283 results match your criteria: "Centre Hospitalier du Mans[Affiliation]"

Further delineation of the duplication syndrome phenotype in 59 French male patients, with a particular focus on morphological and neurological features.

J Med Genet

June 2018

Service de génétique médicale, Institut de Génétique Médicale d'Alsace (IGMA), Centre de Référence Maladies Rares "Anomalies du développement et syndromes malformatifs", Centre de Référence Maladies Rares "Des déficiences intellectuelles de causes rares", Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, Strasbourg, France.

Article Synopsis
  • * Patients commonly exhibited distinct facial characteristics that changed with age, such as midface hypoplasia and prominent ears, along with associated physical issues like hypotonia and spasticity, impacting their ability to walk.
  • * Medical complications in these patients included frequent epilepsy, recurrent lung infections, and significant concerns like pulmonary hypertension leading to early mortality, highlighting the need for early screening.
View Article and Find Full Text PDF

Clinical and Therapeutic Aspects of Linear Psoriasis: A Study of 30 Cases.

Am J Clin Dermatol

August 2018

Service de Dermatologie, Hôpital Victor Dupouy, 69 rue du Lieutenant-Colonel Prud'hon, 95100, Argenteuil, France.

Background: Psoriasis affects 2-4% of the population, with the most common clinical type being plaque psoriasis. The linear form of psoriasis is very rare. The literature on linear psoriasis (LP) consists of only case reports, and data are few.

View Article and Find Full Text PDF

Background: The increasing use of psychotropic drugs to treat anxiety and depressive disorders (ADDs) is concerning. According to the study, 'Etude Pharmacoépidémiologique de l'Impact de Santé Publique des modes de prise en charge pour 3 groupes de pathologies' (EPI3)-LASER, adult ADD patients who consult a general practitioner prescribing homeopathic medicines (GP-Ho) report less psychotropic drug use and are marginally more likely to experience clinical improvement than those receiving conventional care. We determined whether these observations also apply to patients ≥ 65 years old in the EPI3 cohort.

View Article and Find Full Text PDF

Gabriel Richet was one of the great pioneers of European Nephrology. After a pivotal period of work with Jean Hamburger, whom we owe the name of our discipline, Nephrology, he contributed to all aspects of this specialty and was, in particular, a forerunner in dialysis and in the study of interstitial nephropathies.In this passionate and lucid interview, recorded in Paris in 2010, he describes himself as a "lucky man", able to transform folly in happiness.

View Article and Find Full Text PDF

On the shoulders of giants. The story behind the 'Pioneers of Nephrology' project.

BMC Nephrol

March 2018

BioMed Central, The Campus, 4 Crinan Street, London, N1 9XW, UK.

This editorial introduces a series of interviews with the pioneers of Nephrology. It's a story that speaks by itself, given the thousands of people that are now alive thanks to the remarkable advances in renal replacement therapies such as dialysis, and kidney transplantation but also the many scientific advances in our understanding of the pathophysiology and treatment of kidney diseases worldwide.The interviews that we have selected for this series are, however, not dealing with their achievements, and their success; they try to pass on to future generations the idea of how they were, why they were passionate, what they loved, and, not last, where they found poetry in our profession.

View Article and Find Full Text PDF

Objectives: The aims of this study in SLE population were (1) to describe ultrasonography (US) joint abnormalities, (2) to estimate the reliability of clinical swollen joint count (C-SJC) and SLEDAI (C-SLEDAI) versus US-SJC and US-SLEDAI scores, (3) to highlight specific patterns of lupus patients with Power Doppler (PD) abnormalities.

Method: For this cross-sectional multicenter study, 151 consecutive adult SLE patients were recruited. Evaluation included a clinical standardized joint assessment, B-mode and PD US of 40 joints and 26 tendons blinded for clinical examination.

View Article and Find Full Text PDF

Ibrutinib has revolutionized the management of chronic lymphocytic leukemia and is now being increasingly used. Although considered to be less immunosuppressive than conventional immunochemotherapy, the observation of a few cases of invasive fungal infections in patients treated with ibrutinib prompted us to conduct a retrospective survey. We identified 33 cases of invasive fungal infections in patients receiving ibrutinib alone or in combination.

View Article and Find Full Text PDF

MABLE investigated the efficacy and safety of rituximab plus bendamustine or rituximab plus chlorambucil in fludarabine-ineligible patients with chronic lymphocytic leukemia. Patients received rituximab plus bendamustine or rituximab plus chlorambucil every four weeks for six cycles. Rituximab plus chlorambucil-treated patients without a complete response after Cycle 6 received chlorambucil monotherapy for at least six additional cycles or until complete response.

