Search results for "gauche"
showing 10 items of 62 documents
Un discours maoïste sur le syndicat. Le cas de la Gauche prolétarienne
2010
International audience
Molecular analysis of Gaucher disease: distribution of eight mutations and the complete gene deletion in 27 patients from Germany
1997
Gaucher disease is the most common lysosomal storage disease with a high prevalence in the Ashkenazi Jewish population but it is also present in other populations. The presence of eight mutations (1226G, 1448C, IVS2+1. 84GG, 1504T, 1604T, 1342C and 1297T) and the complete deletion of the beta-glucocerebrosidase gene was investigated in 25 unrelated non-Jewish patients with Gaucher's disease in Germany. In the Jewish population, three of these mutations account for more than 90% of all mutated alleles. In addition, relatives of two patients were included in our study. Restriction fragment length polymorphism analysis and sequencing of PCR products obtained from DNA of peripheral blood leukoc…
Retrospective Analysis of Whole-Body Magnetic Resonance Imaging of Bone Manifestations in Long-Term Treated Patients with Gaucher Disease Type 1
2018
We retrospectively assessed bone and visceral manifestations in patients with Gaucher disease type 1 (GD1) with whole-body magnetic resonance imaging (WB-MRI) to determine the effects of different timing in initiating long-term enzyme replacement therapy.In 17 patients with GD1, we performed 2 WB-MRI examinations at a median interval of 13 months. Patients had received enzyme replacement therapy with alglucerase/imiglucerase for a median of 13 years prior to the first examination. MRI results were retrospectively stratified based on treatment initiation into 2 groups: "early" (age ≤12 years, median 5 years) and "late" (during adulthood, median 32 years). We evaluated occurrence of irreversi…
Construction et déconstruction d'une catégorie : l'extrême gauche et ses avatars en France depuis 1989
2012
Da Lotta continua alla religione civile. La memoria di Peppino Impastato nelle storie di quattro militanti
2019
Il saggio vuole studiare il modo in cui si è trasformata la memoria pubblica di Peppino Impastato, ucciso dalla mafia nel 1978 a Cinisi, paese vicino Palermo. Per farlo, si seguiranno i percorsi biografici di quattro militanti di Lotta continua, il gruppo politico nel quale Impastato aveva militato fino al 1976, che si collocava nel vasto e complesso mondo della sinistra italiana esterna al Partito comunista. In particolare, ci si concentrerà sul modo in cui la memoria di Impastato si è intrecciata con lo stratificarsi del discorso pubblico sulla mafia e con i riti pubblici legati ad altre vittime della criminalità organizzata siciliana. Cet article présente la façon dont la mémoire publiqu…
Outcome of enzyme replacement therapy in patients with Gaucher disease type I. The Romanian experience
2007
This study reports the first evaluation of therapeutic response in Romanian patients with Gaucher disease type I, after therapy with Cerezyme recently became available in our country.24 patients (11-50 years) received Cerezyme 20-60 U/kg every two weeks for at least 18 months. Haemoglobin, platelet count, volume of the liver and spleen, plasma chitotriosidase and the severity score were assessed every 6 months; skeletal radiography and osteodensitometry were also monitored.Eleven patients were splenectomized before start of therapy. Eight patients had anaemia (mean haemoglobin 9.4 g/dl) and 14 patients, of whom 13 were without splenectomy, had thrombocytopenia (mean 65,692/mm3). Haemoglobin…
The citadel party : The French Communist Party in the Age of Political Marginalization
2022
Since the late 1970s, the French Communist Party (PCF) has been experiencing a long period of decline, affecting several elements (electoral collapse, decrease in memberships, disintegration of its action system…). Nowadays, it is a minor player in the French party system and becoming politically marginalized. However, despite this weakening over several decades, it is still present in the political landscape and even in some ways, demonstrates a resistant skill. How can we explain that a communist party still exists in a modern society like France ? This work is aimed to answer this question with the following hypothesis : the PCF has undergone a mutation which allows it to survive. This c…
Critical assessment of chitotriosidase analysis in the rational laboratory diagnosis of children with Gaucher disease and Niemann-Pick disease type A…
2006
Laboratory diagnosis of lysosomal storage disorders, especially sphingomyelinase deficiency (Niemann–Pick disease type A/B) and Niemann–Pick disease type C (NPC) can be challenging. We therefore aimed to analyse the feasibility of first-step screening with specific chitotriosidase cut-off values in children ≤ 10 years of age with visceral organomegaly (hepatomegaly, splenomegaly, or hepatosplenomegaly) in whom a storage disorder was suspected. We conducted a retrospective, cross-sectional, referral, single-centre study to assess diagnostic test properties in 106 individuals. Median chitotriosidase activity was 12 655 nmol/h per ml (interquartile range 4693–20982) in Gaucher disease (GD); 78…
Intraventrikuläre Obstruktionen bei der Dobutamin-Streßechokardiographie: Determinanten ihrer Entstehung und klinische Folgen (Dynamic intraventricul…
1997
Die Dobutamin-Stresechokardiographie (DSE) fuhrt zu einer starken Hyperkontraktilitat des Myokards bei Tachykardie und Vesodilatation. Diese Effekte konnen zu einer unphysiologischen Abnahme des endsystolischen Volumenindex (ESVI) mit Obstruktion des linksventrikularen Ausflustraktes (LVOT) fuhren. Bei 100 Patienten (Pat.), die sich einer DSE (5–40) μg/kg/min) unterzogen, wurde die kontinuierliche Dopplerregistrierung (DW-Doppler) vor Infusion und bei maximaler Herzfrequenz durchgefuhrt. Eine dynamische, spat-systolische, Flusakzeleration auf mehr als 2 Meter/Sekunde (m/s) unter maximaler Stimulation wurde als systolische Obstruktion des LVOT interpretiert. Manifestationsfaktoren systolisch…