title: Congenital Aniridia Patient Questionnaire

acronym: Aniridia-Quizz

summary:
Congenital aniridia is a pan-ocular genetic disease characterized by a partial or complete absence of the iris, hence its name. The prevalence ranges from 1 / 40,000 to 1 / 96,000 births, but it may be underestimated. This condition combines several types of eye damage and could associate systemic manifestations, with a variable phenotype and genotype.

This study aims to identify eye and systemic manifestations in congenital aniridia and to determine the patients' knowledge of their own disease through a survey prepared by ophthalmologists from the Ophthalmology Department of Necker-Enfants Malades Hospital, reference center in France for this pathology. The patient fills it out only once.


title: Effect of Bilateral Transversus Thoracis Muscle Plane Block on Postoperative Morphine Consumption in Patients Undergoing Elective On-pump Coronary Artery Bypass Graft Surgery: a Retrospective Study.

acronym: PARACABS

summary:
Coronary artery bypass graft surgery is the standard surgical treatment for coronary disease. However, there is no consensus on analgesic management in patients undergoing CABG.

The aim of the study is to evualuate efficacy of bilateral transversus thoracis muscle plane (TTMP) block combined with systemic analgesia, compared to systemic analgesia only, in patients undergoing elective on-pump CABG surgery.

Our main hypothesis is that a bilateral TTMP block performed after CABG surgery could reduce morphine consumption during the first 48 hours.

The investigators conducted an age, gender and type of surgery-matched retrospective cohort study in the Montpellier University Hospital (France).

study_start_date_type: Actual

status: Completed

study_completion_date_type: Actual

study_type: Observational

time_perspective: Retrospective

enrollment_type: Actual

ipd_sharing: Undecided

ipd_sharing_description: NC

lead_sponsor: University Hospital, Montpellier

intervention_type: Procedure

lead_sponsor_type: academique

status_simplified: Completed


title: Monitoring of the Post-vaccination Immune Response to COVID-19 Among Hospital Staff

acronym: COVIDIM

summary:
Vaccination against COVID-19 is a major public health issue to control the epidemic and reduce the number of severe infections and deaths. Three vaccines currently received use authorization in France: two mRNA vaccines and one recombinant viral vector vaccine. Immunogenicity data demonstrate that they induce the development of neutralizing antibodies and IgG directed against the viral S1 protein and the receptor binding domain (RBD). However, there are currently no data on the persistence over time of the humoral response induced by vaccination, and data are still incomplete on cellular immunogenicity. No immune correlate of protection has been established for COVID-19.

In addition, these data come from assays carried out with current vaccines developed against a lineage of SARS-CoV-2 known as wild type (without genetic mutations).

The main objective of this study is to monitor in hospital staff the temporal evolution of humoral immunity induced by anti-COVID-19 vaccines by using enzyme-linked immunosorbent assays (ELISA), at 3, 6, 12 and 24 months after the last injection of vaccine.

status: Recruiting

study_completion_date_type: Anticipated

study_type: Interventional

primary_purpose: Prevention

enrollment_type: Anticipated

ReferenceType: derived

ReferenceCitation: Bonnet B, Chabrolles H, Archimbaud C, Brebion A, Cosme J, Dutheil F, Lambert C, Junda M, Mirand A, Ollier A, Pereira B, Regagnon C, Vidal M, Evrard B, Henquell C. Decline of Humoral and Cellular Immune Responses Against SARS-CoV-2 6 Months After Full BNT162b2 Vaccination in Hospital Healthcare Workers. Front Immunol. 2022 Mar 2;13:842912. doi: 10.3389/fimmu.2022.842912. eCollection 2022.

oa_colors: green gold

oa_host_type: publisher;repository

evidence: oa journal (via doaj)

host_type: publisher

license: cc-by

license_normalized: cc-by

version: publishedVersion

evidence: oa repository (via OAI-PMH doi match)

host_type: repository

repository_institution: Le Centre pour la Communication Scientifique Directe - HAL - Diderot

repository_normalized: HAL

repository_institution: Le Centre pour la Communication Scientifique Directe - HAL - Université de Nantes

repository_institution: Le Centre pour la Communication Scientifique Directe - HAL - Inria

evidence: oa repository (via pmcid lookup)

license_normalized: no license

repository_normalized: PubMed Central

repositories:
HAL
PubMed Central

repositories_concat: HAL;PubMed Central

unpaywall_oa_status: gold

publisher_dissemination: Frontiers

lead_sponsor: University Hospital, Clermont-Ferrand

intervention_type: Biological

status_simplified: Ongoing


title: Pharmaco-economical Study in Kidney Transplantation

acronym: EPHEGREN

summary:
The prevalence of renal acute rejection in transplantation is reduced thanks to the optimized use of immunosuppressive drugs. However, graft survival at ten years has not improved since then; this may be explained in part by the toxicity of these drugs, viral infections, and other comorbidities (diabetes, cardiovascular diseases …).

According to transplant center, strategies for the combination of immunosuppressive drugs are different, and probably involve a difference in the global cost of patient management. There is no recommendation in France to establish similar practices.

EPHEGREN is an observational, prospective, multicentric, pharmacoeconomic study for the renal transplant patients.

time_perspective: Prospective

lead_sponsor: University Hospital, Limoges


title: Contribution of ESAOTE™ Low Field MRI for Diagnosis of Scaphoid Fractures.

summary:
The Alpes Maritimes county administration (France) equipped Nice University Hospital of a 0,2T low field MRI dedicated to distal articulations, devoted to research, installed in the medical imaging unit.

The aim of our project is to study its interest in diagnosis of scaphoid fractures.

Conventional medical care for patients being suspected to have a scaphoid fracture consists in performing a clinical examination and a radiographic check-up with at least 4 incidences, which 2 specific incidences for the scaphoid bone.

Yet, this check up is sometimes defective, causing false positives unnecessary immobilisation and false negatives delays in patient medical care, increasing the risk of complication. Those diagnosis mistakes can bring serious consequences, from permanent partial inability, to professional reclassification.

Alternative imaging exists, but is often irradiating, and never reaches the optimal specificity and sensitivity.

MRI is totally safe, and allows to affirm or invalidate the diagnosis in almost 100% of cases. The problem is the lack of accessibility for emergency cases; therefore there is a great interest in validating the use of a MRI dedicated to distal articulations.

The few studies that study costs of diagnosis strategies including MRI in scaphoid bone fractures diagnosis show a reduction of medical costs if this technique is used early. Our project represents an essential initial step in evaluating the impact of a low field MRI dedicated to peripheral articulations in diagnosis of scaphoid fractures. The next step will be a medico-economical study, since scaphoid fractures occur frequently.

Finally, a new consensus for medical care of scaphoid fracture suspicions could be proposed.

primary_purpose: Diagnostic

lead_sponsor: Centre Hospitalier Universitaire de Nice

location_facility: Medical and Interventionnal Imaging Unit, Archet 2, Nice University Hospital

intervention_type: Radiation


type: EudraCT Number

title: Impact of Inhaled BGF 160 on Complexity and Variability of Tidal Breathing and Oscillatory Mechanics in Stable COPD Patient

acronym: VARI-OSCIL

summary: Multicentre, prospective, non-randomised, single-arm, open label, mechanistic study to investigate the mechanism of action of BGF 160 on ventilation pattern complexity and variability

primary_purpose: Treatment

lead_sponsor: University Hospital, Lille

sponsor_collaborators:
University Hospital, Lille
AstraZeneca

intervention_type: Drug


title: Validation of the French Translation of the Meaning of Life Questionnaire (MLQ) in Patients Admitted to the Crisis Centre

acronym: MSVCAC

summary:
Suicide is the highest mortality risk in psychiatry and about the only life-threatening risk associated with the evolution of mental illness. Worldwide, more than 800,000 people die by suicide each year. Although the number of deaths by suicide has decreased in France (from 11,000 in the 1990s to 9,000 today), suicide is still a major public health problem (the French rate is one of the highest in Europe).

The World Health Organisation (WHO) has declared a state of emergency and is pushing each country to develop a global multisectoral strategy for effective suicide prevention. To this end, suicide risk assessment tools have been developed for predictive purposes. However, diagnosis remains difficult and the risk of recidivism remains the fear of the psychiatrist. Questioning the subjects about their vision of their existence and the meaning they give to their life would perhaps help to better understand the psychology of the suicidal person. In recent decades, there has been research on meaning and suicide, and more recently a few studies have attempted to quantitatively investigate meaning as a protective factor. More recently, the COVID epidemic has brought the issue of meaning and suicide prevention to the forefront.

ipd_sharing: No

lead_sponsor: Centre Hospitalier Régional Metz-Thionville

location_facility:
CHR Metz-Thionville/Hopital de Mercy
Centre Hospitalier Jury

intervention_type: Behavioral


title: Is the Dynamic of the Pelvic Bone Altered After a Total Hip Replacement ?

acronym: DYPAMAT

summary:
Total hip replacement is one of the most common surgical procedure in France. In 2010, an estimated 147 513 total hip procedures were performed. This number is constantly increasing.

Although it is common, it can lead to many complications. The intra-prosthetic luxation is the second one after aseptic loosening.

The luxation risk is multifactorial and depends on the relative orientation of femoral et acetabular components. This orientation is influenced by statics and dynamics parameters.

So far, the literature shows that only static parameters are considered by surgeons. Thus, in reference to Lewinneck publication, which states that the luxation risk is lower if the cup is oriented with 15° +/- 10° of anteversion and 40°+/- 10° of inclination with respect to the anterior pelvic plan (APP). Defined by the two anterosuperior iliacs spines and pubic symphysis.

However, this approach is only static and do not take into account the variations of pelvic plan orientation during everyday life.

To consider these variations of pelvic inclination, it is possible to measure the angle between APP and the horizontal (in lying position) or the vertical (in standing position) plan.

Several devices allow the measure of pelvic inclination but they all have important limits (EOS radiography, scanner or navigation). A new device has been developed in Brest to measure this inclination in several positions of the daily life.This software has already been tested in healthy volunteers and results demonstrate an excellent accuracy and reproducibility.

The goal of this study is to described the dynamic of the pelvic bone thanks to this ultrasound based device, and to assess the amount of variation induced by hip replacement procedures.

status: Unknown status

primary_purpose: Other

ipd_sharing: Yes

ipd_sharing_description: All collected data that underlie results in a publication.

lead_sponsor: University Hospital, Brest

intervention_type: Other

status_simplified: Unknown


title: Continuous Measurement of the Activity for Clinical Evaluation at Home, for Non-ambulant Neuromuscular Patients

acronym: Pre-Acti

summary:
Validation of a measure specifically suitable to evaluate the residual function of upper limbs of non-ambulant neuromuscular patients, especially with Duchenne muscular dystrophy and spinal muscular atrophy.

