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Article ; Online: Neurofilament light protein as a biomarker for spinal muscular atrophy: a review and reference ranges.

Bayoumy, Sherif / Verberk, Inge M W / Vermunt, Lisa / Willemse, Eline / den Dulk, Ben / van der Ploeg, Ans T / Pajkrt, Dasja / Nitz, Elisa / van den Hout, Johanna M P / van der Post, Julie / Wolf, Nicole I / Beerepoot, Shanice / Groen, Ewout J N / Tüngler, Victoria / Teunissen, Charlotte E

Clinical chemistry and laboratory medicine

2024

Abstract: Spinal muscular atrophy (SMA) is the leading genetic cause of infant mortality, characterized by progressive neuromuscular degeneration resulting from mutations in the survival motor neuron ( ...

Abstract	Spinal muscular atrophy (SMA) is the leading genetic cause of infant mortality, characterized by progressive neuromuscular degeneration resulting from mutations in the survival motor neuron (
Language	English
Publishing date	2024-01-15
Publishing country	Germany
Document type	Journal Article ; Review
ZDB-ID	1418007-8
ISSN	1437-4331 ; 1434-6621 ; 1437-8523
ISSN (online)	1437-4331
ISSN	1434-6621 ; 1437-8523
DOI	10.1515/cclm-2023-1311
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Article ; Online: Longitudinal Effects of 1-Year Smoking Cessation on Human Bronchial Epithelial Transcriptome.

Rathnayake, Senani N H / Ditz, Benedikt / Willemse, Brigitte W M / Timens, Wim / Kooistra, Wierd / Heijink, Irene H / Oliver, Brian G G / van den Berge, Maarten / Faiz, Alen

Chest

2023 Volume 164, Issue 1, Page(s) 85–89

MeSH term(s)	Humans ; Smoking Cessation ; Transcriptome ; Bronchi ; Lung ; Longitudinal Studies
Language	English
Publishing date	2023-01-28
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1032552-9
ISSN	1931-3543 ; 0012-3692
ISSN (online)	1931-3543
ISSN	0012-3692
DOI	10.1016/j.chest.2022.12.050
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Ui III Zs.45: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Research gaps and opportunities in autoimmune hepatitis-Results of the international autoimmune hepatitis group research workshop 2022.

Snijders, Romée J A L M / Assis, David N / Oo, Ye H / Sebode, Marcial / Taubert, Richard / Willemse, José / Tomsin, Bert / Lohse, Ansgar W / Drenth, Joost P H / Gevers, Tom J G

Liver international : official journal of the International Association for the Study of the Liver

2023 Volume 43, Issue 7, Page(s) 1375–1384

Abstract: Autoimmune hepatitis (AIH) is a rare autoimmune liver disease that is characterised by a chronic inflammatory immune reaction directed against hepatocytes. The disease results in a substantial reduction in quality of life and potentially leads to liver- ... ...

Abstract	Autoimmune hepatitis (AIH) is a rare autoimmune liver disease that is characterised by a chronic inflammatory immune reaction directed against hepatocytes. The disease results in a substantial reduction in quality of life and potentially leads to liver-related complications or death. The International Autoimmune Hepatitis Group (IAIHG) initiated a series of research workshops to uncover the scientific gaps and opportunities in AIH. This review summarises the results of the latest workshop in Maastricht in 2022 and reviews the current challenges in adult AIH, particularly in relation to four important aspects of AIH: diagnostics; new immunomodulatory therapies; clinical trial design; and unmet clinical needs. This review also summarises the progress made since the AIH workshop in 2017. Patients and patient representatives were actively involved in the parallel working groups alongside clinicians and researchers. Despite 40 years of experience with diagnosing and treating AIH, false diagnoses occur and treatment is still based on nonselective immunosuppression. In addition to the need for more specific diagnostic tests, prognostic markers and tailor-based treatments, a major unmet clinical need was identified in areas of care delivery and health-related quality of life.
MeSH term(s)	Adult ; Humans ; Hepatitis, Autoimmune/diagnosis ; Hepatitis, Autoimmune/drug therapy ; Evidence Gaps ; Quality of Life ; Liver Diseases ; Inflammation
Language	English
Publishing date	2023-04-10
Publishing country	United States
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	2102783-3
ISSN	1478-3231 ; 1478-3223
ISSN (online)	1478-3231
ISSN	1478-3223
DOI	10.1111/liv.15573
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Book ; Online: A process algebra with global variables

