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detecting neurodegenerative pathology in multiple sclerosis before irreversible brain tissue loss sets in

Translational Neurodegeneration 2019年第1期8卷 466-482页

作者： Jeroen Van Schependom Kaat Guldolf marie béatrice d’hooghe Guy Nagels Miguel d’haeseleer Neurology department Universitair Ziekenhuis BrusselCenter for NeurosciencesVrije Universiteit BrusselLaarbeeklaan 1011090 BrusselBelgium Radiology department Universitair Ziekenhuis brussel BrusselsBelgium Nationaal Multiple Sclerose Centrum MelsbroekBelgium

background:Multiple sclerosis(MS)is a complex chronic inflammatory and degenerative disorder of the central nervous *** brain volume loss,or also termed atrophy,is currently emerging as a popular imaging marker of neurodegeneration in affected patients,but,unfortunately,can only be reliably interpreted at the time when irreversible tissue damage likely has already *** of treatment decisions based on brain atrophy may therefore be viewed as *** body:This Narrative Review focuses on alternative techniques with the potential of detecting neurodegenerative events in the brain of subjects with MS prior to the atrophic ***,metabolic and molecular imaging provide the opportunity to identify early subcellular changes associated with energy dysfunction,which is an assumed core mechanism of axonal degeneration in ***,cerebral hypoperfusion has been observed throughout the entire clinical spectrum of the disorder but it remains an open question whether this serves as an alternative marker of reduced metabolic activity,or exists as an independent contributing process,mediated by endothelin-1 ***,both metabolic and perfusion alterations may lead to repercussions at the level of network performance and structural connectivity,respectively assessable by functional and diffusion tensor *** and finally,elevated body fluid levels of neurofilaments are gaining interest as a biochemical mirror of axonal damage in a wide range of neurological conditions,with early rises in patients with MS appearing to be predictive of future brain ***:Recent findings from the fields of advanced neuroradiology and neurochemistry provide the promising prospect of demonstrating degenerative brain pathology in patients with MS before atrophy has *** the overall level of evidence on the presented topic is still preliminary,this Review may pave the way for further longitudinal and multimodal studi

关键词： brain atrophy magnetic resonance imaging multiple sclerosis neurodegeneration neurofilaments

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Allogeneic stem cell transplantation-A curative treatment for paroxysmal nocturnal hemoglobinuria with PIGT mutation:A case report

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World Journal of Clinical Cases 2022年第17期10卷 5702-5707页

作者： Laurence Schenone Anne-béatrice Notarantonio Véronique Latger-Cannard Veronique Fremeaux-bacchi Marcelo de Carvalho-bittencourt marie-Thérèse Rubio Marc Muller Maud d'Aveni department of Hematology CHRU de NancyNancy F-54000France Genetic Laboratory CHRU de NancyNancy F-54000France IMoPA CNRS 7365University of LorraineNancy F-54000France Hematology Laboratory Cytometry PlatformCHRU de NancyNancy F-54000France Immunology Laboratory Hôpital Européen George PompidouParis 75015France Immunology Laboratory CHRU de NancyNancy F-54000France

bACKGROUNd Patients with paroxysmal nocturnal hemoglobinuria(PNH)have a clonal population of blood cells deficient in glycosylphosphatidylinositol-anchored(GPIanchored)proteins,most of the time resulting from a mutation in the X-linked gene *** report a patient with PNH resulting from a rare biallelic PIGT mutation on chromosome *** SUMMARY A 47-year-old man was referred to our hospital for febrile *** patient reported a history of recurrent urticaria and arthralgia and he presented during 3 mo recurrent acute dermo-hypodermitis and aseptic *** on clinical cases published with PIGT-PNH,with clinically typical PNH and autoinflammatory symptoms,we treated our patients with repeated infusions of eculizumab to decrease autoinflammatory symptoms and then we performed an allogeneic stem cell transplantation(allo-SCT)with a mismatched unrelated *** patient experienced no acute Graft vs Host disease(GvHd)and a moderate chronic GvHd and is now considered cured at 24 mo after *** This case report suggests that allo-SCT should be considered to cure PIGT-PNH patients.