This approach is of fundamental importance for clinical monitoring and preparing the future therapeutic trials.

primary_purpose: Basic Science

ReferenceCitation: Le Moing AG, Seferian AM, Moraux A, Annoussamy M, Dorveaux E, Gasnier E, Hogrel JY, Voit T, Vissiere D, Servais L. A Movement Monitor Based on Magneto-Inertial Sensors for Non-Ambulant Patients with Duchenne Muscular Dystrophy: A Pilot Study in Controlled Environment. PLoS One. 2016 Jun 7;11(6):e0156696. doi: 10.1371/journal.pone.0156696. eCollection 2016.

repository_institution: PubMed Central - Europe PMC

evidence: oa repository (via OAI-PMH title and first author match)

repository_institution: University College London - UCL Discovery

repository_normalized: UCL Discovery

repository_institution: University of Illinois at Chicago - INDIGO

version: submittedVersion

repositories:
PubMed Central
UCL Discovery
figshare.com

publisher_dissemination: Public Library of Science

lead_sponsor: Institut de Myologie, France

location_facility: Institut de Myologie - GH Pitié Salpétrière

lead_sponsor_type: industriel


title: Prospective Follow-up of Patients With Glycogen Storage Disease Type III

acronym: PRO GSDIII

summary: The aim of this study is to improve knowledge of natural history and methods of monitoring the evolution of Glycogen storage disease type III regarding the muscle and to study the prospective approach of large series of patients, and using the same protocol for the follow up of the children and adults.


title: Assessment of Accelerometric Measurement Devices for Gait Analysis

acronym: ACCELERIX

summary: The objective of this study is to obtain normative accelerometry data during the six minute walk test and a 30 second walk test on healthy subjects in order to determine the accelerometric variables that are the most relevant to describe gait disorders in patient populations. Secondary objectives of this study are to investigate the impact of walking speed and muscle strength on the accelerometric parameters, to assess the reproducibility of the method and to compare accelerometric data obtained with two different devices.

location_facility: Institut de Myologie - GH Pitié Salpêtrière


title: Evaluation of Muscle Strength and Inflammatory Response in Patients With Inclusion Body Myositis

acronym: IBMan

summary: Comparison of a group of 20 IBM patients with 20 controls matched on age, gender and weight.

sponsor_collaborators:
Institut de Myologie, France
Assistance Publique - Hôpitaux de Paris


title: Usefulness of Pro-adrenomedullin (MR proADM)as a Predictor of Outcome in Acute Exacerbations of COPD Visiting the Emergency Room

acronym: UTAPE BPCO

summary: The purpose of this study is to determine whether pro-adrenomedullin (Mr proADM)in addition to clinical evaluation is effective to predict outcome of acute exacerbations of COPD patients visiting the emergency room(ER).

lead_sponsor: ThermoFisher Scientific Brahms Biomarkers France

location_facility:
Pitié Salpétrière Hospital
Hôpital Lariboisière


title: Validation of the French Translation of the Scale HSCL25 in the Diagnosis of Depression in Primary Care

summary: Depression has emotional consequences, social and important business. Reaching at least 7.5% of French aged 15 to 85 years. General Practitioners are the first professional consulted in terms of frequency of consultations. Indeed, in primary care, 10 to 25% of patients with depression that General Practitioners are often alone to support and follow. But General Practitioners are faced with diagnostic difficulties facing the diagnosis of depression, because if the patient does not engage itself, no tool is validated in France to help them in their diagnosis. However, standardized questionary exist that allow to overcome this difficulty. Questionary used in other european countries. Among them, the HSCL25 scale was selected by a systematic review of literature and a RAND UCLA type of consensus procedure. This HSCL25 scale is the most efficient for the diagnosis of depression in terms of efficiency tool, reproducibility and ergonomics. However, it remains necessary to confirm its validity in terms of the French translation and in terms of efficiency.

design_allocation: Randomized

location_facility: Cabinet médical en zone rurale - Pôle Santé Universitaire


type: Other Identifier

title: Assessment of Severe Depression Related to Psychotrauma in the COVID-19 Context in Patients Followed in Psychiatry in the Department of Eure

acronym: COVID-SME

summary: Research Hypothesis: Living conditions during COVID-19, and lockdowns and curfews impact the psychological state of patients (assessed by the degree of depression, positive and negative thoughts, insomnia, state of post-traumatic stress).

lead_sponsor: Centre Hospitalier Eure-Seine

sponsor_collaborators:
Centre Hospitalier Eure-Seine
New Hospital of Navarre


title: Quality of Life and Psychosocial Dimensions in Children With Congenital or Acquired Hypopituitarism

acronym: QALHYPO

summary: Relatively little is known about the quality of life (QOL) of patients with hypopituitarism, particularly children. Quality of life studies have mostly focused on improvements following the initiation of growth hormone treatment in adults with growth hormone deficiency. Overall QOL data on patients with multiple pituitary hormone deficiency or another isolated deficiency are much scarcer. These studies suggest however that pituitary deficiencies affect the QOL and social integration of adults. In children, the psychosocial repercussions of the disease are very poorly understood, particularly in the case of multiple congenital pituitary hormone deficiency.

study_start_date_type: Anticipated

status: Not yet recruiting

lead_sponsor: Assistance Publique Hopitaux De Marseille


title: Etude Descriptive Retrospective de la Transfusion Plaquettaire Chez Des Patients Atteints d'hémopathie Maligne en Phase Palliative et Suivis au CHU de Besançon

summary:
Development of palliative care is less effective for patients with hematologic malignancies. Limited data is available for end of life care in this population, moreover with thrombocytopenic patients. Thrombopenia is a frequent complication, specific of bone marrow involvement in those diseases or its treatments. Yet, a few studies was interested in, whereas platelet transfusion is the only treatment indicated. As it represent a scarce, limited resource, the ethical principles are in conflict in this setting.

The purpose of this study was to describe retrospectively platelet transfusion in the six last few month of life of patients with hematologic malignancies at the CHU of Besançon (France) between 01/07/15 and 31/12/16.

ReferenceType: background

ReferenceCitation: Hochsmann B, Moicean A, Risitano A, Ljungman P, Schrezenmeier H. Supportive care in severe and very severe aplastic anemia. Bone Marrow Transplant. 2013 Feb;48(2):168-73. doi: 10.1038/bmt.2012.220. Epub 2012 Dec 3.

ReferenceCitation: Sherbeck JP, Boss RD. Ethical Questions about Platelet Transfusions at the End of Life. AMA J Ethics. 2016 Aug 1;18(8):764-70. doi: 10.1001/journalofethics.2016.18.8.ecas1-1608.

ReferenceCitation: Salacz ME, Lankiewicz MW, Weissman DE. Management of thrombocytopenia in bone marrow failure: a review. J Palliat Med. 2007 Feb;10(1):236-44. doi: 10.1089/jpm.2006.0126.

ReferenceCitation: Lassauniere JM, Bertolino M, Hunault M, Zittoun R, Verspieren P, Moh-Klaren J, Jaulmes D, Colombat P. Platelet transfusions in advanced hematological malignancies: a position paper. J Palliat Care. 1996 Spring;12(1):38-41.

ReferenceCitation: Boggs DR. Jehovah's Witnesses with leukemia. Hosp Pract (Off Ed). 1985 Mar 15;20(3):92, 94-5, 98 passim. doi: 10.1080/21548331.1985.11703015. No abstract available.

ReferenceType: result

ReferenceCitation: Wang WS, Ma JD, Nelson SH, Revta C, Buckholz GT, Mulroney C, Roeland EJ. Transfusion practices at end of life for hematopoietic stem cell transplant patients. Support Care Cancer. 2018 Jun;26(6):1927-1931. doi: 10.1007/s00520-017-4023-y. Epub 2017 Dec 28.

oa_host_type: repository

version: acceptedVersion

repositories_concat: PubMed Central

unpaywall_oa_status: green

publisher_dissemination: Springer-Nature

ipd_sharing_description: patient were dead during the period of recruitment. Characteristic of patient could be used for other purpose

lead_sponsor: Centre Hospitalier Universitaire de Besancon


title: SCOPYD Study: Development of a Predictive Clinical Score of Hypovitaminosis D

acronym: SCOPYD

summary:
Vitamin D has effects on many tissues, and hypovitaminosis D is frequent. In a French survey conducted among 1587 adults, vitamin D insufficiency (<30ng/ml) has been reported in 80% of subjects, including 43% with moderate deficiency (<20ng/ml) and 5% with severe deficiency (<10ng/ml).

Because of the possible consequences of hypovitaminosis D (osteomalacia in adults…), the number of vitamin D determination has increased ten-fold since 2005 in France, reaching 4.5 million € in 2011, and with it the costs for health insurance. However, there is currently no consensus on the strategy for detection, diagnosis and treatment of hypovitaminosis D.

We propose to develop a predictive clinical score of hypovitaminosis D based on the accurate assessment of solar exposure, vitamin D intakes and hypovitaminosis D risk factors collected through a self-administered questionnaire.

lead_sponsor: Hospices Civils de Lyon

appel_a_projets: PHRCI

region: Auvergne-Rhône-Alpes

nom_etablissement: HOSPICES CIVILS DE LYON

type_etablissement: CHUR

acronyme: SCOPYD

titre: Développement d'un score clinique de prédiction de l'hypovitaminose D

discipline_principale: autre

nom_porteur: SCHOTT

prenom_porteur: Anne Marie


title: Dance Workshop for Institutionalized Seniors With Behavioral and Psychological Symptoms of Dementia

acronym: APAISE

summary:
It is estimated that the prevalence of dementia in France is 5% for people over 65 years of age and that this increases to 18% for people over 75 years of age. Behavioral disorders are frequent in patients with dementia and are the main cause of institutionalization, representing up to 50% of cases. Dementia syndromes can be translated into behavioral and psychological symptoms of dementia (BPSD), which are defined as perceptual, mood or behavioral disorders. To date, there is no cure for dementia, but certain measures can be taken to reduce symptoms. The HAS suggests that physical exercise would reduce BPSD and could improve the ability to perform activities of daily living or slow down the cognitive decline of this type of patient. Indeed, several articles have highlighted the benefits of physical activity in demented patients. A meta-analysis has shown a significant decrease in BPSD via the Neuropsychiatric Inventory (NPI) score. Nevertheless, it is often observed that there is a difficulty in adhering to the program in very elderly population groups. Dance is a complete physical activity that integrates physical, cognitive and social elements. A meta-analysis has shown that dance has a significant effect on global cognition but also on neuroplasticity and physical functioning in patients with mental disorders. The music component of dance also has an effect on the behavioral problems of demented patients, stimulating language ability and social and emotional function.

However, at present, no study of good methodological quality has been able to demonstrate the effectiveness of dance-based physical activity for the treatment of dementia symptoms. As a result of these findings, the APAISE project was developed and should help slow the onset of BPSD in patients with dementia.

lead_sponsor: Centre Hospitalier Emile Roux


type: Registry Identifier

title: Clinical and Genetic Analysis of Autosomal Recessive Forms of Cerebellar Ataxias and Spastic Paraplegias

acronym: Spatax

summary: Cerebellar ataxias (CA) and spastic paraplegias (SP) are genetically and clinically very heterogeneous. More than 40 loci are already known but the number of phenotypes is even greater suggesting further genetic heterogeneity. These progressive disorders are often severe and fatal, due to the absence of specific therapy. The SPATAX network combines the experience of European clinicians and scientists working on these groups of diseases. Over the past year, they have assembled the largest collection of families and achieved a number of tasks (initiation of a clinical and genetic database, distribution of DNA to participating laboratories, mapping of three new loci, and refinement of several loci). In addition to clinicians from Europe and Mediterranean countries, who play a major role in collecting families according to evaluation tools developed and validated by the SPATAX members, the group includes major European laboratories devoted to the elucidation of the molecular basis of these disorders. Each laboratory will centralize all families with a subtype of autosomal recessive (AR) CA (n=116) or SP (n=207) in order to efficiently map and identify the responsible gene(s). Genome-wide scans are already underway in 61 families. Given the expertise of the participants, the researchers expect to map and identify several genes during the course of this project. The spectrum of mutations and phenotype/genotype correlations will be analysed thanks to this unique series of patients with various phenotypes. The knowledge gained will be immediately applicable to patients in terms of improved positive diagnosis, follow-up and appropriate genetic counselling. In the long term, models for genetic entity will be developed in order to understand the pathophysiology and to identify new targets for treatment. The series of patients assembled and the precise knowledge of natural history will facilitate the implantation of therapeutic trials based on rational approaches.

