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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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Homing and migration of mesenchymal stromal cells: How to improve the efficacy of cell therapy?

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World Journal of Stem Cells 2016年第3期8卷 73-87页

作者： Ann De Becker Ivan Van Riet department Clinical HematologyStem Cell Laboratory Universitair Ziekenhuis Brussel Vrije Universiteit Brussel (VUB)

Mesenchymal stromal cells(MSCs) are currently being investigated for use in a wide variety of clinical applications. For most of these applications, systemic delivery of the cells is preferred. However, this requires the homing and migration of MSCs to a target tissue. Although MSC hominghas been described, this process does not appear to be highly efficacious because only a few cells reach the target tissue and remain there after systemic administration. This has been ascribed to low expression levels of homing molecules, the loss of expression of such molecules during expansion, and the heterogeneity of MSCs in cultures and MSC culture protocols. To overcome these limitations, different methods to improve the homing capacity of MSCs have been examined. Here, we review the current understanding of MSC homing, with a particular focus on homing to bone marrow. In addition, we summarize the strategies that have been developed to improve this process. A better understanding of MSC biology, MSC migration and homing mechanisms will allow us to prepare MSCs with optimal homing capacities. The efficacy of therapeutic applications is dependent on efficient delivery of the cells and can, therefore, only benefit from better insights into the homing mechanisms.

关键词： Mesenchymal stromal cells Homing Bone marrow Homing receptors Extravasation

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Step-up approach in emphysematous hepatitis: A case report

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World Journal of Hepatology 2022年第2期14卷 464-470页

作者： Silke Francois Maridi Aerts Hendrik Reynaert Ruth Van Lancker Johan Van Laethem Rastislav Kunda Nouredin Messaoudi department of Gastroenterology Universitair Ziekenhuis BrusselBrussels 1090Belgium department of Gastroenterology and Hepatology Vrije Universiteit BrusselUniversitair Ziekenhuis BrusselBrussels 1090Belgium department of Intensive Care Vrije Universiteit BrusselUniversitair Ziekenhuis BrusselBrussels 1090Belgium department of Infectious Diseaeses Vrije Universiteit BrusselUniversitair Ziekenhuis BrusselBrussels 1090Belgium department of Surgery Vrije Universiteit BrusselUniversitair Ziekenhuis BrusselBrussels 1090Belgium department of Surgery Europe HospitalsBrussels 1180Belgium

BACKGROUND Emphysematous hepatitis(EH)is a rare,rapidly progressive fulminant gasforming infection of the liver *** is often fatal and mostly affects diabetes *** SUMMARY We report a case of EH successfully managed by a step-up approach consisting of aggressive hemodynamic support,intravenous antibiotics,and percutaneous drainage,ultimately followed by laparoscopic *** 11 documented cases worldwide,only 1 of the patients survived,treated by urgent laparotomy and surgical *** EH is a life-threatening *** high mortality rate makes timely diagnosis essential,in order to navigate treatment accordingly.

关键词： Emphysematous hepatitis Septic shock Step-up approach Percutaneous drainage Laparoscopic deroofing Case report

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Sturge–Weber syndrome:an update for the pediatrician

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World Journal of Pediatrics 2024年第5期20卷 435-443页

作者： Emilie Dingenen Damien Segers Hannelore De Maeseneer Dirk Van Gysel Ghent University Faculty of Medicine and Health Sciences GhentBelgium department of Pediatrics O.L.Vrouw Hospital AalstMoorselbaan 1649300AalstBelgium Interdisciplinary Unit of Pediatric Dermatology Vrije Universiteit Brussel(VUB)Universitair Ziekenhuis Brussel(UZ Brussel)BrusselsBelgium