关键词： Paroxysmal nocturnal hemoglobinuria Allogeneic stem cell transplantation PIGT mutation Recurrent meningitidis Autoinflammatory symptoms Case report

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bowel endometriosis: Colorectal surgeon's perspective in a multidisciplinary surgical team

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World Journal of Gastroenterology 2014年第42期20卷 15616-15623页

作者： Albert M Wolthuis Christel Meuleman Carla Tomassetti Thomas d'hooghe Anthony de buck van Overstraeten André d'Hoore department of Abdominal Surgery University Hospital Leuven Leuven University Fertility Centre department of Obstetrics and Gynecology University Hospital Leuven

Endometriosis is a gynecological condition that presents as endometrial-like tissue outside the uterus and induces a chronic inflammatory reaction. Up to 15% of women in their reproductive period are affected by this condition. deep endometriosis is defined as endometriosis located more than 5 mm beneath the peritoneal surface. This type of endometriosis is mostly found on the uterosacral ligaments, inside the rectovaginal septum or vagina, in the rectosigmoid area, ovarian fossa, pelvic peritoneum, ureters, and bladder, causing a distortion of the pelvic anatomy. The frequency of bowel endometriosis is unknown, but in cases of bowel infiltration, about 90% are localized on the sigmoid colon or the rectum. Colorectal involvement results in alterations of bowel habits such as constipation, diarrhea, tenesmus, dyschezia, and, rarely, rectal *** diagnosis must be made in case of irritable bowel syndrome,solitary rectal ulcer syndrome,and a rectal tumor.A precise diagnosis about the presence,location,and extent of endometriosis is necessary to plan surgical *** laparoscopic treatment has become the standard of *** on the size of the lesion and site of involvement,fullthickness disc excision or bowel resection needs to be performed by an experienced colorectal *** outcomes,following bowel resection for severe endometriosis,regarding pain and recurrence rate are good with a pregnancy rate of 50%.

关键词： Endometriosis Colorectal endometriosis deeply infiltrative endometriosis Laparoscopy diagno-sis Treatment

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Predictive approach of heterogeneous catalysis

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National Science Review 2018年第5期5卷 633-635页

作者： Jean-marie basset Jérémie d.A.Pelletier King Abdullah University of Science and Technology

‘Predictive catalysis’ or ‘catalysis by design’ has recently advanced heterogeneous catalysis by using the conceptual tool of surface fragments [1] such as ‘surface organometallic fragments’(SOMF)or ‘surface coordination fragments’(SCF) to achieve and understand a presumed catalytic cycle (see Fig. 1).One or several fragments of the molecule

关键词： SCF CR CO EXAFS

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Molecular analysis of the sex chromosomes of the platyfish Xiphophorus maculatus: Towards the identification of a new type of master sexual regulator in vertebrates

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Integrative Zoology 2009年第3期4卷 277-284页

作者： ASTRId bONE Christina SCHULTHEIS delphine GALIANA-ARNOUX Alexander FROSCHAUER Qingchun ZHOU Cornelia SCHMIdT Yvonne SELZ Catherine OZOUF-COSTAZ Agnès dETTAI béatrice SEGURENS Arnaud COULOUX Sylvie bERNARd-SAMAIN Valérie bARbE Stefan CHILMONCZYK Frédéric bRUNET Amandine dARRAS TOMASZKIEWICZ Marta marie SEMON Manfred SCHARTL Jean-Nicolas VOLFF Institute of Functional Genomics Ecole Normale Supérieure de Lyon/Universitéde LyonLyonFrance Physiological Chemistry 1 Biozentrum of the University of WürzburgWürzburgGermany department of Systematics and Evolution Muséum National d’Histoire NaturelleParisFrance CEA/dSV/IG/Genoscope EvryFrance Molecular Virology and Immunology Unit National Institute for Agricultural ResearchJouy-en-JosasFrance

In contrast to mammals and birds,fish display an amazing diversity of genetic sex determination systems,with frequent changes during evolution possibly associated with the emergence of new sex chromosomes and sex-determining *** better understand the molecular and evolutionary mechanisms driving this diversity,several fish models are studied in *** the medaka(Oryzias latipes Temminck and Schlegel,1846)for which the master sex-determination gene has been identified,one of the most advanced models for studying sex determination is the Southern platyfish(Xiphophorus maculatus,Günther 1966).Xiphophorus maculatus belongs to the Poeciliids,a family of live-bearing freshwater fish,including platyfish,swordtails and guppies that perfectly illustrates the diversity of genetic sex-determination mechanisms observed in *** ***,bacterial artificial chro-mosome contigs covering the sex-determination region of the X and Y sex chromosomes have been *** molecular analysis demonstrated that the sex-determination region is very unstable and frequently undergoes duplications,deletions,inversions and other *** gene candidates linked to the master sex-determining gene have been identified,some of them corresponding to *** putative genes are present on both the X and the Y chromosomes,suggesting a poor degree of differentiation and a young evolutionary age for platyfish sex *** compared with other fish and tetrapod genomes,syntenies were detected only with *** observation supports an independent origin of sex chromosomes,not only in different vertebrate lineages but also between different fish species.

关键词： sex chromosomes sex determination fish poeciliid Xiphophorus.