ReferenceCitation: Moreira MC, Klur S, Watanabe M, Nemeth AH, Le Ber I, Moniz JC, Tranchant C, Aubourg P, Tazir M, Schols L, Pandolfo M, Schulz JB, Pouget J, Calvas P, Shizuka-Ikeda M, Shoji M, Tanaka M, Izatt L, Shaw CE, M'Zahem A, Dunne E, Bomont P, Benhassine T, Bouslam N, Stevanin G, Brice A, Guimaraes J, Mendonca P, Barbot C, Coutinho P, Sequeiros J, Durr A, Warter JM, Koenig M. Senataxin, the ortholog of a yeast RNA helicase, is mutant in ataxia-ocular apraxia 2. Nat Genet. 2004 Mar;36(3):225-7. doi: 10.1038/ng1303. Epub 2004 Feb 8.

ReferenceCitation: Le Ber I, Bouslam N, Rivaud-Pechoux S, Guimaraes J, Benomar A, Chamayou C, Goizet C, Moreira MC, Klur S, Yahyaoui M, Agid Y, Koenig M, Stevanin G, Brice A, Durr A. Frequency and phenotypic spectrum of ataxia with oculomotor apraxia 2: a clinical and genetic study in 18 patients. Brain. 2004 Apr;127(Pt 4):759-67. doi: 10.1093/brain/awh080. Epub 2004 Jan 21.

ReferenceCitation: Le Ber I, Moreira MC, Rivaud-Pechoux S, Chamayou C, Ochsner F, Kuntzer T, Tardieu M, Said G, Habert MO, Demarquay G, Tannier C, Beis JM, Brice A, Koenig M, Durr A. Cerebellar ataxia with oculomotor apraxia type 1: clinical and genetic studies. Brain. 2003 Dec;126(Pt 12):2761-72. doi: 10.1093/brain/awg283. Epub 2003 Sep 23.

ReferenceCitation: Le Ber I, Camuzat A, Castelnovo G, Azulay JP, Genton P, Gastaut JL, Broglin D, Labauge P, Brice A, Durr A. Prevalence of dentatorubral-pallidoluysian atrophy in a large series of white patients with cerebellar ataxia. Arch Neurol. 2003 Aug;60(8):1097-9. doi: 10.1001/archneur.60.8.1097.

ReferenceCitation: Bouslam N, Benomar A, Azzedine H, Bouhouche A, Namekawa M, Klebe S, Charon C, Durr A, Ruberg M, Brice A, Yahyaoui M, Stevanin G. Mapping of a new form of pure autosomal recessive spastic paraplegia (SPG28). Ann Neurol. 2005 Apr;57(4):567-71. doi: 10.1002/ana.20416.

ReferenceCitation: Durr A, Camuzat A, Colin E, Tallaksen C, Hannequin D, Coutinho P, Fontaine B, Rossi A, Gil R, Rousselle C, Ruberg M, Stevanin G, Brice A. Atlastin1 mutations are frequent in young-onset autosomal dominant spastic paraplegia. Arch Neurol. 2004 Dec;61(12):1867-72. doi: 10.1001/archneur.61.12.1867.

ReferenceCitation: Stevanin G, Durr A, Dussert C, Penet C, Brice A. Mutations in the FGF14 gene are not a major cause of spinocerebellar ataxia in Caucasians. Neurology. 2004 Sep 14;63(5):936. doi: 10.1212/01.wnl.0000137020.30604.1e. No abstract available.

ReferenceCitation: Stevanin G, Hahn V, Lohmann E, Bouslam N, Gouttard M, Soumphonphakdy C, Welter ML, Ollagnon-Roman E, Lemainque A, Ruberg M, Brice A, Durr A. Mutation in the catalytic domain of protein kinase C gamma and extension of the phenotype associated with spinocerebellar ataxia type 14. Arch Neurol. 2004 Aug;61(8):1242-8. doi: 10.1001/archneur.61.8.1242.

ReferenceCitation: Stevanin G, Bouslam N, Thobois S, Azzedine H, Ravaux L, Boland A, Schalling M, Broussolle E, Durr A, Brice A. Spinocerebellar ataxia with sensory neuropathy (SCA25) maps to chromosome 2p. Ann Neurol. 2004 Jan;55(1):97-104. doi: 10.1002/ana.10798.

ReferenceCitation: Tallaksen CM, Guichart-Gomez E, Verpillat P, Hahn-Barma V, Ruberg M, Fontaine B, Brice A, Dubois B, Durr A. Subtle cognitive impairment but no dementia in patients with spastin mutations. Arch Neurol. 2003 Aug;60(8):1113-8. doi: 10.1001/archneur.60.8.1113.

ReferenceCitation: Fernet M, Gribaa M, Salih MA, Seidahmed MZ, Hall J, Koenig M. Identification and functional consequences of a novel MRE11 mutation affecting 10 Saudi Arabian patients with the ataxia telangiectasia-like disorder. Hum Mol Genet. 2005 Jan 15;14(2):307-18. doi: 10.1093/hmg/ddi027. Epub 2004 Dec 1.

ReferenceCitation: Stevanin G, Durr A, Benammar N, Brice A. Spinocerebellar ataxia with mental retardation (SCA13). Cerebellum. 2005;4(1):43-6. doi: 10.1080/14734220510007923.

ReferenceCitation: Stevanin G, Broussolle E, Streichenberger N, Kopp N, Brice A, Durr A. Spinocerebellar ataxia with sensory neuropathy (SCA25). Cerebellum. 2005;4(1):58-61. doi: 10.1080/14734220510007932.

ReferenceCitation: Klebe S, Durr A, Rentschler A, Hahn-Barma V, Abele M, Bouslam N, Schols L, Jedynak P, Forlani S, Denis E, Dussert C, Agid Y, Bauer P, Globas C, Wullner U, Brice A, Riess O, Stevanin G. New mutations in protein kinase Cgamma associated with spinocerebellar ataxia type 14. Ann Neurol. 2005 Nov;58(5):720-9. doi: 10.1002/ana.20628.

ReferenceCitation: Latouche M, Fragner P, Martin E, El Hachimi KH, Zander C, Sittler A, Ruberg M, Brice A, Stevanin G. Polyglutamine and polyalanine expansions in ataxin7 result in different types of aggregation and levels of toxicity. Mol Cell Neurosci. 2006 Mar;31(3):438-45. doi: 10.1016/j.mcn.2005.10.013. Epub 2005 Dec 1.

ReferenceCitation: Namekawa M, Ribai P, Nelson I, Forlani S, Fellmann F, Goizet C, Depienne C, Stevanin G, Ruberg M, Durr A, Brice A. SPG3A is the most frequent cause of hereditary spastic paraplegia with onset before age 10 years. Neurology. 2006 Jan 10;66(1):112-4. doi: 10.1212/01.wnl.0000191390.20564.8e.

ReferenceCitation: van de Warrenburg BP, Hendriks H, Durr A, van Zuijlen MC, Stevanin G, Camuzat A, Sinke RJ, Brice A, Kremer BP. Age at onset variance analysis in spinocerebellar ataxias: a study in a Dutch-French cohort. Ann Neurol. 2005 Apr;57(4):505-12. doi: 10.1002/ana.20424.

ReferenceCitation: Biancalana V, Toft M, Le Ber I, Tison F, Scherrer E, Thibodeau S, Mandel JL, Brice A, Farrer MJ, Durr A. FMR1 premutations associated with fragile X-associated tremor/ataxia syndrome in multiple system atrophy. Arch Neurol. 2005 Jun;62(6):962-6. doi: 10.1001/archneur.62.6.962.

ReferenceCitation: Ribaï P, Stevanin G, Trefouret S, Nelson I, Soumphonphakdy C, Pouget J, Dürr A, Brice A. Extension of the SPG26 phenotype in a Spanish family and refinement of its locus on chromosome 12. Eletter, Journal of Medical Genetics 42(1):80-82, 2005.

ReferenceCitation: Elleuch N, Depienne C, Benomar A, Hernandez AM, Ferrer X, Fontaine B, Grid D, Tallaksen CM, Zemmouri R, Stevanin G, Durr A, Brice A. Mutation analysis of the paraplegin gene (SPG7) in patients with hereditary spastic paraplegia. Neurology. 2006 Mar 14;66(5):654-9. doi: 10.1212/01.wnl.0000201185.91110.15.

ReferenceCitation: Le Ber I, Rivaud-Pechoux S, Brice A, Durr A. [Autosomal recessive cerebellar ataxias with oculomotor apraxia]. Rev Neurol (Paris). 2006 Feb;162(2):177-84. doi: 10.1016/s0035-3787(06)74997-9. French.

ReferenceCitation: Namekawa M, Nelson I, Ribai P, Durr A, Denis E, Stevanin G, Ruberg M, Brice A. A founder effect and mutational hot spots may contribute to the most frequent mutations in the SPG3A gene. Neurogenetics. 2006 May;7(2):131-2. doi: 10.1007/s10048-006-0028-2. Epub 2006 Apr 13. No abstract available.

ReferenceCitation: Lossos A, Stevanin G, Meiner V, Argov Z, Bouslam N, Newman JP, Gomori JM, Klebe S, Lerer I, Elleuch N, Silverstein S, Durr A, Abramsky O, Ben-Nariah Z, Brice A. Hereditary spastic paraplegia with thin corpus callosum: reduction of the SPG11 interval and evidence for further genetic heterogeneity. Arch Neurol. 2006 May;63(5):756-60. doi: 10.1001/archneur.63.5.756.

ReferenceCitation: Klebe S, Azzedine H, Durr A, Bastien P, Bouslam N, Elleuch N, Forlani S, Charon C, Koenig M, Melki J, Brice A, Stevanin G. Autosomal recessive spastic paraplegia (SPG30) with mild ataxia and sensory neuropathy maps to chromosome 2q37.3. Brain. 2006 Jun;129(Pt 6):1456-62. doi: 10.1093/brain/awl012. Epub 2006 Jan 24.

ReferenceCitation: Ribai P, Stevanin G, Bouslam N, Pontier B, Nelson I, Fontaine B, Dussert C, Charon C, Durr A, Brice A. A new phenotype linked to SPG27 and refinement of the critical region on chromosome. J Neurol. 2006 Jun;253(6):714-9. doi: 10.1007/s00415-006-0094-2. Epub 2006 Mar 6.

ReferenceCitation: Le Ber I, Clot F, Vercueil L, Camuzat A, Viemont M, Benamar N, De Liege P, Ouvrard-Hernandez AM, Pollak P, Stevanin G, Brice A, Durr A. Predominant dystonia with marked cerebellar atrophy: a rare phenotype in familial dystonia. Neurology. 2006 Nov 28;67(10):1769-73. doi: 10.1212/01.wnl.0000244484.60489.50.

ReferenceCitation: Stevanin G, Montagna G, Azzedine H, Valente EM, Durr A, Scarano V, Bouslam N, Cassandrini D, Denora PS, Criscuolo C, Belarbi S, Orlacchio A, Jonveaux P, Silvestri G, Hernandez AM, De Michele G, Tazir M, Mariotti C, Brockmann K, Malandrini A, van der Knapp MS, Neri M, Tonekaboni H, Melone MA, Tessa A, Dotti MT, Tosetti M, Pauri F, Federico A, Casali C, Cruz VT, Loureiro JL, Zara F, Forlani S, Bertini E, Coutinho P, Filla A, Brice A, Santorelli FM. Spastic paraplegia with thin corpus callosum: description of 20 new families, refinement of the SPG11 locus, candidate gene analysis and evidence of genetic heterogeneity. Neurogenetics. 2006 Jul;7(3):149-56. doi: 10.1007/s10048-006-0044-2. Epub 2006 May 13.

ReferenceCitation: Klebe S, Lacour A, Durr A, Stojkovic T, Depienne C, Forlani S, Poea-Guyon S, Vuillaume I, Sablonniere B, Vermersch P, Brice A, Stevanin G. NIPA1 (SPG6) mutations are a rare cause of autosomal dominant spastic paraplegia in Europe. Neurogenetics. 2007 Apr;8(2):155-7. doi: 10.1007/s10048-006-0074-9. Epub 2007 Jan 5. No abstract available.