Sturge–Weber syndrome(SWS)is a rare congenital neurocutaneous disorder characterized by the simultaneous presence of both cutaneous and extracutaneous capillary *** usually presents as a facial port-wine birthmark,with a varying presence of leptomeningeal capillary malformations and ocular vascular *** latter may lead to significant neurological and ocular morbidity such as epilepsy and *** is most often caused by a somatic mutation involving the G protein subunit alpha Q or G protein subunit alpha 11 gene causing various alterations in downstream signaling *** specifically conducted a comprehensive review focusing on the current knowledge of clinical practices,the latest pathophysiological insights,and the potential novel therapeutic avenues they *** sources A narrative,non-systematic review of the literature was conducted,combining expert opinion with a balanced review of the available literature.A search of PubMed,Google Scholar and Embase was conducted,using keywords“Sturge–Weber Syndrome”OR“SWS”,“Capillary malformations”,“G protein subunit alpha 11”OR“G protein subunit alpha Q”.Results One of the hallmark features of SWS is the presence of a port-wine birthmark at birth,and forehead involvement is most indicative for *** most common ocular manifestations of SWS are glaucoma and choroidal *** presents in either in infancy(0–3 years of age)or later in *** complications are common in SWS,occurring in about 70%–80%of patients,with seizures being the most common *** significantly impacts the quality of life for patients and their families,and requires a multidisciplinary approach for diagnosis and ***,no disease-modifying therapies exist,and treatment is mostly focused on symptoms or complications as they *** SWS remains a complex and heterogeneous *** research is needed to optimize diagnostic and therapeutic strategies,and to transl

关键词： Capillary malformations G-protein Gq-G11 Port-wine birthmark Neurocutaneous disorder Sturge-Weber syndrome

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The Diagnostic and Therapeutic Challenges of Fabry Nephropathy—A Review of the Literature, Illustrated by a Clinical Case

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Open Journal of Nephrology 2023年第4期13卷 349-368页

作者： Stefan Van Cauwelaert Caroline Geers Dominique Vandervelde Esther Scheirlynck Alexander Gheldof Karl-Martin Wissing department of Nephrology and Arterial Hypertension Universitair Ziekenhuis Brussel (UZ Brussel) Vrije Universiteit Brussel (VUB) Brussels Belgium department of Pathology Universitair Ziekenhuis Brussel (UZ Brussel) Vrije Universiteit Brussel (VUB) Brussels Belgium department of Nephrology H&ocircpitaux Iris-Sud (HIS) Brussels Belgium department of Cardiology Universitair Ziekenhuis Brussel (UZ Brussel) Vrije Universiteit Brussel (VUB) Brussels Belgium department of Medical Genetics Universitair Ziekenhuis Brussel (UZ Brussel) Vrije Universiteit Brussel (VUB) Brussels Belgium

Fabry Disease (FD) is a rare lysosomal storage disorder characterized by α-galactosidase A (α-Gal A) enzyme deficiency, resulting in glycosphingolipid accumulation. Its clinical spectrum ranges from severe classical to milder nonclassical or late-onset phenotypes. Renal involvement, termed Fabry Nephropathy (FN), can vary from mild proteinuria to kidney failure. FN diagnosis, especially in nonclassical cases with a genetic Variant of Unknown Significance (VUS) in the GLA gene, poses challenges. Measurement of plasma lyso-Gb3 levels is gaining importance in FN diagnosis, while renal biopsy with electron microscopy remains the gold standard in equivocal cases. Treatment options include Enzyme Replacement Therapy (ERT) and chaperone therapy, demanding careful candidate selection due to high treatment costs. Research has predominantly focused on classical FD, revealing modest treatment benefits. However, evidence for treating patients, especially females, with milder nonclassical or late-onset phenotypes is scarce, emphasizing the necessity for placebo-controlled clinical trials in these subgroups. Meanwhile, participation in global FD registries can improve our understanding of disease management. Case Presentation: A woman in her late sixties presented with moderate chronic kidney disease, mild proteinuria, and microscopic hematuria. Her family history included a prevalence of renal, cardiac and cerebrovascular diseases. Kidney biopsy revealed characteristic myelin figures and zebra bodies in podocytes, strongly suggestive of FN. Genetic analysis identified a VUS in the GLA gene (c.655A > C, ***219Leu), introducing diagnostic uncertainty. Further investigations revealed severe cardiac involvement. Considering the recurring difficulty presented by the finding of a VUS in the GLA gene during FN assessments, along with the uncertainty regarding the need for treatment in nonclassical or late-onset FD phenotypes, especially in women, this case becomes a central focus for

关键词： Fabry Disease Fabry Nephropathy Variants of Unknown Significance Diagnosis Treatment Selection Lysosomal Storage Disorder α-Galactosidase A Glycosphingolipid Accumulation Enzyme Replacement Therapy Migalastat

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A Second Dose of GnRHa in Combination with Luteal GnRH Antagonist May Eliminate Ovarian Hyperstimulation Syndrome in Women with≥30 Follicles Measuring≥11 mm in Diameter on Trigger Day and/or Pre-trigger Peak Estradiol Exceeding 10 000 pg/mL

