Time filter

Source Type

Le Mans, France

Laribi K.,Center Hospitalier Le Mans | Denizon N.,Center Hospitalier Le Mans | Ghnaya H.,Center Hospitalier Le Mans | Atlassi M.,Center Hospitalier Le Mans | And 4 more authors.
European Journal of Haematology | Year: 2014

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy which was first included as an independent cutaneous lymphoma in the 2008 World Health Organisation (WHO) classification (1). BPDCN usually has an extremely poor prognosis, with quick relapses after chemotherapy (2; 3). Here, we report two cases of patients diagnosed in 2011 with BPDCN and myelodysplasia, and who were treated for the first time with 5-azacytidine (5-Aza); a drug approved by the Food and Drug Administration (FDA) and mainly used in the treatment of myelodysplastic syndrome (Kaminskas E, et al. 2005 Clin Cancer Res, 11, 3604-8). The first case was an 81-year-old man who presented with unusual CD10+, CD56- immunohistochemistry and 45X, -Y abnormality using fluorescent in situ hybridization (FISH) analysis. The second case was a 78-year-old woman who manifested monosomy 13 and chromosome instability due to D13S319 locus deletion in 13q14 as determined by FISH. Both patients showed excellent responses of their skin lesions after one cycle of chemotherapy, and their hematological disease was stabilized; however, pulmonary sepsis set in, followed by neutropenia after the fourth and the fifth cycle of treatment, that is, eight and 9 months postdiagnosis, respectively, leading to patient death. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. Source

Baroudjian B.,University Paris Diderot | Viguier M.,University Paris Diderot | Battistella M.,University Paris Diderot | Beneton N.,Center Hospitalier du Mans | And 3 more authors.
British Journal of Dermatology | Year: 2014

Idiopathic CD4+ lymphocytopenia (ICL) is a rare immunodeficiency syndrome of unknown origin for which the increased risks of opportunistic infections and of malignancies have been well established; however, skin dysimmune diseases, including psoriasis, have been scarcely reported up to now. We report herein the severe course of psoriasis in four patients with ICL, and show evidence for a defect in the skin recruitment of regulatory CD4 +FoxP3+ T cells. These data raise the apparent paradigm of the occurrence of a severe immunomediated disease together with a profound T-cell defect, a model that might also apply to other immune deficiencies associated with psoriasis. What's already known about this topic? While the risk of opportunistic infections and of malignancies has been previously stressed in idiopathic CD4+ T-cell lymphocytopenia (ICL), the association with skin dysimmune disorders, mainly psoriasis, has been poorly addressed so far. What does this study add? We report four cases of severe psoriasis in patients with ICL and discuss the contribution of a regulatory T-cell defect to the pathogenesis of psoriasis in this setting. © 2014 British Association of Dermatologists. Source

Valade D.,Paris West University Nanterre La Defense | Plaisance P.,Paris West University Nanterre La Defense | Derouet N.,Center Hospitalier du Mans | Meric G.,Pfizer | Giroud M.,Center Hospitalier Rene Dubos
Cephalalgia | Year: 2011

Background: We conducted a prospective study of patients admitted to 22 general emergency departments in France over 1 week. Patients and methods: Of 15,835 adult patients, 483 (3.1%) had headache and 98 (0.6%) had migraine. Results: Compared with the migraine population in France, our migraine patients were similar in terms of proportion of female patients (75%) and mean age (37.6 ± 13.8 years) but presented earlier in their disease course. Patients sought emergency treatment because of a severe attack (49%) or because of ineffective treatment (20%). Non-opioid analgesics excluding non-steroidal anti-inflammatory drugs (NSAIDs), and NSAIDs, were most commonly prescribed as acute treatment, yet it took more than 48 h for symptom resolution in 36% of 92 follow-up patients. Conclusions: Results suggest there is room for improvement in choice of agents prescribed. We propose additional education and training of clinicians to improve adherence to clinical practice guidelines. © International Headache Society 2010. Source

Pidhorz L.,Center Hospitalier du Mans
Orthopaedics and Traumatology: Surgery and Research | Year: 2015

Humeral shaft fractures account for up to 5% of all fractures. Many of these fractures are still being treated conservatively using functional (Sarmiento) bracing or a hanging arm cast. Union is achieved in 10weeks in more than 94% of cases. Angulation of less than 30° varus or valgus and less than 20° flessum or recurvatum can be tolerated by the patient from a functional and esthetic point of view. The ideal candidate for this treatment is a patient with an isolated fracture. Plate and screw fixation of the fracture results in union in 11 to 19weeks. Reported complications include non-union (2.8-21%), secondary radial nerve palsy (6.5-12%) and infection (0.8-2.4%). Anterograde or retrograde locked intramedullary nailing requires knowledge of nailing techniques and regional anatomy to avoid the complications associated with the technique. Union is obtained in 10-15weeks. Reported complications consist of non-union (2-17.4%), infection (0-4%) and secondary radial nerve palsy (2.7-5%). Hackethal bundle nailing is still used for fracture fixation, despite an elevated complication rate (5-24% non-union and 6-29% pin migration) because of its low cost and simple instrumentation. Union is achieved in 8-9weeks. Controversy remains about the course to follow when the radial nerve is injured initially. If the fracture is open, significantly displaced, associated with a vascular injury or requires surgical treatment, the nerve must be explored. In other cases, the recommended approach varies greatly. Conservative treatment is inexpensive and has a low complication rate. Humeral shaft fractures are increasingly being treated surgically, at a greater cost and higher risk of complications. © 2015 Elsevier Masson SAS. Source

Isidor B.,Nantes University Hospital Center | Lindenbaum P.,Nantes University Hospital Center | Lindenbaum P.,French Institute of Health and Medical Research | Lindenbaum P.,University of Nantes | And 26 more authors.
Nature Genetics | Year: 2011

Hajdu-Cheney syndrome is a rare autosomal dominant skeletal disorder with facial anomalies, osteoporosis and acro-osteolysis. We sequenced the exomes of six unrelated individuals with this syndrome and identified heterozygous nonsense and frameshift mutations in NOTCH2 in five of them. All mutations cluster to the last coding exon of the gene, suggesting that the mutant mRNA products escape nonsense-mediated decay and that the resulting truncated NOTCH2 proteins act in a gain-of-function manner. © 2011 Nature America, Inc. All rights reserved. Source

Discover hidden collaborations