Habets E.J.J.,Medical Center Haaglanden |
Taphoorn M.J.B.,Medical Center Haaglanden |
Taphoorn M.J.B.,VU University Amsterdam |
Nederend S.,VU University Amsterdam |
And 11 more authors.
Journal of Neuro-Oncology | Year: 2014
Overall survival of patients with anaplastic oligodendroglial tumors has been improved due to the addition of procarbazine, lomustine and vincristine (PCV) chemotherapy to radiotherapy (RT), especially in 1p/19q-codeleted tumors. With improved survival, quality of survival becomes pivotal. We evaluated cognitive functioning and health-related quality of life (HRQOL) in a cohort of long-term anaplastic oligodendroglioma survivors. Thirty-two out of 37 long-term survivors included in European Organisation for Research and Treatment of Cancer (EORTC) study 26951 in the Netherlands and France participated. Cognition was assessed using neuropsychological tests for 6 domains, and HRQOL with the EORTC Quality of Life Questionnaire (EORTC QLQ-C30) and Brain Cancer Module (EORTC QLQ-BN20). Fatigue and mood were evaluated. Results were compared to healthy controls and to patients' own HRQOL 2.5 years following initial treatment. At the time of assessment, median survival for the patients was 147 months, 27 were still progression-free since initial treatment. Of progression-free patients, 26 % were not, and 30 % were severely cognitively impaired; 41 % were employed and 81 % could live independently. Patients' HRQOL was worse compared to controls, but similar to 2.5 years after initial treatment. Initial treatment (RT versus RT + PCV) was not correlated with cognition or HRQOL. In conclusion, cognitive functioning in long-term anaplastic oligodendroglioma survivors is variable. However, most patients function independently. In progression-free patients, HRQOL is relatively stable during the disease course. In this small sample, no effect of the addition of PCV on cognition or HRQOL was identified. © 2013 Springer Science+Business Media New York.
Tan J.Y.,University of Oxford |
Vance K.W.,University of Oxford |
Varela M.A.,University of Cape Town |
Watson L.M.,University Pierre and Marie Curie |
And 16 more authors.
Nature Structural and Molecular Biology | Year: 2014
What causes the tissue-specific pathology of diseases resulting from mutations in housekeeping genes? Specifically, in spinocerebellar ataxia type 7 (SCA7), a neurodegenerative disorder caused by a CAG-repeat expansion in ATXN7 (which encodes an essential component of the mammalian transcription coactivation complex, STAGA), the factors underlying the characteristic progressive cerebellar and retinal degeneration in patients were unknown. We found that STAGA is required for the transcription initiation of miR-124, which in turn mediates the post-transcriptional cross-talk between lnc-SCA7, a conserved long noncoding RNA, and ATXN7 mRNA. In SCA7, mutations in ATXN7 disrupt these regulatory interactions and result in a neuron-specific increase in ATXN7 expression. Strikingly, in mice this increase is most prominent in the SCA7 disease-relevant tissues, namely the retina and cerebellum. Our results illustrate how noncoding RNA-mediated feedback regulation of a ubiquitously expressed housekeeping gene may contribute to specific neurodegeneration.
Benamrouz S.,Institute Pasteur Of Lille |
Benamrouz S.,University of Lille Nord de France |
Conseil V.,Institute Pasteur Of Lille |
Conseil V.,University of Lille Nord de France |
And 7 more authors.
Parasite | Year: 2012
The International Agency for Research on Cancer (IARC) identifies ten infectious agents (viruses, bacteria, parasites) able to induce cancer disease in humans. Among parasites, a carcinogenic role is currently recognized to the digenetic trematodes Schistosoma haematobium, leading to bladder cancer, and to Clonorchis sinensis or Opisthorchis viverrini, which cause cholangiocarcinoma. Furthermore, several reports suspected the potential association of other parasitic infections (due to Protozoan or Metazoan parasites) with the development of neoplastic changes in the host tissues. The present work shortly reviewed available data on the involvement of parasites in neoplastic processes in humans or animals, and especially focused on the carcinogenic power of Cryptosporidium parvum infection. On the whole, infection seems to play a crucial role in the etiology of cancer.
