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Article: L'hypertension pulmonaire: une cause rare de dyspnée inexpliquée.

Vachiéry, J L

2012 Volume 33, Issue 4, Page(s) 280–282

Abstract: Pulmonary hypertension (PH) is defined by an increase in mean pulmonary artery pressure above 25 mmHg, measured at right heart catheterization. The various conditions (up to 37) leading to PH are described in a clinical classification identifying 5 ... ...

Title translation	Pulmonary hypertension: a rare cause of unexplained dyspnea.
Abstract	Pulmonary hypertension (PH) is defined by an increase in mean pulmonary artery pressure above 25 mmHg, measured at right heart catheterization. The various conditions (up to 37) leading to PH are described in a clinical classification identifying 5 groups, including pulmonary arterial hypertension (PAH). With an incidence of 2-4 cases/million/year, PAH is a rare, rapidly progressive and incurable form or PH. The differential diagnosis of PH relies on a decision tree, which is typically triggered by the presence of unexplained dyspnea and followed by a non invasive approach that includes simple tests such as EKG, chest radiography, pulmonary function tests and echocardiography. Other tests have some value to exclude chronic thromboembolic pulmonary hypertension, such as ventilation/perfusion scintigraphy, angio CT scanner and pulmonary angiogram. Finally, right heart catheterization is mandatory to establish the diagnosis of PH.
MeSH term(s)	Diagnosis, Differential ; Dyspnea/diagnosis ; Dyspnea/etiology ; Dyspnea/therapy ; General Practitioners ; Humans ; Hypertension, Pulmonary/complications ; Hypertension, Pulmonary/diagnosis ; Hypertension, Pulmonary/therapy ; Physician's Role
Language	French
Publishing date	2012-09
Publishing country	Belgium
Document type	English Abstract ; Journal Article ; Review
ZDB-ID	760217-0
ISSN	0035-3639
ISSN	0035-3639
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Pediatric pulmonary arterial hypertension due to a novel homozygous GDF2 missense variant affecting BMP9 processing and activity.

Chomette, L / Hupkens, E / Romitti, M / Dewachter, L / Vachiéry, J L / Bailly, S / Costagliola, S / Smits, G / Tillet, E / Bondue, Antoine

American journal of medical genetics. Part A

2023 Volume 191, Issue 8, Page(s) 2064–2073

Abstract: Pulmonary arterial hypertension (PAH) is a rare and severe disorder characterized by progressive pulmonary vasculopathy. Growth differentiation factor (GDF)2 encodes the pro-protein bone morphogenetic protein (BMP) 9, activated after cleavage by ... ...

Abstract	Pulmonary arterial hypertension (PAH) is a rare and severe disorder characterized by progressive pulmonary vasculopathy. Growth differentiation factor (GDF)2 encodes the pro-protein bone morphogenetic protein (BMP) 9, activated after cleavage by endoproteases into an active mature form. BMP9, together with BMP10, are high-affinity ligands of activin receptor-like kinase 1 (ALK1) and BMP receptor type II (BMPR2). GDF2 mutations have been reported in idiopathic PAH with most patients being heterozygous carriers although rare homozygous cases have been described. The link between PAH occurrence and BMP9 or 10 expression level is still unclear. In this study, we describe a pediatric case of PAH also presenting with telangiectasias and epistaxis. The patient carries the novel homozygous GDF2 c.946A > G mutation, replacing the first arginine of BMP9's cleavage site (R316) by a glycine. We show that this mutation leads to an absence of circulating mature BMP9 and mature BMP9-10 heterodimers in the patient's plasma although pro-BMP9 is still detected at a similar level as controls. In vitro functional studies further demonstrated that the mutation R316G hampers the correct processing of BMP9, leading to the secretion of inactive pro-BMP9. The heterozygous carriers of the variant were asymptomatic, similarly to previous reports, reinforcing the hypothesis of modifiers preventing/driving PAH development in heterozygous carriers.
MeSH term(s)	Child ; Humans ; Bone Morphogenetic Proteins/genetics ; Growth Differentiation Factor 2/genetics ; Mutation ; Mutation, Missense/genetics ; Pulmonary Arterial Hypertension/genetics
Chemical Substances	BMP10 protein, human ; Bone Morphogenetic Proteins ; GDF2 protein, human ; Growth Differentiation Factor 2
Language	English
Publishing date	2023-05-30
Publishing country	United States
Document type	Case Reports ; Research Support, Non-U.S. Gov't
ZDB-ID	2108614-X
ISSN	1552-4833 ; 0148-7299 ; 1552-4825
ISSN (online)	1552-4833
ISSN	0148-7299 ; 1552-4825
DOI	10.1002/ajmg.a.63236
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Article ; Online: Possible biased virulence attenuation in the Senegal strain of Ehrlichia ruminantium by ntrX gene conversion from an inverted segmental duplication.