Bouwman, Mark / Luttik, Bas / Schols, Wouter / Willemse, Tim A. C.

2020

Abstract: In standard process algebra, parallel components do not share a common state and communicate through synchronisation. The advantage of this type of communication is that it facilitates compositional reasoning. For modelling and analysing systems in which ...

Abstract	In standard process algebra, parallel components do not share a common state and communicate through synchronisation. The advantage of this type of communication is that it facilitates compositional reasoning. For modelling and analysing systems in which parallel components operate on shared memory, however, the communication-through-synchronisation paradigm is sometimes less convenient. In this paper we study a process algebra with a notion of global variable. We also propose an extension of Hennessy-Milner logic with predicates to test and set the values of the global variables, and prove correspondence results between validity of formulas in the extended logic and stateless bisimilarity and between validity of formulas in the extended logic without the set operator and state-based bisimilarity. We shall also present a translation from the process algebra with global variables to a fragment of mCRL2 that preserves the validity of formulas in the extended Hennessy-Milner logic. Comment: In Proceedings EXPRESS/SOS 2020, arXiv:2008.12414
Keywords	Computer Science - Logic in Computer Science
Subject code	006
Publishing date	2020-08-31
Publishing country	us
Document type	Book ; Online
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Article ; Online: Paraneoplastic pemphigus associated with post-transplant lymphoproliferative disorder after small bowel transplantation.

Fidder, Sander A R / Bolling, Marieke C / Diercks, Gilles F H / Pas, Hendri H / Hooimeijer, Louise H L / Bungener, Laura B / Willemse, Brigitte W M / Scheenstra, Rene / Stapelbroek, Janneke M / van der Doef, Hubert P J

Pediatric transplantation

2021 Volume 25, Issue 6, Page(s) e14023

Abstract: Background: PNP is a malignancy-associated autoimmune mucocutaneous syndrome due to autoantibodies against plakins, desmogleins, and other components of the epidermis and basement membrane of epithelial tissues. PNP-causing malignancies comprise mainly ... ...

Abstract	Background: PNP is a malignancy-associated autoimmune mucocutaneous syndrome due to autoantibodies against plakins, desmogleins, and other components of the epidermis and basement membrane of epithelial tissues. PNP-causing malignancies comprise mainly lymphoproliferative and hematologic neoplasms. PNP is extremely rare, especially in children. Methods: Here, we present the first case of a child who developed PNP on a PTLD after small bowel transplantation because of a severe genetic protein-losing enteropathy. Results: The patient in this case report had a severe stomatitis, striate palmoplantar keratoderma, and lichenoid skin lesions. In addition, she had marked esophageal involvement. She had lung pathology due to recurrent pulmonary infections and ventilator injury. Although we found no evidence of BO, she died from severe pneumonia and respiratory failure at the age of 12 years. Conclusion: It is exceptional that, despite effective treatment of the PTLD, the girl survived 5 years after her diagnosis of PNP. We hypothesize that the girl survived relatively long after the PNP diagnosis due to strong T-cell suppressive treatments for her small bowel transplantation.
MeSH term(s)	Child ; Fatal Outcome ; Female ; Humans ; Immunosuppressive Agents/therapeutic use ; Intestine, Small/transplantation ; Lymphoproliferative Disorders/complications ; Paraneoplastic Syndromes/diagnosis ; Pemphigus/diagnosis ; Protein-Losing Enteropathies/surgery ; Twins, Monozygotic
Chemical Substances	Immunosuppressive Agents
Language	English
Publishing date	2021-05-20
Publishing country	Denmark
Document type	Case Reports
ZDB-ID	1390284-2
ISSN	1399-3046 ; 1397-3142
ISSN (online)	1399-3046
ISSN	1397-3142
DOI	10.1111/petr.14023
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Zs.A 4947: Show issues

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Article ; Online: Age- and disease-specific reference values for neurofilament light presented in an online interactive support interface.