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Laparoscopic fenestration of pancreatic serous cystadenoma:Minimally invasive approach for symptomatic benign disease

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World Journal of Gastroenterology 2015年第22期21卷 7047-7051页

作者： Safi dokmak béatrice Aussilhou Fanjandrainy Rasoaherinomenjanahary Alain Sauvanet marie-Pierre Vullierme Vinciane Rebours Philippe Lévy department of HPb Surgery and Liver Transplantation Beaujon Hospital department of Radiology Beaujon Hospital department of Gastroenterology Beaujon Hospital

Serous cystadenoma(SC) is a benign pancreatic cystic tumor. Surgical resection is recommended for symptomatic forms, but laparoscopic fenestration of large symptomatic macrocystic SC was not yet described in the literature. In this study, 3 female patients underwent laparoscopic fenestration for macrocystic SC(12-14 cm). diagnosis was established via magnetic resonance imaging and endoscopic ultrasound, with intra-cystic dosage of tumors markers(ACE and CA19-9) in 2 patients. All patients were symptomatic and operated on 15-60 mo after *** evaluation showed constant cyst *** were informed about this new surgical modality that can avoid pancreatic resection. The mean operative time was 103 min(70-150 min) with one *** post-operative course was marked by a grade A pancreatic fistula in one patient and was uneventful in the other two. The hospital stay was 3, 10, and 18 d, respectively. The diagnosis of macrocystic SC was histologically-confirmed in all cases. At the last followup(13-26 mo), all patients were symptom-free, and radiological evaluation showed complete disappearance of the cyst. Laparoscopic fenestration, as opposed to resection, should be considered for large symptomatic macrocystic SC, thereby avoiding pancreatic resection morbidity and mortality.

关键词： Pancreatic serous cystadenoma Laparoscopicfenestration Symptomatic

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Exploratory Study of Intolerance of Uncertainty and Its Cognitive Processes by the Stroop Task among Parents of a Childhood Cancer Survivor

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Psycho-Oncologie 2023年第4期17卷 257-266页

作者： marie Vander Haegen Anne-marie Service de Psychologie de la santé/Unitéde Research Unit for a Life-Course Perspective on Health and Education-(RUCHE ULiège)University of LiègeLiège4000Belgium

*** study has examined the relationship between the factor of intolerance of uncertainty and its effects on the cognitive processes among parents of a childhood cancer *** of uncertainty is a central factor in generalized anxiety disorder;an anxiety disorder in which impaired processing of cognitive information in the form of cognitive biases is also *** aim of the study was to measure,via a classical Stroop test and an emotional Stroop test,the orientation of selective attention in relation to the uncertainty intolerance ***.61 parents of a childhood cancer survivor(aged 4 to 6 years)were recruited from hospitals in the Province of Liege(belgium).Parents completed questionnaires on intolerance of uncertainty and its associated factors(i.e.,usefulness of worry,cognitive avoidance,problem-solving attitude,and ruminations)and performed a Stroop test and an emotional Stroop *** correlations and multiple linear regressions were *** results indicated positive and significant correlations between the emotional Stroop(negative interference index)and intolerance to uncertainty but also with repetitive thoughts(ruminations)and *** regressions showed that depressive symptoms seem to contribute to explaining longer latencies for the interference condition(color word written in another color).*** have been published to improve the management of parents of a childhood cancer survivor,but parent-centered neuropsychological studies are still needed and should be developed to improve prevention and detection initiatives for these families.

关键词： Parents childhood cancer survivor Stroop intolerance of uncertainty

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Modification of the adsorption and catalytic properties of micro-and mesoporous materials by reactions with organometallic complexes

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Science China Chemistry 2010年第9期53卷 1862-1869页

作者： LEFEbVRE Frédéric PUTAJ Piotr bASSET Jean-marie Université de Lyon ICL C2P2 UMR 5265(CNRS-CPE-Université Lyon 1) LCOMS-CPE Lyon

This review describes the work of two laboratories in the field of the modification of micro-and mesoporous molecular sieves through reactions with organometallic *** modification of zeolites can occur inside the pore channels or on the external surface,depending on the size of the organometallic *** the modification occurs on the external surface,it results in a decrease of the pore entrance,which will lead in turn to a modification of the sorption properties of the zeolite,by decreasing the rate of the adsorption(mainly by a kinetic control).Such a material can be also used in catalysis,because the external acid sites,which are responsible for side-reactions,have been removed upon *** small organometallic complexes are used,they can fill the channels and cages of the zeolite and react with internal hydroxyl *** to the high acidity of zeolites,the reaction occurs very easily(for example at-100℃ on faujasite),in contrast to what is observed on the external surface,therefore leading to high metal *** that case,the modification of the sorption properties will be mainly related to a thermodynamic *** resulting materials can be useful in catalysis,by combining the activity of the organometallic complex and properties(for example shape-selectivity) of the *** of mesoporous molecular sieves occurs always in the pores and results in altering of the sorption properties of the solid,by changing the interaction type between the sorbent and the *** example the sorption isotherm of alkanes is changed from type II to type III according to the IUPAC nomenclature.