Gordon, Jonathan L / Oliva Chavez, Adela S / Martinez, Dominique / Vachiery, Nathalie / Meyer, Damien F

PloS one

2023 Volume 18, Issue 2, Page(s) e0266234

Abstract: Ehrlichia ruminantium is a tick-borne intracellular pathogen of ruminants that causes heartwater, a disease present in Sub-saharan Africa, islands in the Indian Ocean and the Caribbean, inducing significant economic losses. At present, three avirulent ... ...

Abstract	Ehrlichia ruminantium is a tick-borne intracellular pathogen of ruminants that causes heartwater, a disease present in Sub-saharan Africa, islands in the Indian Ocean and the Caribbean, inducing significant economic losses. At present, three avirulent strains of E. ruminantium (Gardel, Welgevonden and Senegal isolates) have been produced by a process of serial passaging in mammalian cells in vitro, but unfortunately their use as vaccines do not offer a large range of protection against other strains, possibly due to the genetic diversity present within the species. So far no genetic basis for virulence attenuation has been identified in any E. ruminantium strain that could offer targets to facilitate vaccine production. Virulence attenuated Senegal strains have been produced twice independently, and require many fewer passages to attenuate than the other strains. We compared the genomes of a virulent and attenuated Senegal strain and identified a likely attenuator gene, ntrX, a global transcription regulator and member of a two-component system that is linked to environmental sensing. This gene has an inverted partial duplicate close to the parental gene that shows evidence of gene conversion in different E. ruminantium strains. The pseudogenisation of the gene in the avirulent Senegal strain occurred by gene conversion from the duplicate to the parent, transferring a 4 bp deletion which is unique to the Senegal strain partial duplicate amongst the wild isolates. We confirmed that the ntrX gene is not expressed in the avirulent Senegal strain by RT-PCR. The inverted duplicate structure combined with the 4 bp deletion in the Senegal strain can explain both the attenuation and the faster speed of attenuation in the Senegal strain relative to other strains of E. ruminantium. Our results identify nrtX as a promising target for the generation of attenuated strains of E. ruminantium by random or directed mutagenesis that could be used for vaccine production.
MeSH term(s)	Animals ; Ehrlichia ruminantium/genetics ; Gene Conversion ; Senegal ; Virulence/genetics ; Segmental Duplications, Genomic ; Ruminants/genetics
Language	English
Publishing date	2023-02-17
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2267670-3
ISSN	1932-6203 ; 1932-6203
ISSN (online)	1932-6203
ISSN	1932-6203
DOI	10.1371/journal.pone.0266234
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Management of severe pulmonary arterial hypertension.

Vachiéry, J-L / Simonneau, G

European respiratory review : an official journal of the European Respiratory Society

2010 Volume 19, Issue 118, Page(s) 279–287

Abstract: Pulmonary arterial hypertension (PAH) is a severe, progressive condition of the small pulmonary vessels that leads to increased pulmonary vascular resistance, right ventricular failure and death. Patients in World Health Organization functional class ( ... ...

Abstract	Pulmonary arterial hypertension (PAH) is a severe, progressive condition of the small pulmonary vessels that leads to increased pulmonary vascular resistance, right ventricular failure and death. Patients in World Health Organization functional class (WHO FC) IV are the most severely affected in terms of disease severity, symptomatic impairment, exercise capacity and haemodynamics, with a very poor prognosis and low survival rate. Recent developments in PAH-specific therapies have conferred significant prognostic improvements upon PAH patients, especially when coupled with management strategies such as goal-oriented therapy and combination treatment. Despite these important developments, the outlook for WHO FC IV PAH patients remains poor. This article examines the recommendations for WHO FC IV patients that appear in current PAH treatment guidelines and the research underpinning this guidance, and discusses possible future directions for treatment of this severely unwell patient population.
MeSH term(s)	Drug Therapy, Combination ; Humans ; Hypertension, Pulmonary/classification ; Hypertension, Pulmonary/diagnosis ; Hypertension, Pulmonary/therapy ; Lung Transplantation ; Practice Guidelines as Topic ; Prognosis ; Severity of Illness Index ; World Health Organization
Language	English
Publishing date	2010-11-08
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	1077620-5
ISSN	1600-0617 ; 0905-9180
ISSN (online)	1600-0617
ISSN	0905-9180
DOI	10.1183/09059180.00008010
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: Early-onset and severe pulmonary arterial hypertension due to a novel compound heterozygous association of rare

Chomette, Laura / Migeotte, Isabelle / Dewachter, Céline / Vachiery, Jean-Luc / Smits, Guillaume / Bondue, Antoine

Pulmonary circulation

2022 Volume 12, Issue 2, Page(s) e12052

Abstract: Very rare cases of pulmonary arterial hypertension (PAH) have been linked to homozygous or compound heterozygous von Hippel-Lindau ( ...

