Results 31 to 40 of about 1,670 (110)

Increased intervals in enzyme replacement therapy for stable type 1 Gaucher disease: A non‐inferiority sequential trial emulation

Journal of Internal Medicine, EarlyView.
Abstract Objective To compare the efficacy and safety of extended interval (Q3–4W) enzyme replacement therapy (ERT) versus standard biweekly (Q2W) ERT in clinically stable type 1 Gaucher disease (GD) patients. Methods We emulated a target trial with a sequential trial design, using data from the French Gaucher Disease Registry.
Maxime Beydon, Jérôme Stirnemann, Karima Yousfi, Samira Zebiche, Dalil Hamroun, Anaïs Brassier, Samia Pichard, Laure Swiader, Thierry Billette de Villemeur, Bénédicte Héron, Florence Dalbies, Bérengère Cador, Anne‐Sophie Guemann, Francis Gaches, Bénédicte Hivert, Vanessa Leguy‐Seguin, Agathe Masseau, Yves‐Marie Pers, Magali Pettazzoni, Soumeya Bekri, Catherine Caillaud, Edouard Le Guillou, Marie Szymanowski, Leonardo Astudillo, Wladimir Mauhin, Yann Nadjar, Christine Serratrice, Marc G. Berger, Fabrice Camou, Nadia Belmatoug, Yann Nguyen, the French Evaluation of Gaucher Disease Treatment Committee   +31 more
wiley   +1 more source

Identifying and Mapping Industrial Districts Through a Spatially Constrained Cluster‐Wise Regression Approach

Journal of Regional Science, Volume 65, Issue 2, Page 403-428, March 2025.
ABSTRACT The aim of this article is to exploit an innovative spatial econometric approach to map and study the evolving patterns of industrial districts (IDs). The procedure can be classified as a k‐means cluster‐wise regression procedure and is designed to detect homogeneous areas of subcontracting activity.
Jacopo Canello, Francesco Vidoli, Elisa Fusco, Nicoletta Giudice   +3 more
wiley   +1 more source

Oral Manifestations of Non Vascular Ehlers‐Danlos Syndrome Cross‐Sectional Study

Oral Diseases, EarlyView.
ABSTRACT Background Ehlers–Danlos syndromes are rare hereditary connective tissue disorders; however, their oral manifestations remain poorly characterized in molecularly confirmed individuals. The aim of this study was to describe the oral phenotype of patients with non‐vascular Ehlers‐Danlos syndromes compared to healthy controls.
Aude Grand, Benjamin P. J. Fournier, Grégoire Chevalier, Karelle Benistan, François Côme Ferré   +4 more
wiley   +1 more source

Diagnosis and Management of Stevens‐Johnson Syndrome and Toxic Epidermal Necrolysis in Pediatric Patients: A Systematic Review of Clinical Guidelines and Consensus Statements

Pediatric Dermatology, EarlyView.
ABSTRACT Stevens‐Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, life‐threatening mucocutaneous reactions. Pediatric mortality is lower than in adults, but children face higher risks of recurrence and long‐term sequelae. Despite disease severity, pediatric‐specific guidelines remain scarce and often extrapolated from adult ...
Rachel Creighton, Sana Gupta, Courtney Langille, Ashley Sutherland   +3 more
wiley   +1 more source

Simulation technico-économique d’une filière locale de valorisation du soja en alimentation animale dans le Sud-Ouest de la France

Oilseeds and fats, crops and lipids, 2015
Dans le contexte du développement de la culture du soja dans le Sud-Ouest de la France et à partir d’un diagnostic des filières de production de graines et des débouchés possibles, la mise en place d’une unité de trituration est ...
Quinsac Alain, Labalette Françoise, Carré Patrick, Parachini Elie, Jouffret Pierre   +4 more
doaj   +1 more source

Catene del valore e cibo ai tempi del COVID-19. Una ricerca esplorativa sulle filiere corte in Italia

Fuori Luogo, 2021
Il dibattito sull’impatto che la pandemia da COVID-19 ha avuto sul sistema agroalimentare è animato da posizioni molto contrastanti. Mentre alcuni autori tendono a sottolineare la grande capacità di resilienza mostrata dalle catene del cibo a livello ...
Alessandra Corrado, Marco Fama
doaj   +1 more source

Analyse de la Filière légumes de Niamey : dimension sociale des activités en pleine expansion

Revue des Etudes Multidisciplinaires en Sciences Economiques et Sociales, 2018
Une filière est une chaine de production et de commercialisation d’un produit agricole brut. Elle évolue dans le temps et dans l’espace. Dans le cadre de cette étude nous démontrerons le rôle social de la filière légumes.
HASSOUMI DJIBO
doaj   +1 more source

Cluster of Severe Arginase 1 Deficiency in the Comoros: Clinical, Neuroimaging, and Molecular Features in 17 Patients From Mayotte Compared With 10 From Paris

Journal of Inherited Metabolic Disease, Volume 49, Issue 2, March 2026.
ABSTRACT Arginase 1 deficiency (ARG1D) is the least common urea cycle disorder. Neonatal onset is rarely described, and hyperammonemic coma is less common in patients with ARG1D compared to other urea cycle disorders. In recent years, we diagnosed a high number of ARG1D patients in Mayotte, an insular (Comoro Islands) department of France.
Aurélie De Bruyne, Apolline Imbard, Charles‐Joris Roux, Abdourhahim Chamouine, Camille Schwab, Anaïs Brassier, Jean‐Baptiste Arnoux, Juliette Bouchereau, Cécile Laroche‐Raynaud, Tristan Mekdade, Margaux Gaschignard, Claire‐Marine Berat, Clément Pontoizeau, Stéphanie Gobin‐Limballe, Pascale de Lonlay, Jean‐François Benoist, Manuel Schiff   +16 more
wiley   +1 more source

Fabrication d’aliment du bétail issue de la cueillette de gousses de Piliostigma reticulatum : une opportunité pour les femmes rurales burkinabé

Cahiers Agricultures, 2016
Les gousses de l’arbre fourrager de Piliostigma reticulatum sont très utilisées au Burkina Faso. Comme peu de données existent sur cette activité, une enquête a été conduite en zone nord-soudanienne auprès de 90 personnes.
Dao Madjelia Cangré Ebou, Kone Martine, Somda Jacques   +2 more
doaj   +1 more source

Validation transculturelle du « Developmental Coordination Disorder Questionnaire’07 » à la population francophone d’Europe: le QTAC-FE

Revue Francophone de Recherche en Ergothérapie, 2016
Introduction. Le questionnaire parental Developmental Coordination Disorder Questionnaire’07 (DCDQ’07) est un instrument de mesure fiable et valide pour dépister le trouble de l’acquisition de la coordination (TAC) chez les enfants.
Sylvie Ray-Kaeser, Evelyne Thommen, Rose Martini, Anne Martine Bertrand   +3 more
doaj   +1 more source