ReferenceCitation: Bouslam N, Bouhouche A, Benomar A, Hanein S, Klebe S, Azzedine H, Di Giandomenico S, Boland-Auge A, Santorelli FM, Durr A, Brice A, Yahyaoui M, Stevanin G. A novel locus for autosomal recessive spastic ataxia on chromosome 17p. Hum Genet. 2007 May;121(3-4):413-20. doi: 10.1007/s00439-007-0328-0. Epub 2007 Feb 2.

ReferenceCitation: Stevanin G, Santorelli FM, Azzedine H, Coutinho P, Chomilier J, Denora PS, Martin E, Ouvrard-Hernandez AM, Tessa A, Bouslam N, Lossos A, Charles P, Loureiro JL, Elleuch N, Confavreux C, Cruz VT, Ruberg M, Leguern E, Grid D, Tazir M, Fontaine B, Filla A, Bertini E, Durr A, Brice A. Mutations in SPG11, encoding spatacsin, are a major cause of spastic paraplegia with thin corpus callosum. Nat Genet. 2007 Mar;39(3):366-72. doi: 10.1038/ng1980. Epub 2007 Feb 18.

ReferenceCitation: Latouche M, Lasbleiz C, Martin E, Monnier V, Debeir T, Mouatt-Prigent A, Muriel MP, Morel L, Ruberg M, Brice A, Stevanin G, Tricoire H. A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes. J Neurosci. 2007 Mar 7;27(10):2483-92. doi: 10.1523/JNEUROSCI.5453-06.2007.

ReferenceCitation: Klebe S, Durr A, Bouslam N, Grid D, Paternotte C, Depienne C, Hanein S, Bouhouche A, Elleuch N, Azzedine H, Poea-Guyon S, Forlani S, Denis E, Charon C, Hazan J, Brice A, Stevanin G. Spastic paraplegia 5: Locus refinement, candidate gene analysis and clinical description. Am J Med Genet B Neuropsychiatr Genet. 2007 Oct 5;144B(7):854-61. doi: 10.1002/ajmg.b.30518.

ReferenceCitation: Stevanin G, Paternotte C, Coutinho P, Klebe S, Elleuch N, Loureiro JL, Denis E, Cruz VT, Durr A, Prud'homme JF, Weissenbach J, Brice A, Hazan J. A new locus for autosomal recessive spastic paraplegia (SPG32) on chromosome 14q12-q21. Neurology. 2007 May 22;68(21):1837-40. doi: 10.1212/01.wnl.0000262043.53386.22.

ReferenceCitation: Namekawa M, Muriel MP, Janer A, Latouche M, Dauphin A, Debeir T, Martin E, Duyckaerts C, Prigent A, Depienne C, Sittler A, Brice A, Ruberg M. Mutations in the SPG3A gene encoding the GTPase atlastin interfere with vesicle trafficking in the ER/Golgi interface and Golgi morphogenesis. Mol Cell Neurosci. 2007 May;35(1):1-13. doi: 10.1016/j.mcn.2007.01.012. Epub 2007 Jan 26.

ReferenceCitation: Stevanin G, Azzedine H, Denora P, Boukhris A, Tazir M, Lossos A, Rosa AL, Lerer I, Hamri A, Alegria P, Loureiro J, Tada M, Hannequin D, Anheim M, Goizet C, Gonzalez-Martinez V, Le Ber I, Forlani S, Iwabuchi K, Meiner V, Uyanik G, Erichsen AK, Feki I, Pasquier F, Belarbi S, Cruz VT, Depienne C, Truchetto J, Garrigues G, Tallaksen C, Tranchant C, Nishizawa M, Vale J, Coutinho P, Santorelli FM, Mhiri C, Brice A, Durr A; SPATAX consortium. Mutations in SPG11 are frequent in autosomal recessive spastic paraplegia with thin corpus callosum, cognitive decline and lower motor neuron degeneration. Brain. 2008 Mar;131(Pt 3):772-84. doi: 10.1093/brain/awm293. Epub 2007 Dec 13.

ReferenceCitation: Boukhris A, Feki I, Denis E, Miladi MI, Brice A, Mhiri C, Stevanin G. Spastic paraplegia 15: linkage and clinical description of three Tunisian families. Mov Disord. 2008 Feb 15;23(3):429-33. doi: 10.1002/mds.21848.

ReferenceCitation: Boukhris A, Stevanin G, Feki I, Denis E, Elleuch N, Miladi MI, Truchetto J, Denora P, Belal S, Mhiri C, Brice A. Hereditary spastic paraplegia with mental impairment and thin corpus callosum in Tunisia: SPG11, SPG15, and further genetic heterogeneity. Arch Neurol. 2008 Mar;65(3):393-402. doi: 10.1001/archneur.65.3.393.

ReferenceCitation: Hanein S, Martin E, Boukhris A, Byrne P, Goizet C, Hamri A, Benomar A, Lossos A, Denora P, Fernandez J, Elleuch N, Forlani S, Durr A, Feki I, Hutchinson M, Santorelli FM, Mhiri C, Brice A, Stevanin G. Identification of the SPG15 gene, encoding spastizin, as a frequent cause of complicated autosomal-recessive spastic paraplegia, including Kjellin syndrome. Am J Hum Genet. 2008 Apr;82(4):992-1002. doi: 10.1016/j.ajhg.2008.03.004.

ReferenceCitation: Denora PS, Schlesinger D, Casali C, Kok F, Tessa A, Boukhris A, Azzedine H, Dotti MT, Bruno C, Truchetto J, Biancheri R, Fedirko E, Di Rocco M, Bueno C, Malandrini A, Battini R, Sickl E, de Leva MF, Boespflug-Tanguy O, Silvestri G, Simonati A, Said E, Ferbert A, Criscuolo C, Heinimann K, Modoni A, Weber P, Palmeri S, Plasilova M, Pauri F, Cassandrini D, Battisti C, Pini A, Tosetti M, Hauser E, Masciullo M, Di Fabio R, Piccolo F, Denis E, Cioni G, Massa R, Della Giustina E, Calabrese O, Melone MA, De Michele G, Federico A, Bertini E, Durr A, Brockmann K, van der Knaap MS, Zatz M, Filla A, Brice A, Stevanin G, Santorelli FM. Screening of ARHSP-TCC patients expands the spectrum of SPG11 mutations and includes a large scale gene deletion. Hum Mutat. 2009 Mar;30(3):E500-19. doi: 10.1002/humu.20945.

ReferenceCitation: Denora PS, Muglia M, Casali C, Truchetto J, Silvestri G, Messina D, Boukrhis A, Magariello A, Modoni A, Masciullo M, Malandrini A, Morelli M, de Leva MF, Villanova M, Giugni E, Citrigno L, Rizza T, Federico A, Pierallini A, Quattrone A, Filla A, Brice A, Stevanin G, Santorelli FM. Spastic paraplegia with thinning of the corpus callosum and white matter abnormalities: further mutations and relative frequency in ZFYVE26/SPG15 in the Italian population. J Neurol Sci. 2009 Feb 15;277(1-2):22-5. doi: 10.1016/j.jns.2008.09.039. Epub 2008 Dec 13.

ReferenceCitation: Boukhris A, Stevanin G, Feki I, Denora P, Elleuch N, Miladi MI, Goizet C, Truchetto J, Belal S, Brice A, Mhiri C. Tunisian hereditary spastic paraplegias: clinical variability supported by genetic heterogeneity. Clin Genet. 2009 Jun;75(6):527-36. doi: 10.1111/j.1399-0004.2009.01176.x. Epub 2009 May 5.

ReferenceCitation: Anheim M, Lagier-Tourenne C, Stevanin G, Fleury M, Durr A, Namer IJ, Denora P, Brice A, Mandel JL, Koenig M, Tranchant C. SPG11 spastic paraplegia. A new cause of juvenile parkinsonism. J Neurol. 2009 Jan;256(1):104-8. doi: 10.1007/s00415-009-0083-3. Epub 2009 Feb 9.

ReferenceCitation: Goizet C, Boukhris A, Durr A, Beetz C, Truchetto J, Tesson C, Tsaousidou M, Forlani S, Guyant-Marechal L, Fontaine B, Guimaraes J, Isidor B, Chazouilleres O, Wendum D, Grid D, Chevy F, Chinnery PF, Coutinho P, Azulay JP, Feki I, Mochel F, Wolf C, Mhiri C, Crosby A, Brice A, Stevanin G. CYP7B1 mutations in pure and complex forms of hereditary spastic paraplegia type 5. Brain. 2009 Jun;132(Pt 6):1589-600. doi: 10.1093/brain/awp073. Epub 2009 May 12.

ReferenceCitation: Goizet C, Boukhris A, Maltete D, Guyant-Marechal L, Truchetto J, Mundwiller E, Hanein S, Jonveaux P, Roelens F, Loureiro J, Godet E, Forlani S, Melki J, Auer-Grumbach M, Fernandez JC, Martin-Hardy P, Sibon I, Sole G, Orignac I, Mhiri C, Coutinho P, Durr A, Brice A, Stevanin G. SPG15 is the second most common cause of hereditary spastic paraplegia with thin corpus callosum. Neurology. 2009 Oct 6;73(14):1111-9. doi: 10.1212/WNL.0b013e3181bacf59.

ReferenceCitation: Anheim M, Monga B, Fleury M, Charles P, Barbot C, Salih M, Delaunoy JP, Fritsch M, Arning L, Synofzik M, Schols L, Sequeiros J, Goizet C, Marelli C, Le Ber I, Koht J, Gazulla J, De Bleecker J, Mukhtar M, Drouot N, Ali-Pacha L, Benhassine T, Chbicheb M, M'Zahem A, Hamri A, Chabrol B, Pouget J, Murphy R, Watanabe M, Coutinho P, Tazir M, Durr A, Brice A, Tranchant C, Koenig M. Ataxia with oculomotor apraxia type 2: clinical, biological and genotype/phenotype correlation study of a cohort of 90 patients. Brain. 2009 Oct;132(Pt 10):2688-98. doi: 10.1093/brain/awp211. Epub 2009 Aug 20.

lead_sponsor: Institut National de la Santé Et de la Recherche Médicale, France

collaborators:
Institut des Maladies Rares
National Research Agency, France

location_country:
Italy
Portugal
Israel
Norway
Algeria
Denmark
United Kingdom
Netherlands
Lebanon
Belgium
Tunisia
Saudi Arabia
Morocco
Serbia
France

location_facility: Royal Free and University College Medical School

location_facility: Molecular Medicine and Department of Neurosciences

location_facility: Université Libre de Bruxelles - Hôpital Erasme

location_facility: Radboud University Nijmegen Medical Centre


title: Workshops and Exchange Groups for Laryngectomized Patients

acronym: GELAT

summary:
This research is conducted with the aim of improving the quality of life of laryngectomized patients and their entourage. Worldwide, there are 185,000 new cases of laryngeal cancer per year. In Europe, between 2000 and 2007, the crude annual incidence rates of these cancers were 4.6/100,000 with a 5-year survival rate of 61%.

In France, about 30 to 35% of cancers of the upper aerodigestive tract are localized to the larynx, or about 43,000 cases per year. Most patients are men (89%) between 50 and 70 years old. One of the treatments for these cancers is to perform an excision of the larynx thus removing the entire tumor, it is the total laryngectomy. The trachea is thus permanently removed from the skin and the digestive tract becomes independent.

lead_sponsor: Assistance Publique - Hôpitaux de Paris


title: Age Estimation of Adolescents for Legal Purposes: Determining the Medical Response

acronym: AGE-ADO

summary:
Economic globalisation has been associated with a rise of cross-border migration in Europe. In France, courts commonly demand appropriate medical tests aimed at age estimations of supposed minors without documentation. Determining the age of foreign adolescents may be important in order to define the rights and protection afforded them by law depending on this status, and the conditions in which they may be detained or held in police custody if they are under suspicion. Age estimation is considered to be ideally based on the combination of clinical, skeletal, and dental examinations. A number of forensic physicians do not integrate published recommendations or the results of published studies into their daily practice. Previous studies have shown that medical practice is heterogeneous in this area. Factors determining this heterogeneity are unknown.