Abstract	Very rare cases of pulmonary arterial hypertension (PAH) have been linked to homozygous or compound heterozygous von Hippel-Lindau (
Language	English
Publishing date	2022-04-01
Publishing country	United States
Document type	Case Reports
ZDB-ID	2638089-4
ISSN	2045-8940 ; 2045-8932
ISSN (online)	2045-8940
ISSN	2045-8932
DOI	10.1002/pul2.12052
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: IECA et/ou sartans dans l'insuffisance cardiaque: y a-t-il une différence?

Vachiéry, J L

Revue medicale de Bruxelles

2003 Volume 24, Issue 4, Page(s) A249–52

Abstract: The recent years have witnessed tremendous advances in the understanding of the pathophysiology of chronic heart failure (CHF), leading to significant improvement in therapy. Recognition of the deleterious effects of angiotensin II is an major ... ...

Title translation	ACE inhibitors and/or sartans in heart failure: is there a difference?.
Abstract	The recent years have witnessed tremendous advances in the understanding of the pathophysiology of chronic heart failure (CHF), leading to significant improvement in therapy. Recognition of the deleterious effects of angiotensin II is an major therapeutic target. By blocking the transformation of angiotensin I to angiotensin II, angiotensin-converting enzyme inhibitors (ACEI) improve symptoms, exercise tolerance and survival at all stages of CHF. Angiotensin II-receptors blockers (ARB) have several theoretical advantages over ACEI, including a better tolerance profile, that may lead to a more favourable effect. In CHF with systolic dysfunction, randomised-controlled trials comparing losartan to captopril (ELITE 1 and ELITE 2) or valsartan versus placebo on top of conventional therapy (Val-HeFT) failed to demonstrate a superiority of ARB's compared to ACEI in terms of mortality. However, ARB's appeared better tolerated in this indication. The treatment of CHF with preserved systolic function remains unclear and may be defined in a near future by two studies addressing the role of on candesartan (CHARM study) or irbesartan (I-Preserve study) in this form of CHF. ACEI and beta-blockers remain on first line for treating CHF due to systolic dysfunction. Losartan or valsartan can be considered as an alternative if ACEI are not tolerated or contraindicated.
MeSH term(s)	Angiotensin Receptor Antagonists ; Angiotensin-Converting Enzyme Inhibitors/therapeutic use ; Heart Failure/drug therapy ; Humans ; Losartan/therapeutic use ; Tetrazoles/therapeutic use ; Valine/analogs & derivatives ; Valine/therapeutic use ; Valsartan
Chemical Substances	Angiotensin Receptor Antagonists ; Angiotensin-Converting Enzyme Inhibitors ; Tetrazoles ; Valsartan (80M03YXJ7I) ; Valine (HG18B9YRS7) ; Losartan (JMS50MPO89)
Language	French
Publishing date	2003-09
Publishing country	Belgium
Document type	Comparative Study ; English Abstract ; Journal Article
ZDB-ID	760217-0
ISSN	0035-3639
ISSN	0035-3639
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: The endothelin system in pulmonary and renal vasculopathy: les liaisons dangereuses.

Vachiéry, J-L / Davenport, A

European respiratory review : an official journal of the European Respiratory Society

2009 Volume 18, Issue 114, Page(s) 260–271

Abstract: Endothelial cells regulate vascular tone largely by the actions of endothelin-1. Endothelin-1 is a potent vasoconstrictor, with effects that are dependent on the receptors to which it binds as well as their location. Endothelin-1 dysregulation is ... ...

Abstract	Endothelial cells regulate vascular tone largely by the actions of endothelin-1. Endothelin-1 is a potent vasoconstrictor, with effects that are dependent on the receptors to which it binds as well as their location. Endothelin-1 dysregulation is implicated in pathological conditions, including those of the pulmonary vasculature and the kidney. In this review, we describe the physiology and actions of endothelin-1 in lung and renal tissues and discuss therapies that disrupt these interactions in disease states. We provide an overview of the current clinical progress of these targeted agents and provide perspectives on the treatment of pulmonary and renal diseases with endothelin receptor antagonists.
MeSH term(s)	Endothelin A Receptor Antagonists ; Endothelin-1/physiology ; Humans ; Hypertension, Pulmonary/drug therapy ; Hypertension, Pulmonary/etiology ; Hypertension, Renal/drug therapy ; Hypertension, Renal/etiology
Chemical Substances	Endothelin A Receptor Antagonists ; Endothelin-1
Language	English
Publishing date	2009-12
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	1077620-5
ISSN	1600-0617 ; 0905-9180
ISSN (online)	1600-0617
ISSN	0905-9180
DOI	10.1183/09059180.00005709
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Screening for pulmonary arterial hypertension in systemic sclerosis.

