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Le Touquet – Paris-Plage, France

Cuquemelle E.,University Paris Est Creteil | Soulis F.,Charles University | Villers D.,University of Nantes | Roche-Campo F.,Polyvalent Intensive Care Unit | And 9 more authors.
Intensive Care Medicine | Year: 2011

To determine whether procalcitonin (PCT) levels could help discriminate isolated viral from mixed (bacterial and viral) pneumonia in patients admitted to the intensive care unit (ICU) during the A/H1N1v2009 influenza pandemic. A retrospective observational study was performed in 23 French ICUs during the 2009 H1N1 pandemic. Levels of PCT at admission were compared between patients with confirmed influenzae A pneumonia associated or not associated with a bacterial co-infection. Of 103 patients with confirmed A/H1N1 infection and not having received prior antibiotics, 48 (46.6%; 95% CI 37-56%) had a documented bacterial co-infection, mostly caused by Streptococcus pneumoniae (54%) or Staphylococcus aureus (31%). Fifty-two patients had PCT measured on admission, including 19 (37%) having bacterial co-infection. Median (range 25-75%) values of PCT were significantly higher in patients with bacterial co-infection: 29.5 (3.9-45.3) versus 0.5 (0.12-2) μg/l (P < 0.01). For a cut-off of 0.8 μg/l or more, the sensitivity and specificity of PCT for distinguishing isolated viral from mixed pneumonia were 91 and 68%, respectively. Alveolar condensation combined with a PCT level of 0.8 μg/l or more was strongly associated with bacterial co-infection (OR 12.9, 95% CI 3.2-51.5; P < 0.001). PCT may help discriminate viral from mixed pneumonia during the influenza season. Levels of PCT less than 0.8 μg/l combined with clinical judgment suggest that bacterial infection is unlikely. © 2011 Copyright jointly held by Springer and ESICM. Source

Laforet P.,Institute Of Myologie | Laloui K.,Institute Of Myologie | Granger B.,Center Hospitalo University Pitie Salpetriere | Hamroun D.,Montpellier University | And 10 more authors.
Revue Neurologique | Year: 2013

Pompe disease is a rare autosomal recessive muscle lysosomal glycogenosis, characterised by limb-girdle muscle weakness and frequent respiratory involvement. The French Pompe registry was created in 2004 with the initial aim of studying the natural history of French patients with adult Pompe disease. Since the marketing in 2006 of enzyme replacement therapy (alglucosidase alfa, Myozyme®), the French Pompe registry has also been used to prospectively gather the biological and clinical follow-up data of all adult patients currently treated in France. This report describes the main clinical and molecular features, at the time of inclusion in the French registry, of 126 patients followed up in 21 hospital-based neuromuscular or metabolic centres. Sixty-five men and 61 women have been included in the registry. Median age at inclusion was 49 years, and the median age at onset of progressive limb weakness was 35 years. Fifty-five percent of the patients were walking without assistance, 24% were using a stick or a walking frame, and 21% were using a wheelchair. Forty-six percent of the patients needed ventilatory assistance, which was non-invasive in 35% of the cases. When performed, muscle biopsies showed specific features of Pompe disease in less than two-thirds of the cases, confirming the importance of acid alpha-glucosidase enzymatic assessment to establish the diagnosis. Molecular analysis detected the common c.-32-13T > G mutation, in at least one allele, in 90% of patients. The French Pompe registry is so far the largest country-based prospective study of patients with Pompe disease, and further analysis will be performed to study the impact of enzyme replacement therapy on the progression of the disease. © 2013 Elsevier Masson SAS. Tous droits réservés. Source

Hausfater P.,Center Hospitalo University Pitie Salpetriere | Megarbane B.,Center Hospitalo University Lariboisiere | Dautheville S.,Center Hospitalo University Tenon | Patzak A.,Center Hospitalo University George Pompidou | And 9 more authors.
Intensive Care Medicine | Year: 2010