Vermunt, Lisa / Otte, Marco / Verberk, Inge M W / Killestein, Joep / Lemstra, Afina W / van der Flier, Wiesje M / Pijnenburg, Yolande A L / Vijverberg, Everard G B / Bouwman, Femke H / Gravesteijn, Gido / van de Berg, Wilma D J / Scheltens, Philip / van Harten, Argonde C / Willemse, Eline A J / Teunissen, Charlotte E

Annals of clinical and translational neurology

2022 Volume 9, Issue 11, Page(s) 1832–1837

Abstract: Interpretation of axonal damage biomarker Neurofilament Light chain (NfL) concentrations is difficult due to the lack of age-specific and disease-specific reference values. We here developed an interactive interface to support interpretation of NfL ... ...

Abstract	Interpretation of axonal damage biomarker Neurofilament Light chain (NfL) concentrations is difficult due to the lack of age-specific and disease-specific reference values. We here developed an interactive interface to support interpretation of NfL results in human body fluids. We used NfL values of 1698 individuals without a neurological disorder, aged 19-85 years, and patients with MS and dementias. Percentile regression estimates per diagnosis populate interactive graphs, alongside NfL background information (available on: https://mybiomarkers.shinyapps.io/Neurofilament). This accessible interface provides reference for interpretation of the individual patient results for clinicians. It showcases an adaptable method to support interpretation of age-dependent biomarkers in neurology.
MeSH term(s)	Humans ; Intermediate Filaments ; Reference Values ; Biomarkers ; Axons
Chemical Substances	Biomarkers
Language	English
Publishing date	2022-10-05
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2740696-9
ISSN	2328-9503 ; 2328-9503
ISSN (online)	2328-9503
ISSN	2328-9503
DOI	10.1002/acn3.51676
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Article ; Online: Feasibility of clinical studies of chemical exchange saturation transfer magnetic resonance imaging of prostate cancer at 7 T.

Reesink, Daan J / Arteaga de Castro, Catalina S / Van der Velden, Tijl / Van Vooren, Jeanette / Oost, Petri / Jonges, Trudy G N / Lam, Marnix G E H / de Keizer, Bart / Willemse, Peter-Paul M / Meijer, Richard P / Klomp, Dennis W J

NMR in biomedicine

2023 Volume 36, Issue 10, Page(s) e4958

Abstract: Chemical exchange saturation transfer (CEST) has been explored for differentiation between tumour and benign tissue in prostate cancer (PCa) patients. With ultrahigh field strengths such as 7-T, the increase of spectral resolution and sensitivity could ... ...