关键词： zeolites transition metals organometallic compounds adsorption properties

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Early plasmapheresis and rituximab for acute humoral rejection after AbO-compatible liver transplantation

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World Journal of Gastroenterology 2009年第27期15卷 3426-3430页

作者： Nassim Kamar Laurence Lavayssière Fabrice Muscari Janick Selves Céline Guilbeau-Frugier Isabelle Cardeau Laure Esposito Olivier Cointault marie béatrice Nogier Jean marie Peron Philippe Otal Marylise Fort Lionel Rostaing department of Nephrology Dialysis and Multi-Organ Transplantation CHU RangueilTSA 50032 31059 Toulouse Cédex 9 France INSERM U858 IFR 31 CHU Rangueil TSA 50032 31059 Toulouse Cédex 9 France department of Liver Transplantation CHU RangueilTSA 50032 31059 Toulouse Cédex 9 France department of Histopathology CHU PurpanTSA 50032 31059 Toulouse Cédex 9 France department of Histopathology CHU RangueilTSA 50032 31059 Toulouse Cédex 9 France department of Histopathology CHU PurpanTSA 50032 31059 Toulouse Cédex 9 France department of Radiology CHU RangueilTSA 50032 31059 Toulouse Cédex 9 France department of Immunology CHU RangueilTSA 50032 31059 Toulouse Cédex 9 France INSERM U563 IFR 30 CHU PurpanTSA 50032 31059 Toulouse Cédex 9 France

Acute humoral rejection （AHR） is uncommon after AbO- compatible liver transplantation. Herein, we report two cases of AHR treated with plasmapheresis and rituximab in two AbO-compatible liver-transplant patients with preformed anti-human leukocyte antigen donor-specific antibodies. Patient 1 experienced a biopsy-proven AHR at day 10 post-transplant. She was treated by steroid pulses, and OKT3. because of persisting signs of biopsy-proven AHR at day 26, she was treated by plasmapheresis and rituximab. Uver enzyme levels did not improve, and she died on day 41. Patient 2 experienced a biopsy-proven AHR on day 10 post-transplant. She was treated by steroid pulses, plasmapheresis, and rituximab. Liver enzymes returned to within normal range 18 d after diagnosis. Uver biopsies, at 3 and 9 mo post-transplant, showed complete resolution of AHR. We conclude that plasmapheresis should be started as soon as AHR is diagnosed, and be associated with a b-cell depleting agent. Rituximab may be considered as a first-line therapy.

关键词： Acute humoral rejection Liver transplantation donor-specific antibodies Rituximab Plasmapheresis

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direct molecular-level near-field plasmon and temperature assessment in a single plasmonic hotspot

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Light(Science & Applications) 2020年第1期9卷 1673-1685页

作者： marie Richard-Lacroix Volker deckert Leibniz Institute of Photonic Technology(IPHT) Albert-Einstein-Strasse 9D-07745 JenaGermany Institute of Physical Chemistry and Abbe Center of Photonics University of JenaHelmholtzweg 4D-07743 JenaGermany

Tip-enhanced Raman spectroscopy(TERS)is currently widely recognized as an essential but still emergent technique for exploring the ***,our lack of comprehension of crucial parameters still limits its potential as a user-friendly analytical *** tip’s surface plasmon resonance,heating due to near-field temperature rise,and spatial resolution are undoubtedly three challenging experimental parameters to ***,they are also the most fundamentally relevant parameters to explore,because they ultimately influence the state of the investigated molecule and consequently the probed *** we propose a straightforward and purely experimental method to access quantitative information of the plasmon resonance and near-field temperature experienced exclusively by the molecules directly contributing to the TERS *** detailed near-field optical response,both at the molecular level and as a function of time,is evaluated using standard TERS experimental equipment by simultaneously probing the Stokes and anti-Stokes spectral ***-assembled 16-mercaptohexadodecanoic acid monolayers covalently bond to an ultra-flat gold surface were used as a *** of blinking lines in the spectra also provides crucial information on the lateral resolution and indication of atomic-scale thermally induced morphological changes of the tip during the *** study provides access to unprecedented molecular-level information on physical parameters that crucially affect experiments under TERS *** study thereby improves the usability of TERS in day-to-day *** obtained information is of central importance for any experimental plasmonic investigation and for the application of TERS in the field of nanoscale thermometry.

关键词： straight purely thereby

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