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Current Medical Science 2019年第2期39卷 278-284页

作者： Ling DENG Xue-Ian LI De-sheng YE Christophe Blockeel Xing-yu ZHOU Shi-ling CHEN Xin CHEN Center for Reproductive Medicine Department of Gynecology and ObstetricsNanfang HospitalSouthern Medical UniversityGuangzhou 510515China department of Reproductive Medicine Jiangmen Central HospitalJiangmen 529030China Centre for Reproductive Medicine Universitair Ziekenhuis Brussel(UZ Brussel)Laarbeeklaan 101-1090 BrusselsBelgium

This observational study included 21 patients at remarkably high risk of ovarian hyperstimulation syndrome(OHSS),characterized by more than 30 follicles measuring≥11 mm in diameter on trigger day and/or pre-trigger peak estradiol exceeding 10 000 pg/*** was also the feature of women with established severe early OHSS followed by gonadotrophin-releasing hormone agonist(GnRHa)trigger and freeze-all policy that previously have been *** patients received a second dose of GnRHa 12 h after the first GnRHa trigger combined with administration of GnRH antagonist at 0.25 mg/day for a period of 3 days from the day of oocyte retrieval *** in vitro fertilization(IVF)outcomes may be preferable compared with a bolus of GnRHa trigger and none of the included patients developed moderate-to-severe ***,patients'symptoms,reproductive honnone levels and ultrasound findings were improved *** new strategy seems to be efficacious and could be a further supplement of GnRHa trigger with or without applying freeze-all strategy to completely prevent early-onset moderate to severe OHSS,especially for the patients characterized by≥30 follicles measuring≥11 mm in diameter on trigger day and/or pre-trigger peak estradiol exceeding 10 000 pg/*** studies should be performed to compare this regimen with conventional methods of OHSS prevention.

关键词： in-vitro fertilization ovarian hyperstimulation syndrome GnRH agonist GnRH antagonist freeze-all

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Individualized Isonatremic and Hyponatremic Dialysate Improves Blood Pressure in Patients with Intradialytic Hypertension: A Prospective Cross-Over Study with 24-h Ambulatory Blood Pressure Monitoring

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Open Journal of Nephrology 2020年第2期10卷 144-157页

作者： Tom Robberechts Mandelina Allamani Xavier Galloo Karl Martin Wissing Patricia Van Der Niepen department of Nephrology and Hypertension Universitair Ziekenhuis Brussel VUB Brussels Belgium

Background. Intradialytic hypertension, a paradoxical rise in systolic blood pressure from pre- to postdialysis, is a poorly understood and difficult-to-treat phenomenon. We examined the effects of individually adjusted isonatremic and hyponatremic dialysate on intradialytic and interdialytic blood pressure in patients with intradialytic hypertension. Methods. We enrolled 11 patients with intradialytic hypertension in a prospective randomized cross-over study, with 4 treatment periods of different dialysate sodium concentrations. Period 1 (run-in) and 3 (wash-out) were standardized at 140 mEq/L;period 2 and 4 with iso- or hyponatremic sodium dialysate. Blood pressure was recorded each dialysis session, and 24-hour ambulatory blood pressure monitoring was performed at the end of each treatment period. Results. Isonatremic and hyponatremic dialysate were associated with significantly lower pre- and post-dialysis blood pressure as compared to baseline 140 mEq/L dialysate (predialysis 148.3 ± 24.7/67.7 ± 12.0 and 144.4 ± 16.5/68.8 ± 13.3 vs. 158.0 ± 18.3/75.6 ± 11.4 mmHg, resp p = 0.04 and 0.007 for systolic and p = 0.004 and 0.04 for diastolic blood pressure;postdialysis 154.2 ± 25.5/76.6 ± 14.1 and 142.5 ± 20.7/73.0 ± 12.9 vs. 159.1 ± 21.6/80.3 ± 12.1 mmHg, resp NS and p = 0.01 for systolic and NS and p = 0.04 for diastolic blood pressure). Postdialysis and 24 h systolic blood pressure tended to be lower with hyponatremic compared to isonatremic dialysate. Conclusion. Individually tailoring dialysate sodium concentration, based on the sodium set-point of each patient, resulted in a lower pre- and post-dialysis blood pressure in patients with intradialytic hypertension. 24 h blood pressure values tended to be lower as well with hyponatremic dialysate.

