline predictive factors of CTD occurrence in IPAF patients.
MeSH term(s)	Cohort Studies ; Connective Tissue Diseases/complications ; Connective Tissue Diseases/epidemiology ; Female ; Follow-Up Studies ; Humans ; Incidence ; Lung Diseases, Interstitial/epidemiology ; Retrospective Studies
Language	English
Publishing date	2021-12-23
Publishing country	Netherlands
Document type	Journal Article ; Multicenter Study
ZDB-ID	1038679-8
ISSN	1879-0828 ; 0953-6205
ISSN (online)	1879-0828
ISSN	0953-6205
DOI	10.1016/j.ejim.2021.12.021
Database	MEDical Literature Analysis and Retrieval System OnLINE

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 ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Clinical significance of anti-Ro52 (TRIM21) antibodies in adult patients with connective tissue diseases.

Decker, Paul / Moulinet, Thomas / Lopez, Benjamin / Dubucquoi, Sylvain / Bonnotte, Bernard / Lakomy, Daniela / Revuz, Sabine / Luc, Amandine / Bittencourt, Marcelo De Carvalho / Hachulla, Eric / Jaussaud, Roland

European journal of internal medicine

2021 Volume 91, Page(s) 45–52

Abstract: Objectives: Clinical significance of anti-Ro52 antibodies in connective tissue diseases (CTD) is controversial. Anti-Ro52 antibodies might be associated with a more severe CTD phenotype, especially interstitial lung disease (ILD). The aims of this study ...

Abstract	Objectives: Clinical significance of anti-Ro52 antibodies in connective tissue diseases (CTD) is controversial. Anti-Ro52 antibodies might be associated with a more severe CTD phenotype, especially interstitial lung disease (ILD). The aims of this study were to evaluate ILD prevalence and severity, the prevalence of micro- or macroangiopathy and CTD-associated cancers in CTD with anti-Ro52 antibodies. Methods: CTD patients with anti-Ro52 antibody screening by immunoblot at diagnosis were enrolled. Two groups were retrospectively formed according to the presence of anti-Ro52 antibodies with an unbiased 1:1 matching on CTD types. Unsupervised multiple correspondence analysis and hierarchical clustering analysis were used to aggregate anti-Ro52 positive patients in subgroups. Results: 408 CTD patients were included. Anti-Ro52 antibodies were detected in 33 % of CTD patients. Anti-Ro52 antibodies were associated with ILD at CTD diagnosis (47.8% vs. 23.0%, OR 3.3 95% IC 1.4 to 8.0, p = 0.008), even after adjusting for the presence of anti-Ro60 antibodies, especially in patients with antisynthetase syndrome, primary Sjögren syndrome and systemic sclerosis. Micro- or macroangiopathy was more frequent in anti-Ro52 positive CTD patients (18.6% vs. 9.7%, p = 0.02) and CTD patients with anti-Ro52 antibodies experienced more frequent relapses and required more immunosuppressive drugs. Clusters 4 and 5 identified anti-Ro52 positive CTD patients with severe ILD and with clinical features of systemic sclerosis or antisynthetase syndrome respectively. Conclusions: We found that anti-Ro52 antibodies were independently associated with ILD in CTD patients irrespective of CTD type. Anti-Ro52 antibodies could be associated with severity and a more relapsing disease course in CTD patients.
MeSH term(s)	Adult ; Autoantibodies ; Connective Tissue Diseases/complications ; Connective Tissue Diseases/epidemiology ; Humans ; Myositis/complications ; Myositis/epidemiology ; Retrospective Studies ; Ribonucleoproteins
Chemical Substances	Autoantibodies ; Ribonucleoproteins
Language	English
Publishing date	2021-05-08
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.2021.04.020
Database	MEDical Literature Analysis and Retrieval System OnLINE

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 ▾
- See ZB MED holdings
- Order with fees

Article ; Online: ANCA-associated scleritis: impact of ANCA on presentation, response to therapy and outcome.