We hypothesize that the personal and professional development of the physician, as well as the characteristics and the circumstances of medical examination can affect medical response on age determination, beyond clinical and radiological data. The purpose of this study is to identify factors determining medical response to courts.

ReferenceCitation: Pruvost MO, Boraud C, Chariot P. Skeletal age determination in adolescents involved in judicial procedures: from evidence-based principles to medical practice. J Med Ethics. 2010 Feb;36(2):71-4. doi: 10.1136/jme.2009.031948.

ReferenceCitation: Buken B, Safak AA, Yazici B, Buken E, Mayda AS. Is the assessment of bone age by the Greulich-Pyle method reliable at forensic age estimation for Turkish children? Forensic Sci Int. 2007 Dec 20;173(2-3):146-53. doi: 10.1016/j.forsciint.2007.02.023. Epub 2007 Mar 27.

ReferenceCitation: Schmeling A, Olze A, Reisinger W, Geserick G. Age estimation of living people undergoing criminal proceedings. Lancet. 2001 Jul 14;358(9276):89-90. doi: 10.1016/S0140-6736(01)05379-X. No abstract available.

ReferenceCitation: Garamendi PM, Landa MI, Ballesteros J, Solano MA. Reliability of the methods applied to assess age minority in living subjects around 18 years old. A survey on a Moroccan origin population. Forensic Sci Int. 2005 Nov 10;154(1):3-12. doi: 10.1016/j.forsciint.2004.08.018. Epub 2004 Nov 10.

ReferenceCitation: Hassanali J. The third permanent molar eruption in Kenyan Africans and Asians. Ann Hum Biol. 1985 Nov-Dec;12(6):517-23. doi: 10.1080/03014468500008091.

location_facility: : Jean Verdier hospital, Department of forensic medicine


title: Impact Evaluation of a Multidisciplinary End-of-treatment Day Hospital on the Quality of Life of Adolescents and Young Adults With Cancer

acronym: HOPAYA

summary:
Rational: 800 cases of cancer per year are diagnosed in France among Adolescents and Young Adults (AYA). This population has been specifically targeted since 2008 by the INCa, leading to the development of structures entirely dedicated to its management. Indeed, the occurrence of cancer in this period of transition leads to specific problems, which require a special attention. The various measures taken since then (Cancer Plan 2014-2019, DGOS instruction in 2016, 10-year strategy to fight cancer 2021-2030) have enabled the implementation of multidisciplinary structures, resulting in better access to care, and consideration of the social, family and relational dimensions of this population. However, the transition from the end of oncology treatment to the follow-up period remains a sensitive period, generating both positive (relief, joy) and negative feelings (uncertainty, feelings of abandonment, anxiety).

The investigators therefore hypothesize that the creation of a multidisciplinary end-of-treatment day hospital (DH) involving at least one medical interview, one psychological consultation and one social interview, would improve the quality of life of these former patients during the first year of oncology follow-up.

Method: This is a clinical research study conducted in a single centre. At their last visit for treatment, the study will be offered to patients. If the participants agree to participate, they will be randomized to benefit from DH in addition to their planned follow-up with their oncologist. The main objective is to compare the quality of life of former patients according to participation in DH or not. 210 patients will be included for a 20-month recruitment period.

Expected results: Throughout the development of DH, the investigators plan to improve the quality of life of former patients during this transitional phase.

primary_purpose: Supportive Care

lead_sponsor: Centre Leon Berard


title: Pilot Study to Improve Primary Prevention of Colorectal Cancer by Motivational and Community-based Approaches, According to Individual Risk Level, in People Undergoing Colonoscopy

summary:
It has been estimated that 19,000 Colorectal Cancers (CRC) could be prevented each year in France by changing individual risk behaviours (sedentary lifestyle, overweight, diet, alcohol). The cancer screening appears to be an opportune moment for health promotion and to inform about CRC risk factors.

The PRECÔTION study proposes an innovative scheme based on informative, motivational and community-based approaches. The objectives are to take advantage of patients' visits for colonoscopy to raise awareness and initiate individual prevention actions according to their own risk in order to modify individual risk behaviour.

Patients who come for a colonoscopy at the Centre Léon Bérard and who are negative (80% of cases) will benefit from an evaluation to identify their individual risk factors, determine their level of risk level for CRC and their motivation to change their behaviour.

Participants with a low level of risk will receive remote support in physical activity and nutrition via digital media based on a motivational approach. Participants will be proposed a monthly collective physical activity session, which will allow them to interact with each other and develop a community approach. Participants with an intermediate level of risk will be offered a collective health education session, in addition to the the support offered to participants with a low level of risk. Participants with a high level of risk will receive, in addition to the the support offered to participants with an intermediate level of risk, an individual motivational coaching and a connected watch. A logbook will also be given to all participants to accompany them on the different tools, especially for monitoring their goals.

The PRECÔTION study aims to evaluate these tools as innovative means of prevention before evaluate their effectiveness on a larger scale.


title: Evaluation of the COVID-19 Infection Response in Patients Admitted to the Emergency Department for Dyspnea

acronym: EPRICOD

summary:
This research aims to improve our knowledge of the epidemiology of patients consulting in the COvid-19 Possible REspiratory Units (RECOP unit). Indeed, the epidemic linked to COVID-19 affects France and impacts its health system. The reception of all intermediate patients will be on the Emergency Structures (SU). Indeed, the French healthcare system centralizes unscheduled urgent care on the ER. The aspecific respiratory symptomatology in "intermediate" patients indicates them all the more at an admission to SU or the diagnostic approach to respiratory difficulty may be carried out.

A central issue of this diagnostic strategy will be to be rapid, since the diagnosis will have to be made in the context of significant flows, with a need to quickly refer patients to the most suitable downstream service, while limiting the risk contamination of caregivers and vulnerable patients if a COVID-19 + patient is admitted to an unsuitable service. However, virological tests do not currently allow rapid results for COVID-19.

Research project of investigatory aims to develop a predictive model of the risk of being COVID-19 positive for patients admitted to the emergency room for acute dyspnea.

time_perspective: Other

lead_sponsor: University Hospital, Toulouse


title: Interest of Karate Kata Practice on the Self-esteem of Patients Living With HIV

acronym: KATACHRO

summary:
Since ancient times, physical and sports activity has been recognized as bringing many health benefits. Descriptive and interventional studies on physical activity are developing, demonstrating physical and psychological benefits for patients with various diseases.

Physical activity is effective in improving self-esteem in the general population. Good self-esteem allows for better autonomy to manage a chronic illness.

The benefit of physical activity in patients living with HIV has been the subject of several studies, notably outside France, and seems to improve the quality of life.

We hypothesize that sport, especially karate kata, could improve self-esteem in patients living with HIV, and represent another non-drug aid, in patients living with HIV.

lead_sponsor: Centre Hospitalier le Mans


title: Loss of Autonomy in the Elderly: Relationships Between Gut Microbiota Diversity, Nutrition and Cognitive and Sensory-motor Disturbances in "Small Eaters" and "Non Small Eaters"

acronym: ALMICO

summary:
Older adults currently make up 25% of the population in France, and future health expenditures will be largely related to the consequences of aging. Normal aging is associated with a progressive decrease in cognitive and sensorimotor function, malnutrition and/or under-nutrition and intestinal dysbiosis (i.e. changes in the composition and/or stability of the microbiota). Furthermore, admission to long-term care facilities such as Etablissement d'Hébergement pour Personnes Agées Dépendantes (EHPAD) leads to drastic changes in lifestyle in terms of (i) nutrition and (ii) motor functions. These modifications can influence the composition of the intestinal microbiota and its temporal evolution.

We will study the relationship between the deterioration of cognitive and sensorimotor function, diet and intestinal well-being (i.e. microbiota) of a cohort of older adults (>75 years) defined as "small eaters" from their admission in EHPAD at the University Hospital of Dijon. This study will be carried out by a unique French multidisciplinary consortium that includes academics and health professionals, who will be able to specify the criteria defining the "small eaters" and to envisage suitable dietary strategies aiming to preserve their nutritional status and intestinal well-being. The final goal is to improve quality of life at home by prolonging autonomy and independence, and thus to deferring admission to an EHPAD for as long as possible.

The objective of this study is to build a cohort of elderly "small eaters" and a cohort of elderly "non small eaters". These patients will be followed over a period of one year. The evolution of the composition of the intestinal microbiota will be determined in order to establish the existing relations between the composition of digestive microbiota, the nutritional state, and cognitive and sensorimotor function.

lead_sponsor: Centre Hospitalier Universitaire Dijon


acronym: POOMA

summary:
The incidence of bone and joint infections (BJI) in children (osteomyelitis, septic arthritis and spondylodiscitis) is 22 per 100,000 children in France. Every year, 3,000 children are hospitalized for BJI, 46% of whom are hospitalized for osteomyelitis. The clinical pictures of BJI are varied: some are severe from the outset; others are non-severe, such as BJIs in Kingella kingae, which are most common in children between the ages of 6 months and 5 years. Currently, the management of children's BJI, regardless of their severity, involves initial hospitalization to start intravenous antibiotic therapy. This non-inferiority trial evaluates, in children with acute osteomyelitis with no severity criteria, less invasive outpatient management with an oral antibiotic treatment given at the outset compared to standard management.

Main objective : Demonstrate the non-inferiority of an ambulatory management strategy versus a standard strategy involving hospitalization on complete recovery without relapse at 6 months after an episode of acute osteomyelitis in children aged 1-4 years without severity criteria.

Primary endpoint: Complete cure without relapse at 6 months defined by the absence of clinical signs of osteomyelitis at 6 months AND the absence of secondary septic complications (septic arthritis, periosteal abscess) before the end of antibiotic therapy AND the absence of relapse or rehospitalization for osteomyelitis related to the initial infection. This criterion will be assessed blindly by an adjudication committee.

Randomized controlled trial of non inferiority, with active control, in open multi-center.

The control or experimental arm allocation (1:1 ratio) will be open-label of the physician, patient and parents. This is a PROBE study: The evaluation of the main judgment criterion will be carried out blindly by an adjudication committee.

title: Acute Non-severe Osteomyelitis in Children - Outpatient Management Strategy With Oral Antibiotic Therapy Compared to a Standard Strategy With Conventional Hospitalization and Intravenous Antibiotic Therapy: a Randomized Open-label Non-inferiority Study With Bayesian and Medical-economic Analyses.

source: euctr

region: Île-de-France

nom_etablissement: ASSISTANCE PUBLIQUE-HOPITAUX DE PARIS

acronyme: POOMA

titre: Non-infériorité d'un traitement antibiotique ambulatoire oral versus traitement antibiotique standard (intraveineux puis oral) de l'ostéomyélite aiguë non sévère de l'enfant: un essai randomisé en ouvert avec analyses bayésienne et médico-économique

nom_porteur: LORROT

prenom_porteur: Mathie


title: Genetical, Multi-center, Prospective Study of Phenotyping and Genotyping of Patients Suffering From Congenital Amaurosis of Leber or From an Early Severe Retinal Dystrophy in the Aim of the Realisation of a Clinical Trial of Gene Therapy

summary: Retinal dystrophies are responsible for numerous cases of blindness, and there are no therapeutic possibilities today. Gene therapy is efficient in a dog model concerning dystrophy linked to a mutation of the rpe65 gene. If such a therapy is to be considered for humans, it is urgent to select, at a national level, patients suffering from dystrophy linked to a mutation of the rpe65 gene. The systematic correlation of phenotype/genotype is an anatomical-functional approach, but it also identifies patients who may be potentially included in a future gene therapy study. Indeed, identification of people with a mutation of rpe65 is still insufficient in France (compared to other European countries) because of a lack of systemic genotyping of retinal dystrophy.

design_allocation: Non-Randomized

lead_sponsor: Nantes University Hospital


title: Interest of the Alkalinization of the Adrenaline Lidocaine Solution for Conversion of Epidural Analgesia Into Epidural Anesthesia During an Emergency Caesarean

acronym: BiEpIC

summary:
During labor, pain is systematic. In France, epidural analgesia is the gold standard to fight pain.