Abstract	Chemical exchange saturation transfer (CEST) has been explored for differentiation between tumour and benign tissue in prostate cancer (PCa) patients. With ultrahigh field strengths such as 7-T, the increase of spectral resolution and sensitivity could allow for selective detection of amide proton transfer (APT) at 3.5 ppm and a group of compounds that resonate at 2 ppm (i.e., [poly]amines and/or creatine). The potential of 7-T multipool CEST analysis of the prostate and the detection of PCa was studied in patients with proven localised PCa who were scheduled to undergo robot-assisted radical prostatectomy (RARP). Twelve patients were prospectively included (mean age 68.0 years, mean serum prostate-specific antigen 7.8ng/mL). A total of 24 lesions larger than 2 mm were analysed. Used were 7-T T2-weighted (T2W) imaging and 48 spectral CEST points. Patients received 1.5-T/3-T prostate magnetic resonance imaging and galium-68-prostate-specific membrane antigen-positron emission tomography/computerised tomography to determine the location of the single-slice CEST. Based on the histopathological results after RARP, three regions of interest were drawn on the T2W images from a known malignant zone and benign zone in the central and peripheral zones. These areas were transposed to the CEST data, from which the APT and 2-ppm CEST were calculated. The statistical significance of the CEST between the central zone, the peripheral zone, and tumour was calculated using a Kruskal-Wallis test. The z-spectra showed that APT and even a distinct pool that resonated at 2 ppm were detectable. This study showed a difference trend in the APT levels, but no difference in the 2-ppm levels when tested between the central zone, the peripheral zone, and tumour (H(2) = 4.8, p = 0.093 and H(2) = 0.86, p = 0.651, respectively). Thus, to conclude, we could most likely detect APT and amines and/or creatine levels noninvasively in prostate using the CEST effect. At group level, CEST showed a higher level of APT in the peripheral versus the central zone; however, no differences of APT and 2-ppm levels were observed in tumours.
MeSH term(s)	Male ; Humans ; Aged ; Creatine ; Feasibility Studies ; Magnetic Resonance Imaging/methods ; Prostatic Neoplasms/diagnostic imaging ; Protons ; Amides/chemistry ; Amines
Chemical Substances	Creatine (MU72812GK0) ; Protons ; Amides ; Amines
Language	English
Publishing date	2023-05-08
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1000976-0
ISSN	1099-1492 ; 0952-3480
ISSN (online)	1099-1492
ISSN	0952-3480
DOI	10.1002/nbm.4958
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Zs.A 2869: Show issues

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Article ; Online: Scalable Production of Size-Controlled Cholangiocyte and Cholangiocarcinoma Organoids within Liver Extracellular Matrix-Containing Microcapsules.

van Tienderen, Gilles S / Willemse, Jorke / van Loo, Bas / van Hengel, Eline V A / de Jonge, Jeroen / van der Laan, Luc J W / Leijten, Jeroen / Verstegen, Monique M A

Cells

2022 Volume 11, Issue 22

Abstract: Advances in biomaterials, particularly in combination with encapsulation strategies, have provided excellent opportunities to increase reproducibility and standardization for cell culture applications. Herein, hybrid microcapsules are produced in a flow- ... ...

Abstract	Advances in biomaterials, particularly in combination with encapsulation strategies, have provided excellent opportunities to increase reproducibility and standardization for cell culture applications. Herein, hybrid microcapsules are produced in a flow-focusing microfluidic droplet generator combined with enzymatic outside-in crosslinking of dextran-tyramine, enriched with human liver extracellular matrix (ECM). The microcapsules provide a physiologically relevant microenvironment for the culture of intrahepatic cholangiocyte organoids (ICO) and patient-derived cholangiocarcinoma organoids (CCAO). Micro-encapsulation allowed for the scalable and size-standardized production of organoids with sustained proliferation for at least 21 days in vitro. Healthy ICO (
MeSH term(s)	Humans ; Organoids/metabolism ; Capsules ; Reproducibility of Results ; Cell Differentiation ; Liver/metabolism ; Extracellular Matrix ; Cholangiocarcinoma/metabolism ; Tumor Microenvironment
Chemical Substances	Capsules
Language	English
Publishing date	2022-11-18
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2661518-6
ISSN	2073-4409 ; 2073-4409
ISSN (online)	2073-4409
ISSN	2073-4409
DOI	10.3390/cells11223657
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Article ; Online: New and Interesting Fungi. 5.