Perray, Laura / Nguyen, Yann / Clavel Refregiers, Gaëlle / Chazal, Thibaud / Héron, Emmanuel / Pouchelon, Clara / Dunogué, Bertrand / Costedoat-Chalumeau, Nathalie / Murarasu, Anne / Régent, Alexis / Puéchal, Xavier / Thoreau, Benjamin / Lifermann, François / Graveleau, Julie / Hié, Miguel / Froissart, Antoine / Baudet, Antoine / Deroux, Alban / Lavigne, Christian /

Puigrenier, Sébastien / Mesbah, Rafik / Moulinet, Thomas / Vasco, Claire / Revuz, Sabine / Pugnet, Grégory / Rieu, Virginie / Combes, Anaïs / Brézin, Antoine / Terrier, Benjamin

Rheumatology (Oxford, England)

2023 Volume 63, Issue 2, Page(s) 329–337

Abstract: Objectives: To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests.: Methods: This retrospective multicentre case-control study was ... ...

Abstract	Objectives: To describe the characteristics, treatment and outcome of isolated ANCA-associated scleritis at diagnosis compared with idiopathic scleritis with negative ANCA tests. Methods: This retrospective multicentre case-control study was performed within the French Vasculitis Study Group (FVSG) network and in three French tertiary ophthalmologic centres. Data from patients with scleritis without any systemic manifestation and with positive ANCA results were compared with those of a control group of patients with idiopathic scleritis with negative ANCA tests. Results: A total of 120 patients, including 38 patients with ANCA-associated scleritis and 82 control patients, diagnosed between January 2007 and April 2022 were included. The median follow-up was 28 months (IQR 10-60). The median age at diagnosis was 48 years (IQR 33-60) and 75% were females. Scleromalacia was more frequent in ANCA-positive patients (P = 0.027) and 54% had associated ophthalmologic manifestations, without significant differences. ANCA-associated scleritis more frequently required systemic medications, including glucocorticoids (76% vs 34%; P < 0.001), and rituximab (P = 0.03) and had a lower remission rate after the first- and second-line treatment. Systemic ANCA-associated vasculitis (AAV) occurred in 30.7% of patients with PR3- or MPO-ANCA, after a median interval of 30 months (IQR 16.3-44). Increased CRP >5 mg/l at diagnosis was the only significant risk factor of progression to systemic AAV [adjusted hazard ratio 5.85 (95% CI 1.10, 31.01), P = 0.038]. Conclusion: Isolated ANCA-associated scleritis is mostly anterior scleritis with a higher risk of scleromalacia than ANCA-negative idiopathic scleritis and is more often difficult to treat. One-third of patients with PR3- or MPO-ANCA scleritis progressed to systemic AAV.
MeSH term(s)	Female ; Humans ; Male ; Antibodies, Antineutrophil Cytoplasmic ; Scleritis/diagnosis ; Scleritis/drug therapy ; Scleritis/etiology ; Case-Control Studies ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis ; Rituximab/therapeutic use ; Retrospective Studies ; Peroxidase ; Myeloblastin
Chemical Substances	Antibodies, Antineutrophil Cytoplasmic ; Rituximab (4F4X42SYQ6) ; Peroxidase (EC 1.11.1.7) ; Myeloblastin (EC 3.4.21.76)
Language	English
Publishing date	2023-05-17
Publishing country	England
Document type	Multicenter Study ; Journal Article
ZDB-ID	1464822-2
ISSN	1462-0332 ; 1462-0324
ISSN (online)	1462-0332
ISSN	1462-0324
DOI	10.1093/rheumatology/kead252
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.B 240: 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)
Zs.MO 497: Show issues

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Absence of Anti-Glomerular Basement Membrane Antibodies in 200 Patients With Systemic Lupus Erythematosus With or Without Lupus Nephritis: Results of the GOODLUPUS Study.

Bourse Chalvon, Nellie / Orquevaux, Pauline / Giusti, Delphine / Gatouillat, Gregory / Tabary, Thierry / Tonye Libyh, Marcelle / Chrusciel, Jan / Drame, Moustapha / Stockton-Bliard, Grace / Amoura, Zahir / Arnaud, Laurent / Lorenz, Hanns-Martin / Blaison, Gilles / Bonnotte, Bernard / Magy-Bertrand, Nadine / Revuz, Sabine / Voll, Reinhard Edmund / Hinschberger, Oliver / Schwarting, Andreas /

Pham, Bach Nga / Martin, Thierry / Pennaforte, Jean-Loup / Servettaz, Amelie

Frontiers in immunology

2020 Volume 11, Page(s) 597863

Abstract: Introduction: Anti-glomerular basement membrane (GBM) antibodies are pathogenic antibodies first detected in renal-limited anti-GBM disease and in Goodpasture disease, the latter characterized by rapidly progressive crescentic glomerulonephritis ... ...