Sometimes, emergency situations involve the maternal or fetal prognosis and require an emergency fetal extraction by caesarean. When an effective epidural analgesia is in place, an injection of adrenaline lidocaine converts this epidural analgesia into an epidural anesthesia allowing a surgical procedure. Sometimes, the time required to set up the anesthesia cannot be expected and a general anesthesia is performed.

Local anesthetics used during epidural analgesia have Pka between 7.8 and 8.1. In solution, local anesthetics exist in two forms: an un-ionized form and an ionized form. The non-ionized form is liposoluble and crosses the lipid membranes to reach the site of intracellular action. The non-ionized form conditions the time taken to install anesthesia.

When the pH of the solution is equal to Pka, un-ionized and ionized form are present in equal quantity. Commercial local anesthetic solutions have acidic pH and so contained a majority of ionized form. Alkalinization of local anesthetics solution should bring the pH closer to pKa and therefore to favor a greater proportion of non-ionized form.

lead_sponsor: CHU de Reims


title: Impact of the French and International Definitions and the Type of Muscle Assessment Tool on the Prevalence of Undernutrition in Hospitalized Patients

acronym: PROTEIN

summary:
In France, the prevalence of undernutrition among hospitalized patients varies from 30 to 50%. Undernutrition is strongly associated with a decrease in the patient's functional capacities and an increase in morbidity and mortality and in healthcare costs.

In 2019, the Global Leadership Initiative on Malnutrition (GLIM) and the Haute Autorité de Santé (HAS) are each publishing updated diagnostic criteria for undernutrition in adults aged <70 years. Aetiological and phenotypic criteria are retained: reduced food intake, inflammatory state, weight loss, BMI and reduced muscle mass. Reduced muscle mass has become a major diagnostic criterion and various measurement tools are suggested, such as bioelectrical impedancemetry, grip strength measurement or magnetic resonance imaging. Collaboration between dieticians, physiotherapists, nurses, care assistants and doctors makes it possible to respond to the need to screen for undernutrition according to these new definitions, which involve a multidisciplinary assessment.

These two definitions are very similar but differ on the time period of weight loss, on the BMI cut-off values and on the thresholds for muscle mass loss. The GLIM definition may be less selective than the HAS definition.

The investigators hypothesise that the prevalence of undernutrition in a population of adult patients hospitalised in diabetology-obesity, pneumology, oncology and gastro-nutrition, aged < 70 years, is different according to the diagnostic criteria recommended by the HAS or by the GLIM, and may be associated with a different patient morbi-mortality. In addition, the choice of the method of assessment of muscle function could impact this prevalence.

The main objective of this study is to compare the prevalence of global undernutrition based on the diagnostic criteria recommended by the HAS with that based on the diagnostic criteria recommended by the GLIM, in patients hospitalised in diabetes-obesity, pneumology, oncology and gastro-nutrition units.

The secondary objectives will be, in patients hospitalized in diabetes-obesity, pneumology, oncology and gastro-nutrition units:

To compare the prevalence of severe undernutrition between the HAS and GLIM groups;

To compare the morbi-mortality of undernutrition between the HAS and GLIM groups on :

Length of hospital stay ;
Mortality rate;
Autonomy at discharge.
To evaluate the impact of the choice of the muscle function assessment tool on the prevalence of undernutrition in the HAS and GLIM groups.

This is a prospective, monocenter, observationnal and cohort study.

Patients hospitalised in acute and rehabilitation care for diabetes-obesity, pneumology, oncology and gastro-nutrition purpose.

Univariate comparisons will use the usual statistical tests after verification of the distribution of the variables (Chi2 or Fisher's test, t-test, anova or their non-parametric equivalents Wilcoxon and Kruskal-Wallis tests). The variables will be compared between the two groups by the appropriate tests according to the type of variables (quantitative or qualitative) and their distribution.

The results of this study will make it possible to verify whether the GLIM definition increases the prevalence of undernutrition compared to that of the HAS. The use of one or other of the definitions could thus have an impact on the medical and paramedical management of undernutrition. On the other hand, muscle function benefits from different assessment tools, which could lead to a different estimate of the reduction in muscle mass and therefore a different prevalence of undernutrition. The results of our study will help to evaluate this and guide professionals in the choice of tools for assessing muscle function.

ReferenceCitation: Cederholm T, Jensen GL, Correia MITD, Gonzalez MC, Fukushima R, Higashiguchi T, Baptista G, Barazzoni R, Blaauw R, Coats A, Crivelli A, Evans DC, Gramlich L, Fuchs-Tarlovsky V, Keller H, Llido L, Malone A, Mogensen KM, Morley JE, Muscaritoli M, Nyulasi I, Pirlich M, Pisprasert V, de van der Schueren MAE, Siltharm S, Singer P, Tappenden K, Velasco N, Waitzberg D, Yamwong P, Yu J, Van Gossum A, Compher C; GLIM Core Leadership Committee; GLIM Working Group. GLIM criteria for the diagnosis of malnutrition - A consensus report from the global clinical nutrition community. Clin Nutr. 2019 Feb;38(1):1-9. doi: 10.1016/j.clnu.2018.08.002. Epub 2018 Sep 3.

ReferenceCitation: Correia MI, Waitzberg DL. The impact of malnutrition on morbidity, mortality, length of hospital stay and costs evaluated through a multivariate model analysis. Clin Nutr. 2003 Jun;22(3):235-9. doi: 10.1016/s0261-5614(02)00215-7.

ReferenceCitation: Cruz-Jentoft AJ, Baeyens JP, Bauer JM, Boirie Y, Cederholm T, Landi F, Martin FC, Michel JP, Rolland Y, Schneider SM, Topinkova E, Vandewoude M, Zamboni M; European Working Group on Sarcopenia in Older People. Sarcopenia: European consensus on definition and diagnosis: Report of the European Working Group on Sarcopenia in Older People. Age Ageing. 2010 Jul;39(4):412-23. doi: 10.1093/ageing/afq034. Epub 2010 Apr 13.

ipd_sharing_description: The protocole, the statistical analysis plan and data will be available as supplementary materials with the publication. The other data that support the findings of this study will be available from the corresponding author upon reasonable request.

lead_sponsor: Hopital Forcilles


title: Tricho-hepato-enteric Syndrome: From Abnormal RNA Decay to Disease Clinical and Biological Characterization of Patients and Collection of Samples -THE-RNA

acronym: THE-RNA

summary:
Objectives: Collect clinical and biological data about patients with SD/THE, collect samples of patients; create a secure on line database to collect worldwide data about SD/THE Partners : APHM, HCL, APHP

Currently10 patients (8 with TTC37 mutations and 2 with SKIV2l mutations) present a SD/THE and are managed in France in 5 different centers (Marseille, Paris Trousseau, Paris Necker, Paris Robert Debrés, and Lyon). Most of them are followed in hepato-gastro-enterology units for their intractable diarrhea. Three aspects of the disease: intractable diarrhea, immune defect and liver disease are responsible for the main part of the burden of the disease .For each aspect, the investigators will propose a close follow-up with collection of clinical, biochemical, functional and microbial data.

Collect of clinical date: during a programmed consultation clinical data about symptom will be collected twice a year. A detailed form will be used for better delineation of the symptoms. These data included growth, symptom (diarrhea, pain …), and clinical signs. Most of these children have recurrent sample for follow up. During them some blood will be take for study the immune side but also the platelet function.

sponsor_collaborators: Assistance Publique Hopitaux De Marseille

location_facility: Assistance Publique Hopitaux de Marseille


title: Pharmacokinetics Study of Intraperitoneal Administration of Daptomycin in Patients With Peritoneal Dialysis and Peritoneal Infection

acronym: DAPTODP

summary:
Peritoneal infection is still a frequent complication in peritoneal dialysis patients . In France, It contributes to the technique failure, responsible for about 20% of cases of transfer in hemodialysis. The risk of direct mortality is estimated at 1 to 6% according to studies. Peritoneal infection is involved in the dysfunction of the peritoneal membrane.

Based on the recommendations of the International Society for Peritoneal Dialysis, the intraperitoneal route is preferentially recommended. For many antibiotics, pharmacokinetics (intravenous and intraperitoneal) was studied and protocols for IP administration were validated.

Daptomycin, is a cyclic lipopeptide natural, active only on Gram-positive bacteria. It is presented as an alternative to vancomycin in infections resistant pathogens. The stability of daptomycin in peritoneal dialysis fluids (PDF) has been tested, and antimicrobial activity as well.

Seven patients were treated with daptomycin intraperitoneally successfully. But no study has reported pharmacokinetics of daptomycin via the IP route. We propose a pharmacokinetic study of daptomycin administered intraperitoneally in 12 patients on CAPD and with Gram-positive peritoneal infection.

lead_sponsor: University Hospital, Caen


title: Evaluation of the Efficacy of Negative Pressure Wound Therapy on Incisional Wound Healing After a Total Ankle Arthroplasty: a Randomized Study.

acronym: PICO-PTC

summary:
About 500 total ankle prostheses are implanted each year in France. Wound healing issues are the most common complications after Total Ankle Arthroplasty (TAA). In 2010, a French retrospective study observed a wound infection rate of 8% among 592 prostheses implanted. Wound infection rate can vary up to 28% from the data available in the literature. Delayed wound healing is a known risk factor of surgical site infection (SSI). Even if it remains a rare event, its consequences for the patient can be severe and lead to removal and/or replacement of the prosthesis, arthrodesis, or transtibial amputation.

Negative Pressure Wound Therapy (NPWT) is an adjuvant therapy for wound management and healing: it delivers a controlled negative pressure (sub-atmospheric) applied topically onto the wound from a vacuum pump. The wound is filled with wound filler and sealed with an airtight adhesive drape. It allows creating a moist wound environment, removing exudate, and reducing edema and risk of infection.

Incisional NPWT can accelerate wound healing regardless of the surgery performed, including orthopedics. A retrospective study compared the incidence of healing complications after TAA between the administration of the NPWT PICO™ (Smith&Nephew) and the standard of care of postoperative wounds in 74 patients. A significant reduction of wound healing issues (3% vs. 24%, respectively; p=0.004) and a non-significant reduction of SSI (3% vs. 8%, respectively) were observed.

To the investigator knowledge, no randomized study has been conducted to assess the efficacy of the NPWT PICO™ for postoperative wound healing after TAA: Investigators propose to set up this study to obtain a high level of evidence in this population and investigators assume that the use of the PICO™ system after TAA would reduce delayed wound healing compared to the standard of care with conventional dressings.

location_facility: Service de Chirurgie orthopédique - Groupement Hospitalier Lyon Sud

intervention_type: Device


title: Functional and Mechanistic Characterization of Limb Ulcers in Patients With Sickle Cell Disease

summary:
Sickle Cell Disease is the most frequent genetic disease in the world (representing one birth over 1900, in France). The polymerization of the abnormal hemoglobin (i.e., HbS) when deoxygenated is at the origin of a mechanical distortion of red blood cells (RBC) into a crescent-like shape. Sickled RBCs are very fragile and rigid, which lead patients to have severe anemia and to develop frequent and repeated painful vaso-occlusive crises. Furthermore, the repetition of sickling-unsickling cycles causes irreversible damages to the RBCs, which shorten their half-life. Accumulation of free hemoglobin and heme in the plasma is involved in blood vessels lesions in both the macro- and micro- circulation.

