Contribution a l'Etude des Toxines de Venin de Serpent: Etude Immunologique et Clonage Moleculaire de Toxines du Serpent Bungarus Multicinctus

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Transient ciliogenesis involving Bardet-Biedl syndrome proteins is a fundamental characteristic of adipogenic differentiation.

International audienceBardet-Biedl syndrome (BBS) is an inherited ciliopathy generally associated with severe obesity, but the underlying mechanism remains hypothetical and is generally proposed to be of neuroendocrine origin. In this study, we show that while the proliferating preadipocytes or mature adipocytes are nonciliated in culture, a typical primary cilium is present in differentiating preadipocytes. This transient cilium carries receptors for Wnt and Hedgehog pathways, linking this organelle to previously described regulatory pathways of adipogenesis. We also show that the BBS10 and BBS12 proteins are located within the basal body of this primary cilium and inhibition of their expression impairs ciliogenesis, activates the glycogen synthase kinase 3 pathway, and induces peroxisome proliferator-activated receptor nuclear accumulation, hence favoring adipogenesis. Moreover, adipocytes derived from BBS-patients' dermal fibroblasts in culture exhibit higher propensity for fat accumulation when compared to controls. This strongly suggests that a peripheral primary dysfunction of adipogenesis participates to the pathogenesis of obesity in BBS

Two-level non-invasive positive pressure ventilation in the initial treatment of acute respiratory failure in an emergency department.

There are few data on the use of two-level non-invasive positive pressure ventilation (two-level nIPPV) in the initial treatment of severe acute respiratory failure in emergency departments (ED). In a prospective, non-randomized, pilot study, we assessed (1) the feasability of this method in an ED, (2) its effect on clinical and laboratory data, and (3) its effect on the need of intubation and the final outcome of patients. During a 1-year period all eligible patients admitted for acute respiratory failure, with absence of improvement after periods of specific classic treatments, were included in the study. Each patient received a specific classic treatment and two-level nIPPV with a two-level positive pressure ventilator through a face mask. We recorded parameters on admission, after 15 and 45 minutes of nIPPV and at the end of nIPPV. Sixty-two patients were included: 29 with acute pulmonary oedema (APO), 16 with acute exacerbation of chronic obstructive pulmonary disease (COPD), four with asthma, and 13 with various diseases. In the APO-group, we observed a statistically significant improvement of respiratory and pulse rates, diastolic blood pressure, pH, PaCO2 and SaO2. In acute exacerbation of COPD, we observed only a statistical improvement of respiratory and pulse rates without any significant change of PaCO2 and pH. In the two other groups, there was a clinical, gasometric and haemodynamic improvement in all patients. Four patients were intubated and 10 died, but none in the ED or in the first 24 hours after hospital admission. We were able to institute two-level nIPPV for severe acute respiratory failure in an ED without complications. Its addition to the rest of classic specific treatment seems to bring about a rapid improvement of various clinical and laboratory parameters in most patients. We found no deleterious effect of nIPPV when implemented for short periods of time in the emergency department setting

Adult liver transplantation: the Université Catholique de Louvain experience.

Liver transplantation remains a formidable surgical and medical procedure. The larger single centre experience confirms that standardization of perioperative care and simplification of the surgical procedure markedly improve results. Further efforts must be made in relation to immunosuppressive therapy in order to minimize late morbidity and mortality

La ventilation non invasive dans l'asthme aigu grave, une nouvelle attitude thérapeutique?

[Noninvasive ventilation in severe status asthmaticus, a new therapeutic approach? Two case reports] The use of noninvasive ventilation in acute asthma in an open uncontrolled study has already been reported. We report two cases of acute status asthmaticus where the clinical condition deteriorates even with an optimal medical treatment. The appearance of acute respiratory acidosis, the worsening of the dyspnea, auscultatory silence and the consciousness state necessitate an endotracheal intubation and mechanical ventilation. This has been obviated by using noninvasive bilevel ventilation. With this technique, the above mentioned parameters improved rapidly

Confirmation of TFAP2A gene involvement in branchio-oculo-facial syndrome (BOFS) and report of temporal bone anomalies.

International audienceBranchio-oculo-facial syndrome (BOFS) is an autosomal-dominant condition characterized by three main features, respectively: branchial defects, ocular anomalies, and craniofacial defects including cleft lip and/or palate (CL/P). We report on one family with three affected, and two sporadic cases that have been found to carry missense mutations in the newly reported BOFS gene: TFAP2A. This report confirms the involvement of this transcription factor in this developmental syndrome with clinical variability. Moreover, we present CT scan temporal bone anomalies in the familial cases, related to branchial arch defects, highlighting the importance of radiological investigations for differential diagnosis

Pathological responses after angiogenesis or EGFR inhibitors in metastatic colorectal cancer depend on the chemotherapy backbone.

BACKGROUND: Optimal preoperative treatment before colorectal cancer metastases (CRCM) resection remains unclear. This study evaluated pathological responses (pR) in CRCM resected after chemotherapy alone or combined with angiogenesis or epidermal growth factor receptor (EGFR) inhibitors. METHODS: Pathological response was retrospectively evaluated on 264 resected metastases from 99 patients. The proportion of responding metastases after different preoperative treatments was reported and compared. Patient's progression-free survival (PFS) and overall survival (OS) were compared based on pR. RESULTS: The combination of anti-angiogenics with oxaliplatin-based chemotherapy resulted in more pR than when they were combined with irinotecan-based chemotherapy (80% vs 50%; P<0.001). Inversely, the combination of EGFR inhibitors with oxaliplatin-based chemotherapy seemed to induce fewer pR than when they were combined with irinotecan-based treatment (53% vs 72%; P=0.049). Overall survival at 5 years was improved for patients with a pR in all resected metastases compared with those who did not achieve a pR (68.5% vs 32.6%; P=0.023) and this response was the only factor predicting OS in a multivariate analysis. CONCLUSION: The chemotherapy partner combined with angiogenesis or EGFR inhibitors influenced pR in resected CRCM. In our exploratory analysis anti-angiogenic/oxaliplatin-based regimens and anti-EGFR/irinotecan-based regimens were associated with the highest pR. Prospective randomised trials should be performed to validate these observations