Vambergue A.,Service de Diabetologie |
Perimenis P.,Groupe Hospitalier
Correspondances en MHND | Year: 2015
The intra uterine diabetic environment affects the fetoplacental unit. The fetoplacental unit has to adapt itself to an influx of nutriments (glucose, amino acids, lipids) in excess. In case of maternal diabetes, the placenta sets up hormonal, infl ammatory and immune adaptative mechanisms. In case of maternal diabetes, the PPARs (peroxisome proliferatoractivated receptors) are involved in the placentation, in the modifi cation of the mediators of the infl ammation, and in the lipid metabolism. These adaptative mechanisms have for consequence a modifi cation of transfers, immune responses and angiogenesis associated with an oxidative stress. These placental adaptative mechanisms will have consequences in clinical practice, as abnormalities of growth, macrosomia or intra-uterine growth restriction (IUGR).
Nae I.-C.,Groupe hospitalier |
Chevet J.-B.,Groupe hospitalier |
Delaporte B.,Groupe hospitalier
Archives de Pediatrie | Year: 2011
Postnatal urinary ascites is a rare occurrence compared with other causes of peritoneal effusion at this age and its frequency in prenatal diagnosis. Spontaneous rupture of the bladder was diagnosed in a premature male infant presenting with postnatal ascites and anuric renal insufficiency. Surgical repair was required. No predisposing factor was associated with this rare complication. The urinary origin of the ascites was diagnosed based on the biochemical composition, allowing us to understand its mechanism and to treat it. © 2010 Elsevier Masson SAS.
Reyes S.,University Paris Diderot |
Kurtz A.,University Paris Diderot |
Herve D.,University Paris Diderot |
Tournier-Lasserve E.,Groupe Hospitalier |
Chabriat H.,University Paris Diderot
Journal of Neurology | Year: 2012
Genetic counselling has been poorly investigated in cerebrovascular diseases. Characteristics, motivations and long-term outcome of presymptomatic tests (PT) in subjects at risk of CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy) were investigated at the National Centre for Rare Vascular Diseases of the Brain and/or Retina (CERVCO). Sociodemographic, motivational and psychological variables were collected between 2003 and 2010 for PT applicants. Multidisciplinary consultations (with a geneticist, neurologist and psychologist) were proposed over a 6 month period. When PT showed a deleterious mutation of the NOTCH3 gene, cognitive performances, mood, autonomy and quality of life were also assessed. Over 7 years, only 33 subjects asked for a PT of CADASIL. They were predominantly women, lived as a couple, had children and were of high sociocultural level. The dropout rate after the first step of the procedure was 63%. The characteristics of the 11 subjects who reached the end of the procedure did not differ from the 22 who dropped out. Six were carriers of the deleterious mutation and were still asymptomatic after a mean follow-up of 19 months. They did not experience any particular negative event and all of them indicated a high score of overall quality of life. Indeed, two carriers gave birth to their first child. These initial data in CADASIL show that PT is rarely requested and that there is a high dropout rate. Our study also highlights that a multidisciplinary and multistep procedure in genetic counselling testing appears useful to obtain minimal harmful consequences of genetic testing. © Springer-Verlag 2012.
Verny M.,Groupe hospitalier |
Berrut G.,Nantes University Hospital Center
Geriatrie et Psychologie Neuropsychiatrie du Vieillissement | Year: 2012
The definition of normal pressure hydrocephalus (NPH), in adults, associates clinical signs (Adams and Hakim triad) involving gait disorders, urinary incontinence and dementia, associated with aspects on brain imaging that are consistent with this hypothesis and also normal or slightly increased intracranial pressure. The aim of this study was to clarify the techniques and methods facilitating the diagnosis of NPH. The literature review has been conducted from the Medline database without date limitation including the keywords "normal pressure hydrocephalus" and "diagnosis." They should appear in the article title. From the 43 initially sorted, only 13 have been selected using exclusion criteria. The proposed methods are very sparse and focused on the improvement after surgical shunt. This focus is independent from the diagnosis criteria proposed in 2005. This introduces an ambiguity in the interpretation of the results. In practice, the diagnosis of NPH is more difficult in the elderly population where differential diagnoses are frequent, particularly vascular lesions (notably microangiopathy) and Alzheimer's disease. The more invasive techniques as continuous spinal drainage (usually during 3 days) or some features of CSF dynamics (Rout, compliance) seem to be the best predictors of after shunt improvement. However, these techniques are difficult to use in routine in the elderly. The combination of Evans index and corpus callosum angle on MRI is very useful to improve the differential diagnosis with cerebral atrophy. Spinal tap test (lumbar puncture with the removal of 40 mL of CSF) can be repeated two or three times for consecutive days to improve the predictive value before shunting. Gait and balance often improve after shunt, more than cognition and bladder disorders. In the elderly population, the prognosis after 3 years is non conclusive despite initial improvement. Poor prognosis seems to be due to associated pathologies in particular neurodegenerative diseases. This should be considered in decision-making of CSF shunt.