Vachiéry, J-L / Coghlan, G

European respiratory review : an official journal of the European Respiratory Society

2009 Volume 18, Issue 113, Page(s) 162–169

Abstract: The onset and progression of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) can be particularly aggressive; however, effective treatments are available. Therefore, early identification of patients with suspected PAH, ... ...

Abstract	The onset and progression of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) can be particularly aggressive; however, effective treatments are available. Therefore, early identification of patients with suspected PAH, confirmation of diagnosis, and intervention is essential. PAH may be challenging to diagnose in its earliest stages, particularly in populations that have multiple causes of breathlessness, and, therefore, screening is required. The optimal screening tools and methodology are, as yet, unknown, and this is confounded by a lack of consensus over which patients to screen. Current practice favours annual screening of all SSc patients using Doppler echocardiography to detect elevated right heart pressures. This will typically identify most patients with the various forms of pulmonary hypertension found in SSc. The optimum thresholds for Doppler echocardiography are still subject to investigation, especially for patients with mild pulmonary hypertension, and this technique may, therefore, yield a significant number of false-positives and a currently unknown number of false-negatives. Confirmatory right heart catheterisation remains necessary in all suspected cases. Further research is needed to identify the optimal tools and the screening approach with greatest specificity and selectivity.
MeSH term(s)	Echocardiography, Doppler ; Familial Primary Pulmonary Hypertension ; Humans ; Hypertension, Pulmonary/complications ; Hypertension, Pulmonary/diagnostic imaging ; Mass Screening/methods ; Scleroderma, Systemic/complications ; Sensitivity and Specificity
Language	English
Publishing date	2009-09
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	1077620-5
ISSN	1600-0617 ; 0905-9180
ISSN (online)	1600-0617
ISSN	0905-9180
DOI	10.1183/09059180.00003209
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Article ; Online: Book review

J-L. Vachiery

European Respiratory Review, Vol 22, Iss 130, Pp 591-

Introduction to Cardiopulmonary Exercise Testing

2013 Volume 591

Keywords	Diseases of the respiratory system ; RC705-779 ; Specialties of internal medicine ; RC581-951 ; Internal medicine ; RC31-1245 ; Medicine ; R ; DOAJ:Internal medicine ; DOAJ:Medicine (General) ; DOAJ:Health Sciences
Language	English
Publishing date	2013-12-01T00:00:00Z
Publisher	European Respiratory Society
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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Article ; Online: How to detect disease progression in pulmonary arterial hypertension.

Vachiéry, J-L / Yerly, P / Huez, S

European respiratory review : an official journal of the European Respiratory Society

2011 Volume 21, Issue 123, Page(s) 40–47

Abstract: Pulmonary arterial hypertension (PAH) is a rapidly progressive disease, ultimately leading to right heart failure and death. Accumulating evidence indicates that intervention early in disease progression results in better outcomes than delaying treatment. ...

Abstract	Pulmonary arterial hypertension (PAH) is a rapidly progressive disease, ultimately leading to right heart failure and death. Accumulating evidence indicates that intervention early in disease progression results in better outcomes than delaying treatment. In this review we will discuss the assessments and strategies that can be used to monitor disease progression and guide clinical management. Many tools, such as symptoms, functional classification, exercise capacity, haemodynamic measures, findings on cardiac imaging and levels of biomarkers, have shown to be prognostic for survival both at diagnosis and during treatment. However, attempts to define goal thresholds have produced a variety of results. Several groups have developed risk calculators to estimate individual patients' mortality risk, but the accuracy of these tools across different patient populations remains unknown. What is clear is the importance of regularly assessing a range of parameters and then tailoring treatment goals to each patient. In addition, the use of a multidisciplinary team approach is crucial in order to support patients through all aspects of managing their condition. There is still an urgent need for prospective collaborative initiatives to assess novel goals and improve treatment strategies that would allow physicians to personalise and optimise clinical management for their patients with PAH.
MeSH term(s)	Disease Management ; Disease Progression ; Exercise Tolerance/physiology ; Familial Primary Pulmonary Hypertension ; Hemodynamics/physiology ; Humans ; Hypertension, Pulmonary/diagnosis ; Hypertension, Pulmonary/physiopathology ; Hypertension, Pulmonary/therapy ; Prognosis
Language	English
Publishing date	2011-10-14
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	1077620-5
ISSN	1600-0617 ; 0905-9180
ISSN (online)	1600-0617
ISSN	0905-9180
DOI	10.1183/09059180.00009011
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