The resulting vascular dysfunction could explain why limb ulcers are 10 fold more frequent in patients with sickle cell disease compared to the general population and may happen at a younger age. Limb ulcers induce significant morbidity (delay of healing between 9 and 26 weeks in the french cohort), and are associated to significant pain (needing opioid pain-killer) and increase the risk of infection. Cost of care is also increased. Moreover, ulcers induce missed school and work days.

Data on cutaneous microcirculation and ulcers physiopathology in patients with sickle cell disease are scarce. We want to realise a microcirculatory and neurological functional study of patients with with and without ulcers and a characterization of biomarkers present in the blood or in the wound fluid which can participate to ulcers physiopathology.

To ensure healing, adapted therapeutics are essential. Several strategies are proposed such as: lifestyle measures (venous compression, lower limb elevation, rest), dressings, hyperbaric oxygenotherapy (also used in diabetic ulcers). The project is devoted to study the mechanisms involved in leg ulcers and the effects of therapeutical/behavioral strategies.

intervention_type: Diagnostic Test


title: Establishment of a Database of Patients Suffering From Obesity With a Medical Treatment in the Framework of The Specialized Obesity Center Languedoc-Roussillon CHU Montpellier

acronym: Elodie Cohort

summary:
Obesity is a public health problem, given its prevalence (15% in France in 2012, Obépi survey) but also by the complications that it generates. We were firstly, intersted in bone mineral density during obesity.

We were able to confirm in an observational study that BMD (Z-score)was augmented. We also showed, for the first time, that this increase in Z-score was dependent on sex, severity of obesity and age but also bone site. If the increase in BMD at the level of the bearing zones seems logically to be explained by the hypothesis that the mechanical stresses have an osteogenic effect on the bone tissue, the latter can not in any way respond to the concomitant increase in BMD at non-bearing areas such as the radius.These results suggest that changes in certain systemic compounds induced by obesity may interfere with bone metabolism. recent data also suggest a change in the concentration of pro-glucagon peptides (GLP-1, glicentin, glucagon, etc.) in obese subjects and in bariatric surgery). Although body composition and its variation after surgery could be largely involved in the modification of these peptides, no data on these potential relationships is currently available. However, no data are available for baseline body composition and weight loss data. Finally, 80% of obesity is associated with insulin resistance, a key mechanism in the pathophysiology of type 2 diabetes. A metabolically healthy obesity phenotype is described, although the definition criteria are not consensual.The evolution of this phenotype with aging and with the duration of evolution of obesity is not well described.

Our goal is to create a database of weight-bearing patients in the care setting and to determine the "hormonal dialogue" between peripheral (muscular and adipose) and bone tissues, which may be associated with changes in body composition during obesity and aging, and that according to sex.

lead_sponsor: University Hospital, Montpellier


title: Organochlorine Concentration in Adipose Tissue and Pancreatic Adenocarcinoma : a Case Control Study.

acronym: PESTIPAC

summary:
Pancreatic adenocarcinoma represents more than 90% of pancreatic neoplasms. Around 14000 new cases of pancreatic adenocarcinoma are being diagnosed each year in France and about 8.6% in Grand Est region. National incidence has doubled for men and tripled for women between 1982 and 2012. Pancreatic adenocarcinoma is the deadliest digestive cancer, with only 7 to 8% all stages 5-year survival. It will become the second deadliest in Europe, behind bronchopulmonary cancer.

Several risk factors have been identified such as diabetes, tobacco, chronic pancreatitis or obesity. However, a mismatch exists between incidence forecasts and actual risk factors knowledge. Several hypothesis plead for an environnemental cause.

Plant protection products oncogenetic effects are known and have been proved to be responsible for tumors, including haematological malignancies. Its role in pancreatic carcinogenesis is still poorly studied and show heterogeneous results. They do not allow to conclude for causality. Organochlorines is a specific subset of plant protection product that store in lipids during lifetime.

The aim is to study association between organochlorine concentration in adipose tissue with pancreatic adenocarcinoma.


title: Sleep Related Memory Consolidation in Children With Age Related Focal Epilepsy.

acronym: EPIMEM

summary:
Age related focal epilepsies in children encompasses, according to the ILAE criteria, benign epilepsy with centro temporal spikes (BECTS), atypical benign partial epilepsy (ABPE) and epileptic encephalopathy with continuous spike and waves during sleep (ECSWS). These non structural epilepsies are associated with interictal sleep spike and waves activated by sleep. Moreover, high prevalence of learning disorders occur in children with age related epilepsies. A correlation is suspected between learning disorders and sleep activation of spike and waves. The investigators suppose that learning dysfunction is linked to loss of information during sleep of epileptic children, unlike for control patients. As sleep allows memory consolidation of words learned during wakefulness, an epileptic activity during sleep may disrupt this consolidation, leading to a loss of information.

Hypothesis: the investigators hypothesize a disruption of memory consolidation after one night in children affected with ABPE and ECSWS (severe group) compared to memory consolidation in children affected with BECTS (benign group), and control group.

Primary purpose:

To demonstrate that the deficit of delayed recall in 15 word learning test after one night is higher for the "severe group", compared to the "benign group" and the control group.

Secondary purposes:

to study the evolution over time of memory consolidation
to evaluate the correlation of the deficit of delayed recall with executive dysfunction, clinical factors of epilepsy, neurophysiological factors of epilepsy, and sleep architecture

lead_sponsor: University Hospital, Strasbourg, France

location_facility: Hopital D'Enfants Pediatrie Medicale Ambulatoire

location_facility: Hopital de Hautepierre-Service de Pediatrie/Neurologie

region: Grand Est

nom_etablissement: HOPITAUX UNIVERSITAIRES DE STRASBOURG

type_etablissement: CH

acronyme: EPINEM

titre: Etude de la consolidation mnésique chez les enfants atteints d'epilepsie focale idiopathique

nom_porteur: DE SAINT MARTIN

prenom_porteur: Anne


title: Emotional Dysregulation and Cyclothymia in Adult Patients With ADHD: Cohort Follow-up of Patients in Two Referral Centers

acronym: EMO-TDA

summary:
Attention deficit disorder in adults with or without hyperactivity (ADHD) is a common disorder, affecting around 3% of the population. ADHD increases the risk of psychiatric disorders (mood disorders, sleep disorders, personality disorders, addictive behavior), risky behaviors, and vocational difficulties. Emotional dysregulation (ED) constitute a major hindrance in the daily life of subjects, with a great impact on the general functioning and the quality of life of the patients.

The investigators want to determine the characteristics of patients with each type of ED (impulsivity, exacerbated emotional intensity, cyclothymia, borderline personality traits), and study the stability of these traits over time. Since circadian rhythms influence mood and circadian rhythms frequently occur in patients with ADHD, the investigators want to determine if there is a link between ED and instability in circadian rhythms. Finally, they would like to observe whether the ED evolves and according to whether or not treatment is taken

status: Active, not recruiting

location_facility: Centre Hospitalier Spécialisé de Rouffach - Secteur 8

location_facility: Hôpitaux Universitaires de Strasbourg - Service de Psyhciatrie 2/PPSAM


title: Study of Microcirculation in Cardiogenic Shock

acronym: MicroShock

summary:
Cardiogenic shock is usually defined as primary cardiac dysfunction with low cardiac output leading to critical organ hypo perfusion and tissue hypoxia. Despite progress in the management of cardiogenic shock, mortality remains unacceptably high.

This significant mortality, close to 40 %, is partly due to profound alterations of microcirculatory blood flow in cardiogenic shock, leading to multi organ failure, despite restoration of macro-hemodynamic parameters such as blood pressure and cardiac output. The microcirculation is the terminal vascular network of the systemic circulation consisting of microvessels with diameters < 20 μm including arterioles, capillaries, and venules. This part of the circulation is critical as it is responsible for nutrient delivering and oxygen transfer from the erythrocytes in the capillaries to the parenchymal cells to meet their metabolic demands, but it is also the area where water, other gases, hormones and waste products are exchanged.

Hence, the evaluation of clinical signs of peripheral hypoperfusion reflecting microvascular perfusion is of interest. We aimed to study these parameters such as skin capillary refill time (CRT), mottling and central-to-toe temperature difference (ΔTc-p) in a cardiogenic shock population. Assessing the prognosis of these microcirculation parameters and their interaction with macrocirculation parameters such as arterial pressure, cardiac index, left ventricular ejection fraction is also the aim of this study. Lastly, looking at the prognostic value of these markers seems interesting.

location_facility: Les Hôpitaux Universitaires de Strasbourg


title: The Treatment of Hypertension Associated With Severe Preeclampsia. A Randomize Controlled Trial of Urapidil Versus Nicardipine. The Uranic Trial

acronym: Uranic

summary:
The objective of this study is to demonstrate that urapidil is not inferior to nicardipine for the treatment of hypertension associated with preeclampsia (PE) and that it is better tolerated.

efficacy endpoint : mean arterial blood pressure corrected to 100-120 mmHg after 120 min of study drug administration.
safety endpoints : clinical and biological observation for any side effect. All infants will be observed in the neonatology unit (during 48h).

Pharmacokinetic study included to study :

transplacental transfer,
transfer in breast milk,
and neonatal elimination (premature babies of mothers treated with urapidil (less than 33 WG))

status: Terminated

location_facility: Service d'Anesthésie et de Réanimation Médicale, Hôpital de Hautepierre, Hôpitaux Universitaires de Strasbourg


title: Identification of Mutations Responsible for Rare Familial Skin Diseases by Next Generation Sequencing

acronym: DERMA-SEQ

summary:
The primary purpose of the protocol is to use next generation sequencing to identify pathogenic variants in genes involved in very rare skin diseases.

The secondary purpose will be to study the genotype-phenotype correlation in order to re-evaluate the classification of these disorders. This work could help in the understanding of the physiopathology of very rare skin disorders.

location_facility: Service de Dermatologie, Hôpital Civil, Hôpitaux Universitaires de Strasbourg

location_facility: Fédération de Génétique, Service de Génétique Médicale, Hôpital de Hautepierre, Hôpitaux Universitaires de Strasbourg


title: ENDmetriosis and Reserve Ovarienne

acronym: ENDRO

summary:
Endometriosis is the ectopic implantation of endometrial glands and stroma, and can be ovarian and peritoneal (superficial or deep). There are 4 stages in endometriosis according to severity, and the stage is established on the basis of intra-operative observations. The AFSr classification is currently most used (I-IV,minimal, mild, moderate, severe). Most associated with endometriosis are subfertility and pelvic pain.

In the surgical management of deep endometriosis, the issue of fertility is pivotal. There is a higher rate of infertility in a population of women with endometriosis as compared to the general population, even though the mechanisms are not yet elucidated. Patients with deep endometriosis can be referred to the surgeon for subfertility, but even when they are referred for chronic pain, future fertility considerations are taken into account in the planning of the surgery, as the patients are often young.

It is now well documented that ovarian cystectomy is deleterious with regards to the ovarian reserve, and more so in endometriomas than in any other type of benign cysts. The ovarian reserve is the functional potential of the ovaries, reflecting the quantity and quality of remaining follicles. Studies have also relied greatly on the measure of serum anti-mullerian hormone (AMH) to evaluate the effect of cystectomy on ovarian reserve, as AMH is currently the most reliable marker to assess ovarian reserve. A significant difference was found between AMH before and following cystectomy in several studies. The deleterious effect of deep endometriosis surgery which comprises a wide dissection and adhesiolysis of the pelvis in many cases, even when no cystectomy has been performed, is therefore not entirely ruled out. To the best of our knowledge, there are no studies on the effect of deep endometriosis surgery, apart from ovarian surgery, on ovarian reserve.