Abstract	Introduction: Anti-glomerular basement membrane (GBM) antibodies are pathogenic antibodies first detected in renal-limited anti-GBM disease and in Goodpasture disease, the latter characterized by rapidly progressive crescentic glomerulonephritis combined with intra-alveolar hemorrhage. Studies have suggested that anti-GBM antibody positivity may be of interest in lupus nephritis (LN). Moreover, severe anti-GBM vasculitis cases in patients with systemic lupus erythematosus (SLE) have been described in the literature, but few studies have assessed the incidence of anti-GBM antibodies in SLE patients. Objective: The main study objective was to determine if positive anti-GBM antibodies were present in the serum of SLE patients with or without proliferative renal damage and compared to a healthy control group. Methodology: This retrospective study was performed on SLE patients' sera from a Franco-German European biobank, developed between 2011 and 2014, from 17 hospital centers in the Haut-Rhin region. Patients were selected according to their renal involvement, and matched by age and gender. The serum from healthy voluntary blood donors was also tested. Anti-GBM were screened by fluorescence enzyme immunoassay (FEIA), and then by indirect immunofluorescence (IIF) in case of low reactivity detection (titer >6 U/ml). Results: The cohort was composed of 100 SLE patients with proliferative LN (27% with class III, 67% with class IV, and 6% with class V), compared to 100 SLE patients without LN and 100 controls. Patients were mostly Caucasian and met the ACR 1997 criteria and/or the SLICC 2012 criteria. Among the 300 tested sera, no significant levels of anti-GBM antibodies were detected (>10 U/ml) by the automated technique, three sera were found "ambivalent" (>7 U/ml): one in the SLE with LN group and two in the SLE without LN group. Subsequent IIF assays did not detect anti-GBM antibodies. Conclusion: Anti-GBM antibodies were not detected in the serum of Caucasian patients with SLE, even in case of renal involvement, a situation favoring the antigenic exposure of glomerular basement membranes. Our results reaffirm the central role of anti-GBM antibodies as a specific diagnostic biomarker for Goodpasture vasculitis and therefore confirm that anti-GBM antibody must not be carried out in patients with SLE (with or without LN) in the absence of disease-suggestive symptoms.
MeSH term(s)	Adult ; Anti-Glomerular Basement Membrane Disease/blood ; Anti-Glomerular Basement Membrane Disease/epidemiology ; Anti-Glomerular Basement Membrane Disease/immunology ; Autoantibodies/blood ; Autoantibodies/immunology ; Biomarkers ; Case-Control Studies ; Female ; Humans ; Lupus Erythematosus, Systemic/blood ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/epidemiology ; Lupus Erythematosus, Systemic/immunology ; Lupus Nephritis/diagnosis ; Lupus Nephritis/epidemiology ; Lupus Nephritis/etiology ; Male ; Middle Aged ; Retrospective Studies ; Severity of Illness Index
Chemical Substances	Autoantibodies ; Biomarkers ; antiglomerular basement membrane antibody
Language	English
Publishing date	2020-12-14
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2020.597863
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article ; Online: Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies.

Galland, Joris / Mohamed, Shirine / Revuz, Sabine / de Maistre, Emmanuel / de Laat, Bas / Marie, Pierre-Yves / Zuily, Stéphane / Lévy, Bruno / Regnault, Véronique / Wahl, Denis

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis

2016 Volume 27, Issue 5, Page(s) 580–582

Abstract: Lupus anticoagulant-hypoprothrombinemia syndrome is a rare condition characterized by the association of acquired factor II deficiency and lupus anticoagulant. Contrary to classical antiphospholipid syndrome, it may cause severe life-threatening bleeding ...