Crous, P W / Begoude, B A D / Boers, J / Braun, U / Declercq, B / Dijksterhuis, J / Elliott, T F / Garay-Rodriguez, G A / Jurjević, Ž / Kruse, J / Linde, C C / Loyd, A / Mound, L / Osieck, E R / Rivera-Vargas, L I / Quimbita, A M / Rodas, C A / Roux, J / Schumacher, R K /

Starink-Willemse, M / Thangavel, R / Trappe, J M / van Iperen, A L / Van Steenwinkel, C / Wells, A / Wingfield, M J / Yilmaz, N / Groenewald, J Z

Fungal systematics and evolution

2022 Volume 10, Page(s) 19–90

Abstract: Nine new genera, 17 new species, nine new combinations, seven epitypes, three lectotypes, one neotype, and 14 interesting new host and / or geographical records are introduced in this study. New genera: ...

Abstract	Nine new genera, 17 new species, nine new combinations, seven epitypes, three lectotypes, one neotype, and 14 interesting new host and / or geographical records are introduced in this study. New genera:
Language	English
Publishing date	2022-09-08
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	3007859-3
ISSN	2589-3831 ; 2589-3823
ISSN (online)	2589-3831
ISSN	2589-3823
DOI	10.3114/fuse.2022.10.02
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Article ; Online: Haemorrhagic stroke related to the use of 4-fluoroamphetamine.

Wijers, C H W / Visser, M C / van Litsenburg, R T H / Niesink, R J M / Willemse, R B / Croes, Esther A

Journal of neurology

2018 Volume 265, Issue 7, Page(s) 1607–1611

Abstract: ... hemiparalysis and severe aphasia, requiring clinical rehabilitation. Patient B had a subarachnoid haemorrhage ...

Abstract	Introduction: The use of the new psychoactive substance 4-fluoroamphetamine (4-FA) and the number of 4-FA-related intoxications substantially increased in The Netherlands in recent years. We describe two patients with severe 4-FA-related complications and the characteristics of a large sample of 4-FA-intoxicated patients. Methods: Information on patients with 4-FA-related intoxications between January 2009 and June 2017 was available from the Monitor Drug-related Incidents. Detailed clinical information was obtained of two patients with haemorrhagic stroke after toxicologically confirmed 4-FA use. Results: We report on two patients who presented with headache and mild hypertension after 4-FA use. Patient A developed one-sided weakness and decreased consciousness after a few hours. A computed tomography scan showed a left-sided intracerebral haemorrhage. Because of life-threatening cerebral herniation, haematoma evacuation was performed. Postoperatively, she suffered from a right-sided hemiparalysis and severe aphasia, requiring clinical rehabilitation. Patient B had a subarachnoid haemorrhage without neurological deficits. In total, 939 4-FA-intoxicated patients were registered. These patients used 4-FA alone (44%) or in combination with alcohol (13%) and/or other drugs (43%). Discussion: Patients using 4-FA are at risk for life-threatening health problems, including intracranial haemorrhage. Additional brain imaging should be considered in 4-FA-intoxicated patients, not only in the presence of neurological deficits, but also in the case of severe headache.
MeSH term(s)	Adolescent ; Adult ; Amphetamines/adverse effects ; Female ; Frontal Lobe/diagnostic imaging ; Humans ; Intracranial Hemorrhages/chemically induced ; Intracranial Hemorrhages/complications ; Intracranial Hemorrhages/diagnostic imaging ; Intracranial Hemorrhages/epidemiology ; Male ; Retrospective Studies ; Street Drugs/adverse effects ; Stroke/chemically induced ; Stroke/complications ; Stroke/diagnostic imaging ; Stroke/epidemiology ; Young Adult
Chemical Substances	Amphetamines ; Street Drugs ; 4-fluoroamphetamine (S5744XYR1Z)
Language	English
Publishing date	2018-05-08
Publishing country	Germany
Document type	Case Reports ; Journal Article ; Review
ZDB-ID	187050-6
ISSN	1432-1459 ; 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
ISSN (online)	1432-1459
ISSN	0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
DOI	10.1007/s00415-018-8888-6
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