Larger E.,Groupe Hospitalier |
Lemoine A.-Y.,University of Paris Descartes |
Faucher P.,University of Paris Descartes
Medecine des Maladies Metaboliques | Year: 2015
The long-term follow-up of the patients having participated to glucose trials in diabetes have shown diverging trajectories with regards to diabetes-specific complications, according to the intervention group patients had been randomized to during the intervention phases of the trials. Group-specific risk in the incidence of complication has been observed although blood glucose levels have been largely similar during the long-term follow-up. The long-term persistence of differences has been called metabolic memory. The present manuscript presents the long-term, 30 years, results of the Diabetes control and complications trial (DCCT) and the molecular basis of the metabolic memory, based on several experimental models. © 2015 Elsevier Masson SAS.
El Rachkidi R.,Groupe Hospitalier |
Sari-Leret M.-L.,Groupe Hospitalier |
Wolff S.,Groupe Hospitalier
Spine | Year: 2011
Study Design: A case report with review of the literature. Objective: To present a first case of an atraumatic bilateral pedicle fracture of L5 in a patient with long-term risedronate therapy and without any previous history of surgery or fracture. Summary of Background Data: A few cases of bilateral osteoporotic pedicle fractures have been reported. All were associated with previous or concomitant corporeal compression fractures. Atypical femoral fractures were recently described with long-term bisphosphonate treatment. To our knowledge, there are no previous reports of isolated bilateral pedicle fracture associated with prolonged bisphosphonate therapy. Methods: A 66-year-old woman treated with risedronate for 10 years and without any history of trauma presented with exacerbation of low back pain. No fracture was noted in her past medical history. Results: A complete workup showed an isolated bilateral recent fracture of the L5 pedicles without evident etiology. Consolidation was achieved after 3 months of bracing. Conclusion: We reported the first case of isolated bilateral pedicle fracture in a patient treated with risedronate for 10 years. This may be another atypical fracture in long-term bisphosphonate treatment. © 2011 Lippincott Williams & Wilkins.
Training on health dispensed at the hospital: A medicine chest simulation for the safe use of drugs at home [Education pour la santéreáliseé à l'hôpital: une simulation d'armoire à pharmacie pour sécuriser l'usage des médicaments au domicile]
Laincer A.,Groupe Hospitalier |
Lukat S.,Groupe Hospitalier |
Dalle-Pecal M.,Groupe Hospitalier |
Divine C.,Groupe Hospitalier
Pharmacien Hospitalier et Clinicien | Year: 2016
Introduction. As part of 2014 patient security week, a medicine chest simulation took place at the hospital in order to communicate on correct storage and use of drugs at home. Method. A home medicine chest has been reproduced in the hospital hall and then within the cardiac re-adaptation hospital day. The medicine chest included seventeen products such as drugs and medical devices, of which eleven purposely contained errors. The objective for the attendees (patients, visitors and hospital staff) consisted in identifying mistakes. Results. Thirty-six people took part in this event. Average number of mistakes was eight (between two and eleven). The three most notified errors were the presence of a cleaning product bottle (100 %), unable to identify loose pills (97 %) and single dose kept after opening (86 %). Ninety-four percent of attendees consider that they have learnt useful information and 53 % of them claim that they are willing to change organization of their own medicine chest. Conclusion. The home medicine chest simulation is a tool to educate in an enjoyable way on health topics and is interesting to set up within health institutions. © 2015 Elsevier Masson SAS.