Abstract	Lupus anticoagulant-hypoprothrombinemia syndrome is a rare condition characterized by the association of acquired factor II deficiency and lupus anticoagulant. Contrary to classical antiphospholipid syndrome, it may cause severe life-threatening bleeding (89% of published cases). We report a patient, positive for antidomain I antibodies, with initially primary lupus anticoagulant-hypoprothrombinemia syndrome without previous clinical manifestation or underlying systemic disease. Five years later, he experienced the first systemic lupus erythematous flare. Within a few days, catastrophic antiphospholipid syndrome was diagnosed with heart, liver and kidney involvement. The patient recovered under pulse steroids, intravenous heparin and intravenous immunoglobulins.
MeSH term(s)	Adult ; Antibodies, Antiphospholipid/blood ; Antibodies, Antiphospholipid/immunology ; Antiphospholipid Syndrome/complications ; Antiphospholipid Syndrome/diagnosis ; Antiphospholipid Syndrome/drug therapy ; Antiphospholipid Syndrome/immunology ; Heparin/therapeutic use ; Humans ; Hypoprothrombinemias/complications ; Hypoprothrombinemias/diagnosis ; Hypoprothrombinemias/drug therapy ; Hypoprothrombinemias/immunology ; Immunoglobulins, Intravenous/therapeutic use ; Kidney/immunology ; Kidney/metabolism ; Kidney/pathology ; Liver/immunology ; Liver/metabolism ; Liver/pathology ; Lupus Coagulation Inhibitor/blood ; Lupus Coagulation Inhibitor/immunology ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/drug therapy ; Lupus Erythematosus, Systemic/etiology ; Lupus Erythematosus, Systemic/immunology ; Male ; Myocardium/immunology ; Myocardium/metabolism ; Myocardium/pathology ; Steroids/therapeutic use
Chemical Substances	Antibodies, Antiphospholipid ; Immunoglobulins, Intravenous ; Lupus Coagulation Inhibitor ; Steroids ; Heparin (9005-49-6)
Language	English
Publishing date	2016-07
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	1033551-1
ISSN	1473-5733 ; 0957-5235
ISSN (online)	1473-5733
ISSN	0957-5235
DOI	10.1097/MBC.0000000000000545
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 2920: 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 ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability.

Revuz, Sabine / Decullier, Evelyne / Ginon, Isabelle / Lamblin, Nicolas / Hatron, Pierre-Yves / Kaminsky, Pierre / Carette, Marie-France / Lacombe, Pascal / Simon, Anne-Claire / Rivière, Sophie / Harlé, Jean-Robert / Fraisse, Alain / Lavigne, Christian / Leguy-Seguin, Vanessa / Chaouat, Ari / Khouatra, Chahera / Dupuis-Girod, Sophie / Hachulla, Eric

PloS one

2017 Volume 12, Issue 10, Page(s) e0184227

Abstract: Background: Different pulmonary hypertension (PH) mechanisms are associated with hereditary haemorrhagic telangiectasia (HHT).: Methods and results: We conducted a retrospective study of all suspected cases of PH (echocardiographically estimated ... ...

Abstract	Background: Different pulmonary hypertension (PH) mechanisms are associated with hereditary haemorrhagic telangiectasia (HHT). Methods and results: We conducted a retrospective study of all suspected cases of PH (echocardiographically estimated systolic pulmonary artery pressure [sPAP] ≥ 40 mmHg) in patients with definite HHT recorded in the French National Reference Centre for HHT database. When right heart catheterization (RHC) was performed, PH cases were confirmed and classified among the PH groups according to the European guidelines. Among 2,598 patients in the database, 110 (4.2%) had suspected PH. Forty-seven of these 110 patients had RHC: 38/47 (81%) had a confirmed diagnosis of PH. The majority of these had isolated post-capillary PH (n = 20). We identified for the first time other haemodynamic profiles: pre-capillary pulmonary arterial hypertension (PAH) cases (n = 3) with slightly raised pulmonary vascular resistances (PVR), and combined post- and pre-capillary PH cases (n = 4). Compared to controls, survival probability was lower in patients with PAH. Conclusion: This study revealed the diversity of PH mechanisms in HHT. The description of combined post- and pre-capillary PH with/or without high cardiac output (CO) suggests either a continuum between the pre- and post-capillary haemodynamic profiles or a different course in response to high CO.
MeSH term(s)	Cardiac Output/physiology ; Databases, Factual ; Echocardiography ; Female ; Hemodynamics/physiology ; Humans ; Hypertension, Pulmonary/diagnostic imaging ; Hypertension, Pulmonary/mortality ; Hypertension, Pulmonary/physiopathology ; Male ; Retrospective Studies ; Survival Rate ; Telangiectasia, Hereditary Hemorrhagic/diagnostic imaging ; Telangiectasia, Hereditary Hemorrhagic/mortality ; Telangiectasia, Hereditary Hemorrhagic/physiopathology ; Vascular Resistance/physiology
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.0184227
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

Order via subito

More links

Kategorien

Order via subito

Inter-library loan at ZB MED

Full text online

More links

Kategorien

Inter-library loan at ZB MED

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

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

Order via subito

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