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101.
Rituximab combined with chemotherapy and interferon in follicular lymphoma patients: results of the GELA-GOELAMS FL2000 study 下载免费PDF全文
Salles G Mounier N de Guibert S Morschhauser F Doyen C Rossi JF Haioun C Brice P Mahé B Bouabdallah R Audhuy B Ferme C Dartigeas C Feugier P Sebban C Xerri L Foussard C 《Blood》2008,112(13):4824-4831
The FL2000 study was undertaken to evaluate the combination of the anti-CD20 monoclonal antibody rituximab with chemotherapy plus interferon in the first-line treatment of follicular lymphoma patients with a high tumor burden. Patients were randomly assigned to receive either 12 courses of the chemotherapy regimen CHVP (cyclophosphamide, adriamycin, etoposide, and prednisolone) plus interferon-alpha2a (CHVP+I arm) over 18 months or 6 courses of the same chemotherapy regimen combined with 6 infusions of 375 mg/m(2) rituximab and interferon for the same time period (R-CHVP+I arm). After a median follow-up of 5 years, event-free survival estimates were, respectively, 37% (95% confidence interval [CI], 29%-44%) and 53% (95% CI, 45%-60%) in the CHVP+I and R-CHVP+I arm (P = .001). Five-year overall survival estimates were not statistically different in the CHVP+I (79%; 95% CI, 72%-84%) and R-CHVP+I (84%; 95% CI, 78%-84%) arms. In a multivariate regression analysis, event-free survival was significantly influenced by both the Follicular Lymphoma International Prognostic Index score (hazard ratio = 2.08; 95% CI, 1.6%-2.8%) and the treatment arm (hazard ratio = 0.59; 95% CI, 0.44%-0.78%). With a 5-year follow-up, the combination of rituximab with CHVP+I provides superior disease control in follicular lymphoma patients despite a shorter duration of chemotherapy. This study's clinical trial was registered at the National Institutes of Health website as no. NCT00136552. 相似文献
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The third international meeting on genetic disorders in the RAS/MAPK pathway: Towards a therapeutic approach 下载免费PDF全文
Bruce Korf Reza Ahmadian Judith Allanson Yoko Aoki Annette Bakker Emma Burkitt Wright Brian Denger Ype Elgersma Bruce D. Gelb Karen W. Gripp Bronwyn Kerr Maria Kontaridis Conxi Lazaro Corinne Linardic Reymundo Lozano Calum A. MacRae Ludwine Messiaen Sonia Mulero‐Navarro Benjamin Neel Scott Plotkin Katherine A. Rauen Amy Roberts Alcino J. Silva Sitta G. Sittampalam Chao Zhang Lisa Schoyer 《American journal of medical genetics. Part A》2015,167(8):1741-1746
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André Maues De Paula Alexandre Vasiljevic Roch Giorgi Anne Gomez-Brouchet Sébastien Aubert Xavier Leroy Hélène Duval Gonzague de Pinieux Corinne Bouvier 《Virchows Archiv : an international journal of pathology》2014,465(4):487-494
Some primary malignant or benign tumours of bone contain numerous multinucleated cells. These “giant cell-rich tumours of bone” have overlapping features and clinical and radiological data are needed to reach an accurate pathological diagnosis. We studied the potential contribution of p63 immunohistochemistry to the reliability of the histological diagnosis. We performed a multicentric retrospective study of 291 giant cell-rich tumours of bone which included 119 giant cell tumours of bone (GCTB), 76 aneurysmal bone cysts (ABC), 49 chondroblastomas (CB), 15 nonossifying fibromas (NOF), 10 giant cell reparative granulomas (RG) of jaws, 1 giant cell lesion of small bones, 2 hyperparathyroidism-related brown tumours (BT), 17 bone sarcomas with numerous osteoclasts and 2 malignant giant cell tumours of bone. p63 is expressed in ABC, CB, NOF, RG, BT and GCTB, but its expression in more than 50 % of mononuclear cells is strongly suggestive of a diagnosis of GCTB. In contrast, malignant GCTB were mostly negative. Our results show that p63 is expressed in a broad range of benign giant cell-rich tumours of bone, consistent with data in the recent literature, while infrequent in malignant tumours. With a cut-off 50 %, the presence of p63 positive cells is useful in supporting a diagnosis of giant cell-rich tumour of bone. However, a final diagnosis cannot be made without due consideration of all clinical/radiological and pathological data. 相似文献
106.
Federico di Rocco Geneviève Baujat Eric Arnaud Dominique Rénier Jean-Louis Laplanche Valérie Cormier Daire Corinne Collet 《European journal of human genetics : EJHG》2014,22(12):1413-1416
TCF12 mutations have been reported very recently in coronal synostosis. We report several cases of familial coronal synostosis among four families harbouring novel TCF12 mutations. We observed a broad interfamilial phenotypic spectrum with features overlapping with the Saethre–Chotzen syndrome. TCF12 molecular testing should be considered in patients with unilateral- or bilateral-coronal synostosis associated or not with syndactyly, after having excluded mutations in the TWIST1 gene and the p.Pro250Arg mutation in FGFR3. 相似文献
107.
Jean-François Brugère Guillaume Borrel Nadia Gaci William Tottey Paul W O’Toole Corinne Malpuech-Brugère 《Gut microbes》2014,5(1):5-10
Trimethylamine (TMA) is produced by gut bacteria from dietary ingredients. In individuals with a hereditary defect in flavin-containing monooxygenase 3, bacterial TMA production is believed to contribute to the symptoms of trimethylaminuria (TMAU; fish-odor syndrome). Intestinal microbiota TMA metabolism may also modulate atherosclerosis risk by affecting trimethylamine oxide (TMAO) production levels. We propose that reducing TMA formation in the gut by converting it to an inert molecule could be used to prevent or limit these human diseases, while avoiding the major drawbacks of other clinical interventions. Reducing TMA levels by microbiological interventions could also be helpful in some vaginoses. Particular members of a recently discovered group of methanogens, that are variably present in the human gut, are unusual in being apparently restricted to utilizing only methyl compounds including TMA as substrates. We confirmed experimentally that one of these strains tested, Methanomassiliicoccus luminyensis B10, is able to deplete TMA, by reducing it with H2 for methanogenesis. We therefore suggest that members of this archaeal lineage could be used as treatments for metabolic disorders. 相似文献
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Valérie Biran An Phan Duy Fabrice Decobert Nathalie Bednarek Corinne Alberti Olivier Baud 《Developmental medicine and child neurology》2014,56(8):717-723
The prevention of neurological disabilities following preterm birth remains a major public health challenge and efforts are still needed to test the neuroprotective properties of candidate molecules. Melatonin serves as a neuroprotectant in adult models of cerebral ischemia through its potent antioxidant and anti‐inflammatory effects. An increasing number of preclinical studies have consistently demonstrated that melatonin protects the damaged developing brain by preventing abnormal myelination and an inflammatory glial reaction, a major cause of white matter injury. The main questions asked in this review are whether preclinical data on the neuroprotective properties of melatonin are sufficient to translate this concept into the clinical setting, and whether melatonin can reduce white matter damage in preterm infants. This review provides support for our view that melatonin is now ready to be tested in human preterm neonates, and discusses ongoing and planned clinical trials. 相似文献