Our center is very active in the laparoscopic surgical treatment of deep endometriosis, with more than 200 cases every year. The objective of this trial is to assess the effect of deep endometriosis surgery on the ovarian reserve, whether a cystectomy is performed or not, by measuring serum AMH before and after surgery, at 6 months and 1 year post-operatively.

location_facility: Hôpital de Hautepierre - Hôpitaux Universitaires de Strasbourg

location_facility: CMCO - Hôpitaux Universitaires de Strasbourg


title: Analgesic Non Inferiority of the Thoracic Bi-block Combining an Erector Spinae Muscle Plane Block and a Serratus Anterior Muscle Plane Block in Comparison With Thoracic Epidural for Video-Assisted Thoracic Surgery.

acronym: ANESSTEVATS

summary:
Video-Assisted thoracic surgery (VATS) is the standard treatment for localized lung cancer. However, there is no consensus on analgesic management in patients undergoing VATS.

The aim of the study is to compare the analgesic efficacy of thoracic epidural with that a "Bi-block" combining an Erector Spinae muscle plane Block (ESP) and a Serratus Anterior Block (SAP) in patients undergoing VATS for lung or pleural surgery.

Our main hypothesis is that the analgesic efficacy of the Bi-block, assessed by morphine consumption, is not inferior to that provided by a thoracic epidural during the first 48 hours after VATS. We conducted a age, gender and type of surgery-matched retrospective cohort study in the Department of Thoracic Anesthesia of the Montpellier University Hospital (France).

location_facility: Intensive Care Unit, D - University hospital of Montpellier


title: Multicentrique Prospective Evaluation of Radiofrequency Surgical Treatment of Homorrhoidal Disease

acronym: RF-GREP

summary:
Hemorrhoids consist of a tissue rich in blood vessels and are present in all individuals inside the anus. Hemorrhoidal disease (HD) is when hemorrhoids become troublesome and cause symptoms such as pain, bleeding, prolapse or seepage.

The first steps in the treatment of HD involve either drugs or instrumental gestures (sclerosis, ligation). In the event of failure or of a disease that is significant from the outset, it is possible to envisage a surgical treatment.

The use of a radiofrequency is a new technique, already used frequently by vascular surgeons in the treatment of varicose veins of the lower limbs. This technique has been developed for radiofrequency destruction of hemorrhoidal vascular tissue.

In France, no studies have been carried out to evaluate this new technique.

lead_sponsor: Ramsay Générale de Santé

sponsor_collaborators:
Ramsay Générale de Santé
Dr Abramowitz


title: Evaluation of Local Capillary Blood Lactate in Chronic Critical Ischemia of Lower Limb Before and After Revascularization.

acronym: ELIT

summary: Evaluation of a new biological criterion, the local capillary blood lactates to optimize the management of patients with chronic critical ischemia requiring revascularization fast. Potential use in terms of capillary blood lactate as a diagnostic indicator of recurrent ischemia in a limb revascularized.

location_facility: Hôpitaux Universitaires de Strasbourg, Service d' Anesthésie Réanimation Chirurgicale


title: Evaluation of Non Alcoholic Metabolic Liver Diseases in Patients Harboring Central Adiposity and Insulin Resistance by Biochemical and Functional Genomic Approaches

summary: Non alcoholic fatty liver diseases (NAFLD) are represented by two main pathological conditions, hepatic steatosis (HS) and non alcoholic steatohepatitis (NASH), which are characterized by the accumulation of fat in the liver. The diagnosis of these two entities is achieved by histology and neither imaging nor biochemical markers are accurate enough to discriminate them. At the contrary of HS, NASH features hepatocyte necrosis, inflammation and fibrosis of variable intensity that could progress and ultimately evolve to cirrhosis. Therefore, it is important to distinguish between HS and NASH in order to treat the patients accordingly. In this study, the investigators aim to understand the molecular mechanisms that govern the transition from benign steatosis to complicated NASH. The investigators will analyze by "Q-RT-PCR" and "DNA microarray" technologies in the liver of obese patients, the expression of genes that are susceptible to be involved in the pathogenesis of NAFLD and identify the potential signaling pathways responsible for the progression of the disease.

location_facility: Service de Chirurgie Digestive et Endocrinienne - Nouvel Hôpital Civil

location_facility: Service d'Hépato-Gastro-Entérologie - Nouvel Hôpital Civil


title: Comparison of Two Types of Exercise Training for Asthmatic Subjects : Interests and Limits

summary: The aim of the study is to compare two methods of exercise training: alternate load versus constant load realized at moderate or high intensities, in order to define the interests and the limits of each one of these two methods. The finality is to make possible to the expert to choose what exercise training induces the best results quickly and to make its choice according to the therapeutic objectives which it lays down.We also evaluate the effects of these exercises training on the vagal response and the bronchial tone of the asthmatics. From a clinical point of view, a close attention will be paid to the effects of these exercises training on the control of asthma and the quality of life of the subjects

location_facility: Service de Physiologie et d'Explorations Fonctionnelles - Pôle de Pathologie Thoracique - Nouvel Hôpital Civil

location_facility: Service de Pneumologie - Pôle de Pathologie Thoracique - Nouvel Hôpital Civil


title: Endothelial Dysfunction Reversibility in Patients With Peripheral Arterial Disease

summary: To determine whether fruit juice might reverse endothelial dysfunction and whether this reversibility has a prognosis value

location_facility: Service de Physiologie et d'Explorations Fonctionnelles


title: Transcranial Magnetic Stimulation and Anti-epileptic Effect: Optimization and Evaluation With Electrophysiology.

summary: Epileptic disease is characterised by enhanced brain excitability. Low frequency repetitive transcranial magnetic stimulation (rTMS) can be an effective treatment for refractory frontal epilepsy. Thought, physiological mechanisms of its effectivity are still unknown. It is yet possible to evaluate cortical excitability and inhibition with TMS-coupled electromyography before and after rTMS sessions ; this could provide clues for basic mechanisms of rTMS effects on the epileptic brain. We assume that rTMS decrease brain excitability by improving brain inhibition. Such an information could help for treating patients with both pharmacological and non-pharmacological methods.

location_facility: Département de Neurologie - Hôpital Civil


title: Assessment of Respiratory Functions After a Conditioning Regimen With Busulfan for Allograft or Autograft in a Unicenter Pediatric and Prospective Population

summary: Recently, several conditioning regimens did not include total body irradiation (TBI) anymore, especially in the case of young children due to cognitive sequelae and late effects on growth and height. Thus, such effective chemotherapy conditioning regimens were developed to avoid these complications. Busulfan is one of the major drugs used in these treatments, but, is also administered in high dose chemotherapy followed by autograft. In both situations, long term pulmonary side effects were diagnosed in a few cases. Even if the occurrence is not very frequent, the clinical management is a real challenge regarding the reduced quality of life and life expectancy of these patients. Up to now, no correlation was done between respiratory sequelae and busulfan pharmacokinetics. Hence, in the investigators' pediatric onco-hematological unit, a prospective study will begin and last three years to assess the respiratory side effects due to busulfan and their potential links with individual drug pharmacokinetic measures. These results will be compared to patients treated with TBI during the same period of time.

location_facility: Service de Pédiatrie Onco-Hématologie - Hôpital de Hautepierre

location_facility: Service d'Explorations Fonctionelles Respiratoires - Hôpital Civil


title: Early Assessment of Anti-Angiogenic Treatment Efficacy in Advanced Renal Cancer by Using Contrast-Enhanced Sonography

summary:
RATIONALE: Diagnostic procedures, such as contrast-enhanced ultrasound, may help measure a patient's response to treatment with drugs such as sunitinib malate, sorafenib tosylate, or bevacizumab, and allow doctors to plan better treatment.

PURPOSE: This clinical trial is studying ultrasonography in assessing antiangiogenic treatment in patients with advanced kidney cancer.

lead_sponsor: National Cancer Institute, France

location_facility: Centre Hospitalier Universitaire Bretonneau de Tours


title: Frontline Chemotherapy "Reinforced" for Cancers of the Colon and Rectum With Potentially Resectable Hepatic and/or Pulmonary Metastases: Association of FOLFIRI and ERBITUX

acronym: ERBIFORT

summary:
RATIONALE: Monoclonal antibodies, such as cetuximab, can block tumor growth in different ways. Some block the ability of tumor cells to grow and spread. Others find tumor cells and help kill them or carry tumor-killing substances to them. Drugs used in chemotherapy, such as irinotecan, leucovorin, and fluorouracil, work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. Giving cetuximab together with combination chemotherapy may kill more tumor cells.

PURPOSE: This phase II trial is studying how well giving cetuximab together with combination chemotherapy works as first-line therapy in treating patients with colorectal cancer that has spread to the liver and/or lung.

sponsor_collaborators:
National Cancer Institute, France
University Hospital, Grenoble


title: Mesenchymal Stem Cell Administration in the Treatment of Coronary Graft Disease in Heart Transplant Patients

acronym: MESHT

summary:
MES-HT is a pilot multicenter prospective study conducted in transplant patients who developed severe coronary vasculopathy. A preparation of autologous mesenchymal cells of bone marrow is administered by endomyocardial injection, guided by the Noga® cardiac mapping system.

The main objective is to determine the effect of the administration of autologous mesenchymal cells of the bone marrow by intramyocardial injection on myocardial perfusion in cardiac transplant patients with severe coronary vasculopathy.

status: Suspended

location_facility: Département de cardiologie du Pr Michel KOMADJA


title: Evaluation of Focal Treatments of Localized Prostate Cancers With High Intensity Focused Ultrasound Using the Focal One® Device

summary:
Focal treatment of localized prostate cancer aims to destroy the cancerous foci without damaging surrounding tissues to avoid the side effects of radical treatments. This is why focal treatment is positioned as an alternative to radical surgery as well as an alternative to active surveillance.

This study is a feasibility study. It aims at treating the index tumor (the most aggressive one) which determines the vital prognosis of patient, while preserving the maximum surrounding prostatic tissue to preserve quality of life. The expected result is the total destruction of aggressive tumor areas by focal or multifocal treatment strategy using the Focal One® device (EDAP-TMS, Vaulx-en-Velin, France). The tumor destruction is validated by the negative biopsy rate between 6 and 12 months post-treatment.

location_facility: Hospices Civils de Lyon - Hôpital Edouard Herriot


title: Pilot Study of the Feasibility of Routine Inpatient Hepatitis C Screening

acronym: HOSPI-VHC

summary:
The Ministry of Health has set the target of eradicating hepatitis C (HCV) in France by 2025.

The goal is to validate the feasibility and value of conducting routine HCV screening in hospitalized patients.

lead_sponsor: Centre Hospitalier Intercommunal Creteil

collaborators: Association Nationale des Hépato-Gastroentérologues des Hôpitaux Généraux

location_facility: Grand Hôpital de l'Est Francilien - Site de Marne-la-Vallée


title: Study of the Functional Effects of Nusinersen in 5q-spinal Muscular Amyotrophy Adults (SMA Type 2 or 3 Forms): a Multicenter Single-case Experimental Design in Multiple Baselines Across Subjects, Randomized, Single-blinded Evaluation

summary: Spinal Muscular Atrophy (SMA) is an autosomal recessive disease caused by a mutation of exon 7, in 95% of cases, encoding the gene for the motor neuron survival protein called SMN1 (Survival Motor Neuron) located on chromosome 5q. Patients with an SMA-5q mutation suffer from progressive muscle deficiency and subsequent atrophy induced by degeneration of motor neurons in the spinal cord. Gene therapy is now available for the management of spinal muscular atrophy and nusinersen is the first approved treatment. Nusinersen has been granted marketing authorization in France since May 30, 2017. Nusinersen has a high level of medical service rendered (MSR) for types I, II, and III, but the improvement in medical service rendered (IMSR) is assessed as moderate for types I and II. For Type III, IMSR is not known.