关键词： Intradialytic Hypertension Ambulatory Blood Pressure Monitoring Dialysate Sodium Concentration

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专门设计的低于0.1mSv 3D CT联合使用MBIR诊断疑似颅缝早闭的儿童:质量评价

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国际医学放射学杂志 2016年第3期 328-329页

作者： C.W.Ernst T.L.Hulstaert D.Belsack N.Buls G.V.Gompel K.H.Nieboer 陈思璇 department of radiology Universitair Ziekenhuis Brussel

摘要目的对怀疑颅缝早闭的儿童进行回顾性分析比较低剂量CT和标准CT的影像质量。方法对48例年龄0～35个月颅骨畸形的患儿,进行颅骨3D CT扫描来评估颅缝开放程度:2009—2010年,24例患儿进行标准CT(CTDIvol32.18 m Gy)成像,从2011—2012年... 详细信息

摘要目的对怀疑颅缝早闭的儿童进行回顾性分析比较低剂量CT和标准CT的影像质量。方法对48例年龄0～35个月颅骨畸形的患儿,进行颅骨3D CT扫描来评估颅缝开放程度:2009—2010年,24例患儿进行标准CT(CTDIvol32.18 m Gy)成像,从2011—2012年,24例患儿进行低剂量

关键词：儿童 CT MBIR 颅缝早闭

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通过低管电压结合先进图像重建算法降低腹部CT的对比剂和辐射剂量

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国际医学放射学杂志 2015年第3期 299-300页

作者： N.Buls G.Van Gompel T.Van Cauteren K.Nieboer I.Willekens G.Verfaillie 何杰 department of radiology Universitair Ziekenhuis Brussel ( UZ Brussel )Laarbeeklaan 101 1090 Brussels Belgium

目的评估低管电压联合两种类型的迭代重建(IR)方法用于4种不同低对比剂用量的腹部CT中的影像质量。方法用120 k V及标准的320 mg I/m L对比剂用量对小

关键词：低管电压迭代重建对比剂辐射剂量腹部躯干 CT

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It takes two to tango: the recruiter’s role in accepting or refusing to participate in group antenatal care among pregnant women—an exploration through in-depth interviews

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Family Medicine and Community Health 2023年第3期11卷 102-113页

作者： Florence Talrich Astrid Van Damme Hilde Bastiaens Marlies Rijnders Jochen Bergs Katrien Beeckman department of Public Health Nursing and Midwifery Research Group(NUMID)Faculty of Medicine and PharmacyVrije Universiteit Brussel(VUB)BrusselsBelgium department of Nursing and Midwifery Research Group(NUMID) Universitair Ziekenhuis Brussel(UZ Brussel)BrusselsBelgium department of Family Medicine and Population Health Faculty of Medicine and Health SciencesUniversiteit AntwerpenAntwerpBelgium department of Child Health TNOLeidenThe Netherlands Research Group of Healthcare and Ethics Faculty of Medicine and Life SciencesUniversiteit HasseltHasseltBelgium Centre for Research and Innovation in Care(CRIC) Universiteit AntwerpenAntwerpBelgium

Objective The purpose of this study was to explore how women are recruited for group antenatal care(GANC)in primary care organisations(PCOs),what elements influence the behaviour of the recruiter,and what strategies recruiters use to encourage women to *** Using a qualitative research design,we conducted 10 in-depth interviews with GANC facilitators working in *** constructs of the domains of the Consolidated Framework for Implementation Research and the Theoretical Domains Framework helped to develop interview questions and raise awareness of important elements during interviews and thematic *** facilitators working in multidisciplinary PCOs located in brussels and Flanders(Belgium)were invited to participate in an *** purposively selected participants because of their role as GANC facilitators and *** recruited GANC facilitators up until data saturation and no new elements *** We identified that the recruitment process consists of four phases or actions:identification of needs and potential obstacles for participation;selection of potential participants;recruitment for GANC and reaction to *** on the phase,determinants at the level of the woman,recruiter,organisation or environment have an influence on the recruitment *** Our study concludes that it takes two to tango for successful recruitment for *** participants’needs and wishes are of importance,but the care providers’behaviour should not be ***,successful recruitment may be improved when introducing a multidisciplinary recruitment plan consisting of specific strategies,as we suggest.

关键词： interview behaviour qualitative

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