Imiglucerase in the management of Gaucher disease type 1: an evidence-based review of its place in therapy

Gaucher disease is the first lysosomal disease to benefit from enzyme replacement therapy, thus serving as model for numerous other lysosomal diseases. Alglucerase was the first glucocerebrosidase purified from placental extracts, and this was then replaced by imiglucerase - a Chinese hamster ovary cell-derived glucocerebrosidase

MANIFESTATIONS CARDIOVASCULAIRES DE LA MALADIE D'ERDHEIM CHESTER (DES MEDECINE INTERNE)

AIX-MARSEILLE2-BU Méd/Odontol. (130552103) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Détection d’une bactériémie par des hémocultures : qui en bénéficie ?

Blood cultures are often prescribed in the hospital setting, even if their yield is weak. It's important to try to predict which patient will really benefit from them. There are some parameters that increase the chances of positivity of a blood culture, such as septic choc, chills or suspicion of Staphylococcus aureus bacteremia. Fever is not indicative. There are some clinical scores that permit to determinate the probability of a blood culture's positivity ; they haven't a high specificity, thus allowing mainly to avoid the taking of blood cultures whose risk of being negative is high. A new subject of interest in the field of infectious disease is the prediction of a resistant-organism bacteremia. This article aims to summarize the main predictions that we can draw when it comes to evaluate the utility of collecting a blood culture

An adult case of plastic bronchitis: a rare and multifactorial disease

Plastic bronchitis is a rare and potentially fatal disease. Mainly a disease of the pediatric age, a few adult cases occurring after cardiac surgery have been described. We describe a case of a 41-year-old man suffering from several episodes of acute dyspnea and cough with expectoration of mucous plugs in the context of chronic allergic airway inflammation. We believe that the occurrence of plastic bronchitis in adulthood should not be overlooked particularly in patients with chronic inflammatory lung disease

Approche pragmatique des syndromes de microangiopathie thrombotique

Whilst the pathophysiology underlying the diverse thrombotic microangiopathy syndromes is better understood, management of these entities and of the various therapeutic options currently available remains challenging. Early identification of these syndromes is essential to improving prognosis. In the absence of treatment, mortality is high, including in the first days following diagnosis. We present a summary of the diagnostic strategy and therapeutic management of thrombotic microangiopathy in adults.Alors que la physiopathologie de la microangiopathie thrombotique est de mieux en mieux comprise, la gestion de cette entité en aigu et les différentes options thérapeutiques à disposition restent moins claires. Son identification précoce est indispensable pour permettre une amélioration du pronostic. En l’absence de traitement, le taux de mortalité est très élevé, et ce dans les premiers jours qui suivent le diagnostic. Cet article a pour objectif de permettre une synthèse des stratégies diagnostique et thérapeutique à adopter devant une suspicion de microangiopathie thrombotique chez l’adulte

Étude par RMN du carbone-13 de solutions de tensioactifs fluorés non- ioniques de type perfluoroalkylethylthiopolyethyleneoxy alcool

Les paramètres des spectres de carbone-13 (déplacements chimiques δ et temps de relaxation spin-réseau T1) d'une nouvelle série de tensioactifs fluorés non-ioniques, de formule C6F13C2H4SC2H4(OC2H4)nOH ou C6F13C2H4SC2H4(OC2H4)ρSC2H4(OC2H4)ρOH sont déterminés en solution dans le méthanol ou dans un fluorocarbure. Dans ce dernier cas, on examine tout particulièrement l'effet d'addition d'eau sur les paramètres RMN. Ainsi les variations de δ sont attribuées à l'hydratation de la partie hydrophile du tensioactif. Elles sont plus prononcées pour le groupement C2H4SC2H4, situé à la jonction hydrophile-hydrophobe du surfactif, qui subirait des changements conformationnels empêchant la pénétration de l'eau vers la partie hydrophobe. Les valeurs de T1 indiquent une grande rigidité de la partie hydrophile et du groupement C2H4SC2H4