View Article and Find Full Text PDF

Whole exome sequencing diagnoses the first fetal case of Bainbridge-Ropers syndrome presenting as pontocerebellar hypoplasia type 1.

Birth Defects Res

April 2018

Unité d'Embryofœtopathologie, Service d'Histologie Embryologie Cytogénétique, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris (APHP), Paris, France.

Background: Bainbridge-Ropers syndrome (BRPS) is a recently identified severe disorder characterized by failure to thrive, facial dysmorphism, and severe developmental delay, caused by de novo dominant loss of function mutation in the ASXL3 gene.

Case: We report here the first case of prenatal BRPS in a fetus presenting with arthrogryposis on ultrasound and for pontocerebellar hypoplasia type 1 (PCH1) following neuropathological examination. The diagnosis was done by whole exome sequencing that identified a novel de novo ASXL3 mutation.

View Article and Find Full Text PDF

Vaccination coverage among school children aged 14-15 years in the French department of Sarthe.

Med Mal Infect

March 2018

Centre de prévention, de vaccination et de dépistage, centre hospitalier du Mans, 194, avenue Rubillard, 720037 Le Mans cedex 9, France. Electronic address:

Objectives: Vaccinations are the main pillar of primary prevention for infectious diseases. To guide and prioritize public health interventions, the Regional Health Agency has asked the Prevention, Vaccination, and Screening Center of the Sarthe department to assess vaccination coverage (VC) at the sub-departmental level. The main objective was to measure the VC among school children aged 14-15 years in Sarthe at the sub-departmental level.

View Article and Find Full Text PDF

Background: Most patients with chronic lymphocytic leukaemia relapse after initial therapy combining chemotherapy with rituximab. We assessed the efficacy and safety of rituximab maintenance treatment versus observation for elderly patients in remission after front-line abbreviated induction by fludarabine, cyclophosphamide, and rituximab (FCR).

Methods: This randomised, open-label, multicentre phase 3 trial at 89 centres in France enrolled treatment-naive and fit patients aged 65 years or older with chronic lymphocytic leukaemia without del(17p).

View Article and Find Full Text PDF

T-prolymphocytic leukemia (T-PLL) is a rare T-cell neoplasm with an aggressive clinical course. Leukemic T-cells exhibit a post-thymic T-cell phenotype (Tdt, CD1a, CD5, CD2 and CD7) and are generally CD4/CD8, but CD4/CD8 or CD8/CD4 T-PLL have also been reported. The hallmark of T-PLL is the rearrangement of chromosome 14 involving genes for the subunits of the T-cell receptor (TCR) complex, leading to overexpression of the proto-oncogene TCL1.

View Article and Find Full Text PDF

Introduction: Sanoia is an online interactive electronic e-health platform developed to allow patient self-assessment and self-monitoring. The objective was to assess in rheumatoid arthritis (RA) patients, the efficacy on patient-physician interactions, of giving access to Sanoia.

Methods: In this French, multi-center, 12-months randomized controlled trial (CarNET: NCT02200068), patients with RA and internet access were randomized to: access without incentives to the Sanoia platform after minimal training, or usual care.

View Article and Find Full Text PDF

Background: The aim of ESCAP-2011-CPHG, promoted by the French College of General Hospital Respiratory Physicians, was to describe therapeutic strategies in lung cancer in the first 2 years after diagnosis, in a real-life setting. This article focuses on patients undergoing surgical management of a non-small cell lung cancer (NSCLC).

Methods: A prospective multicentre study was conducted in 53 French general hospitals.

View Article and Find Full Text PDF

Background: The impact of intraoperative neuromonitoring on recurrent laryngeal nerve palsy remains debated. Our aim was to evaluate the potential protective effect of intraoperative neuromonitoring on recurrent laryngeal nerve during total thyroidectomy.

Methods: This was a prospective, multicenter French national study.

View Article and Find Full Text PDF

Introduction: The objective of the ESCAP-2011-CPHG cohort study was to perform a real-life analysis of therapeutic strategies used during the first 2years of follow-up after a diagnosis of primary lung cancer. This paper presents the study and its first results in non-small-cell lung cancer (NSCLC).

Methods: Pulmonologists in the respiratory disease departments of 53 general hospitals consecutively included all patients aged 18years and over with lung cancer newly diagnosed in 2010.

