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Le Kremlin-Bicêtre, France

Chaumais M.-C.,University Paris - Sud | Chaumais M.-C.,French Institute of Health and Medical Research | Chaumais M.-C.,Departement Hospitalo University Thorax Innovation | Guignabert C.,French Institute of Health and Medical Research | And 18 more authors.
American Journal of Cardiovascular Drugs | Year: 2015

Pulmonary arterial hypertension (PAH) is a devastating life-threatening disorder characterized by elevated pulmonary vascular resistance leading to elevated pulmonary arterial pressures, right ventricular failure, and ultimately death. Vascular endothelial cells mainly produce and secrete endothelin (ET-1) in vessels that lead to a potent and long-lasting vasoconstrictive effect in pulmonary arterial smooth muscle cells. Along with its strong vasoconstrictive action, ET-1 can promote smooth muscle cell proliferation. Thus, ET-1 blockers have attracted attention as an antihypertensive drug, and the ET-1 signaling system has paved a new therapeutic avenue for the treatment of PAH. We outline the current understanding of not only the pathogenic role played by ET-1 signaling systems in the pathogenesis of PH but also the clinical pharmacology of endothelin receptor antagonists (ERA) used in the treatment of PAH. © 2014, Springer International Publishing Switzerland.


Thierry G.,Nantes University Hospital Center | Beneteau C.,Nantes University Hospital Center | Pichon O.,Nantes University Hospital Center | Flori E.,Service de cytogenetique | And 28 more authors.
American Journal of Medical Genetics, Part A | Year: 2012

Patients with a submicroscopic deletion at 1q43q44 present with intellectual disability (ID), microcephaly, craniofacial anomalies, seizures, limb anomalies, and corpus callosum abnormalities. However, the precise relationship between most of deleted genes and the clinical features in these patients still remains unclear. We studied 11 unrelated patients with 1q44 microdeletion. We showed that the deletions occurred de novo in all patients for whom both parents' DNA was available (10/11). All patients presented with moderate to severe ID, seizures and non-specific craniofacial anomalies. By oligoarray-based comparative genomic hybridization (aCGH) covering the 1q44 region at a high resolution, we obtained a critical deleted region containing two coding genes-HNRNPU and FAM36A-and one non-coding gene-NCRNA00201. All three genes were expressed in different normal human tissues, including in human brain, with highest expression levels in the cerebellum. Mutational screening of the HNRNPU and FAM36A genes in 191 patients with unexplained isolated ID did not reveal any deleterious mutations while the NCRNA00201 non-coding gene was not analyzed. Nine of the 11 patients did not present with microcephaly or corpus callosum abnormalities and carried a small deletion containing HNRNPU, FAM36A, and NCRNA00201 but not AKT3 and ZNF238, two centromeric genes. These results suggest that HNRNPU, FAM36A, and NCRNA00201 are not major genes for microcephaly and corpus callosum abnormalities but are good candidates for ID and seizures. © 2012 Wiley Periodicals, Inc.


Soubeyrand M.,Service de chirurgie orthopedique | Ciais G.,Service de chirurgie orthopedique | Wassermann V.,Service de chirurgie orthopedique | Kalouche I.,Service de chirurgie orthopedique | And 5 more authors.
Journal of Bone and Joint Surgery - Series B | Year: 2011

Disruption of the interosseous membrane is easily missed in patients with Essex-Lopresti syndrome. None of the imaging techniques available for diagnosing disruption of the interosseous membrane are completely dependable. We undertook an investigation to identify whether a simple intra-operative test could be used to diagnose disruption of the interosseous membrane during surgery for fracture of the radial head and to see if the test was reproducible. We studied 20 cadaveric forearms after excision of the radial head, ten with and ten without disruption of the interosseous membrane. On each forearm, we performed the radius joystick test: moderate lateral traction was applied to the radial neck with the forearm in maximal pronation, to look for lateral displacement of the proximal radius indicating that the interosseous membrane had been disrupted. Each of six surgeons (three junior and three senior) performed the test on two consecutive days. Intra-observer agreement was 77% (95% confidence interval (CI) 67 to 85) and interobserver agreement was 97% (95% CI 92 to 100). Sensitivity was 100% (95% CI 97 to 100), specificity 88% (95% CI 81 to 93), positive predictive value 90% (95% CI 83 to 94), and negative predictive value 100%). This cadaveric study suggests that the radius joystick test may be useful for detecting disruption of the interosseous membrane in patients undergoing open surgery for fracture of the radial head and is reproducible. A confirmatory study in vivo is now required. © 2011 British Editorial Society of Bone and Joint Surgery.


We present a case of bilateral posterior shoulder dislocation after an epileptic seizure. The anterior humeral-head impression fracture was 60% of the articular surface on the right shoulder and 30% on the left shoulder. We performed an early one-stage reconstruction of both humeral heads. A cancellous autograft was used on the left side and an iliac cortico-cancellous autograft on the right side, with preservation of the patient's cartilage. Three years later, the clinical and morphological results were excellent. The discussion focuses on surgical options that range from conservative treatment with excision of the damaged cartilage to immediate hemiarthroplasty. This case is original because of the preservation of the patient's cartilage during reconstruction. © 2012 Elsevier Masson SAS.


Apostolo A.,University of Milan | Laveneziana P.,Hopital University Of Bicetre | Laveneziana P.,Paris-Sorbonne University | Laveneziana P.,French Institute of Health and Medical Research | And 20 more authors.
International Journal of Cardiology | Year: 2015

Background: Heart failure (HF) and chronic obstructive pulmonary disease (COPD) coexistence increases morbidity and mortality. The intercept of ventilation (VEint) on the VE vs. carbon dioxide production (VCO2) relationship during exercise has been found to vary in proportion with dead space (VD) in HF. Considering that increased VD is the key pathophysiological abnormality in COPD but a secondary finding in HF we hypothesized that a high VEint would be useful in suggesting COPD as HF co-morbidity. Our aim was to assess whether an elevated VEint suggests the presence of COPD in HF. Methods: In a multicenter retrospective study, the VE-VCO2 relationship was analyzed both as slope and intercept in HF (n = 108), HF-COPD (n = 106) and COPD (n = 95). Patients with pulmonary arterial hypertension (PAH) (n = 85) and healthy subjects (HF) (n = 56) served as positive and negative controls relative to VE-VCO2 abnormalities, respectively. Results: Slope and VEint varied in opposite directions in all groups (p < 0.05) being VE-VCO2 slope highest and lowest in PAH and healthy subjects, respectively. No slope differences were observed among HF, HF-COPD and COPD (32 ± 7, 31 ± 7, and 31 ± 6, respectively). VEint was higher in HF-COPD and COPD compared to HF, PAH and controls (4.8 ± 2.4 L/min, 5.9 ± 3.0 L/min, 3.0 ±2.6 L/min, 2.3 ± 3.3 L/min and 3.9 ±2.5 L/min, respectively; p < 0.01). A VEint ≥4.07 L/min identified patients with high probability of having COPD or HF-COPD (sensitivity of 71.6% and specificity of 72.0%). Conclusion: These data provide novel evidence that a high VEint (≥4.07 L/min) should be valued to suggest coexistent COPD in HF patients. © 2015 Published by Elsevier Ireland Ltd.

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