Purpose: To identify the prognostic factors associated with mortality in heat-related illness. Methods: Multi-center observational cohort-study in 16 emergency departments (ED) belonging to the teaching hospital network of the Paris area. The cohort comprised all patients admitted to one of the EDs during the August 2003 heat wave in Paris and having a core temperature > 38.5°C. Baseline clinical and biological data in ED, patient's course and 1-year survival rate were recorded. Potential prognostic factors associated with death were assessed by Cox proportional-hazards analysis. Results: A total of 1,456 patients were included. Mean age was 79 ± 19 years. Critically ill conditions were noted in 391 patients (27%), but only 72 (5%) were admitted into an intensive care unit. The survival rate was 57% at 1 year as compared to an expected 90% (P < 0.001). Nine independent prognostic factors were identified: previous treatment with diuretics, living in an institution, age[80 - years, cardiac disease, cancer, core temperature >40°C, systolic arterial pressure <100 mmHg, Glasgow coma scale <12 and transportation to hospital by ambulance. We defined three risk groups: low, intermediate and high risk, with a 1-year survival rate of 85, 61 and 18%, respectively. Conclusions: We observed a low survival rate and developed a risk score based on easily obtained variables that may be useful to clinicians managing casualties from future heat waves. © Copyright jointly hold by Springer and ESICM 2009. Source

Sartorius D.,University of Lyon | Sartorius D.,Lyon University Hospital Center | Le Manach Y.,Center Hospitalo University Pitie Salpetriere | David J.-S.,Lyon University Hospital Center | And 8 more authors.
Critical Care Medicine | Year: 2010

Objectives: Prehospital triage of trauma patients is of paramount importance because adequate trauma center referral improves survival. We developed a simple score that is easy to calculate in the prehospital phase. Design: Multicenter prospective observational study. Setting: Prehospital physician-staffed emergency system in university and nonuniversity hospitals. Interventions: We evaluated 1360 trauma patients receiving care from a prehospital mobile intensive care unit in 22 centers in France during 2002. The association of prehospital variables with in-hospital death was tested using logistic regression, and a simple score (the Mechanism, Glasgow coma scale, Age, and Arterial Pressure [MGAP] score) was created and compared with the triage Revised Trauma Score, Revised Trauma Score, and Trauma Related Injury Severity Score. The model was validated in 1003 patients from 2003 through 2005. Measurements and Main Results: Four independent variables were identified, and each was assigned a number of points proportional to its regression coefficient to provide the MGAP score: Glasgow Coma Scale (from 3-15 points), blunt trauma (4 points), systolic arterial blood pressure (>120 mm Hg: 5 points, 60 to 120 mm Hg: 3 points), and age <60 yrs (5 points). The area under the receiver operating characteristic curve of MGAP was not significantly different from that of the triage Revised Trauma Score or Revised Trauma Score, but when sensitivity was fixed >0.95 (undertriage of 0.05), the MGAP score was more specific and accurate than triage Revised Trauma Score and Revised Trauma Score, approaching those of Trauma Related Injury Severity Score. We defined three risk groups: low (23-29 points), intermediate (18-22 points), and high risk (<18 points). In the derivation cohort, the mortality was 2.8%, 15%, and 48%, respectively. Comparable characteristics of the MGAP score were observed in the validation cohort. Conclusion: The MGAP score can accurately predict in-hospital death in trauma patients. Copyright © 2010 by the Society of Critical Care Medicine and Lippincott Williams & Wilkins. Source

Granger B.,University Pierre and Marie Curie | Granger B.,Center Hospitalo University Pitie Salpetriere | Gueneau L.,French Institute of Health and Medical Research | Gueneau L.,University Pierre and Marie Curie | And 10 more authors.
Human Genetics | Year: 2011

Autosomal dominant Emery-Dreifuss muscular dystrophy is caused by mutations in LMNA gene encoding lamins A and C. The disease is characterized by early onset joint contractures during childhood associated with humero-peroneal muscular wasting and weakness, and by the development of a cardiac disease in adulthood. Important intra-familial variability characterized by a wide range of age at onset of myopathic symptoms (AOMS) has been recurrently reported, suggesting the contribution of a modifier gene. Our objective was to identify a modifier locus of AOMS in relation with the LMNA mutation. To map the modifier locus, we genotyped 291 microsatellite markers in 59 individuals of a large French family, where 19 patients carrying the same LMNA mutation, exhibited wide range of AOMS. We performed Bayesian Markov Chain Monte Carlo-based joint segregation and linkage methods implemented in the Loki© software, and detected a strong linkage signal on chromosome 2 between markers D2S143 and D2S2244 (211 cM) with a Bayes factor of 28.7 (empirical p value = 0.0032). The linked region harbours two main candidate genes, DES and MYL1 encoding desmin and light chain of myosin. Importantly, the impact of the genotype on the phenotype for this locus showed an overdominant effect with AOMS 2 years earlier for the homozygotes of the rare allele and 37 years earlier for the heterozygotes than the homozygotes for the common allele. These results provide important highlights for the natural history and for the physiopathology of Emery-Dreifuss muscular dystrophy. © 2010 Springer-Verlag. Source

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