View Article and Find Full Text PDF

R-CHOP 14 with or without radiotherapy in nonbulky limited-stage diffuse large B-cell lymphoma.

Blood

January 2018

Hematology and Cell Therapy Department, CIC INSERM U1415, CHU de Poitiers, and CIC INSERM 1402, Université de Poitiers, Poitiers, France.

The benefit of radiotherapy (RT) after chemotherapy in limited-stage diffuse large B-cell lymphoma (DLBCL) remains controversial. We conducted a randomized trial in patients with nonbulky limited-stage DLBCL to evaluate the benefit of RT after rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). Patients were stratified according to the modified International Prognostic Index, including lactate dehydrogenase, Eastern Cooperative Oncology Group performance status, age, and disease stage.

View Article and Find Full Text PDF

[An atypical case of lipoid proteinosis].

Ann Pathol

October 2017

Service d'anatomie et cytologie pathologiques, centre hospitalier du Mans, 72037 Le Mans cedex 09, France.

The lipoid proteinosis is a rare autosomic recessive genodermatosis characterized histologically by deposits of hyaline-like eosinophilic material of characteristic distribution. We herein report the case of a 56-year-old man admitted for progressive aggravated dementia associated with a late-onset dysphonia. Histologic examination of cutaneous and laryngeal biopsies showed deposits of an amorphous and eosinophilic material arranged around vessels, and adnexal structures, stained by PAS and congo red negative.

View Article and Find Full Text PDF

Background: No effective treatment is currently available and dialysis related amyloidosis continues to be invalidating in long-term dialysis patients. A recent case series reported reduction of osteoarticular pain on doxycycline treatment, extending the indications of this drug, used in other uncommon forms of amyloidosis, to dialysis patients. Explanations of the antalgic effect were the anti-inflammatory properties and anti-coiling effects of tetracycline.

View Article and Find Full Text PDF

Radioisotope synovectomy has been extensively used to treat patients with chronic inflammatory joint disease but has moved to a less prominent position since the introduction of new and highly effective drugs. Remaining indications are refractory synovitis, pigmented villonodular synovitis as an adjunct to surgery, and hemophilic arthropathy. The three main radioisotopes used are yttrium-90, rhenium-186, and erbium-189.

View Article and Find Full Text PDF

Prospective validation of a lymphocyte infiltration prognostic test in stage III colon cancer patients treated with adjuvant FOLFOX.

Eur J Cancer

September 2017

Université Paris Descartes, Sorbonne Paris Cité, Paris, France; Paris Descartes University, Paris Sorbonne cité, Department of Digestive Oncology, HEGP, Paris, France.

Background: The prognostic value of lymphocyte infiltration (LI) of colorectal carcinoma (CC) has been demonstrated by several groups. However, no validated test is currently available for clinical practice. We previously described an automated and reproducible method for testing LI and aimed to validate it for clinical use.

View Article and Find Full Text PDF

[Psoriatic patients: Analysis of patients dissatisfied with their management].

Ann Dermatol Venereol

June 2018

Service de dermatologie, centre hospitalier Victor-Dupouy, 69, rue du Lieutenant-Colonel Prud'hon, 95107 Argenteuil cedex, France.

Background: The French are frequently regarded as grouchy. In a recent study, we observed a high proportion of patients initially consulting for psoriasis because they were dissatisfied with their previous therapy. We analyzed the characteristics of these patients.

View Article and Find Full Text PDF

Background: Decision-making is a complex process. The aim of our study was to assess factors associated with the choice of the first biological treatment in patients with moderate-to-severe psoriasis.

Methods: Data on all patients included in the French prospective, observational, cohort, Psobioteq and initiating a first biologic prescription between July 2012 and July 2016 were analysed.

View Article and Find Full Text PDF

[Pathophysiology of detrusor underactivity in the elderly].

Prog Urol

June 2017

Sorbonne universités, UPMC université Paris 06, AP-HP, GRC 01, groupe de recherche clinique en neuro-urologie (GREEN), service de neuro-urologie, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France.

Aim: The aim of this study was to review the evidence regarding the pathophysiology of detrusor underactivity in the elderly.

Methods: A literature review was conducted in July 2016 using the Medline/Pubmed database limiting the search to works in English or French.

Results: The prevalence of detrusor underactivity has been reported to range from 8% to 48% depending on the definition used and the age of the population studied.

View Article and Find Full Text PDF