Search Results - "Jan Stolk"

Hereditary alpha-1-antitrypsin deficiency and its clinical consequences by Fregonese, Laura, Stolk, Jan

“…Alpha-1-antitrypsin deficiency (AATD) is a genetic disorder that manifests as pulmonary emphysema, liver cirrhosis and, rarely, as the skin disease…”
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European Respiratory Society statement: diagnosis and treatment of pulmonary disease in α1-antitrypsin deficiency by Miravitlles Marc, Dirksen Asger, Ferrarotti Ilaria, Koblizek Vladimir, Lange, Peter, Mahadeva Ravi, McElvaney, Noel G, Parr, David, Piitulainen Eeva, Roche, Nicolas, Stolk, Jan, Thabut Gabriel, Turner, Alice, Vogelmeier Claus, Stockley, Robert A

“…α1-antitrypsin deficiency (AATD) is the most common hereditary disorder in adults. It is associated with an increased risk of developing pulmonary emphysema…”
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Mesenchymal stromal cells: a novel therapy for the treatment of chronic obstructive pulmonary disease? by Broekman, Winifred, Khedoe, Padmini P S J, Schepers, Koen, Roelofs, Helene, Stolk, Jan, Hiemstra, Pieter S

“…COPD is characterised by tissue destruction and inflammation. Given the lack of curative treatments and the progressive nature of the disease, new treatments…”
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Governmental Non-Pharmaceutical Interventions during the COVID-19 Pandemic and the COPD Exacerbation and Respiratory Infection Rate in Patients with Alpha-1 Antitrypsin Deficiency by Kappe, Naomi Nanke, Alagem, Noga, Tov, Naveh, Stolk, Jan

“…During the COVID-19 pandemic the number of hospital admissions due to chronic obstructive pulmonary disease (COPD) exacerbations was significantly reduced. The…”
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Methods of Purification and Application Procedures of Alpha1 Antitrypsin: A Long-Lasting History by Viglio, Simona, Iadarola, Paolo, D'Amato, Maura, Stolk, Jan

“…The aim of the present report is to review the literature addressing the methods developed for the purification of alpha1-antitrypsin (AAT) from the 1950s to…”
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Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry by Miravitlles, Marc, Turner, Alice M, Torres-Duran, María, Tanash, Hanan, Rodríguez-García, Carlota, López-Campos, José Luis, Chlumsky, Jan, Guimaraes, Catarina, Rodríguez-Hermosa, Juan Luis, Corsico, Angelo, Martinez-González, Cristina, Hernández-Pérez, José María, Bustamante, Ana, Parr, David G, Casas-Maldonado, Francisco, Hecimovic, Ana, Janssens, Wim, Lara, Beatriz, Barrecheguren, Miriam, González, Cruz, Stolk, Jan, Esquinas, Cristina, Clarenbach, Christian F

“…Alpha-1 antitrypsin deficiency (AATD) is a rare disease that is associated with an increased risk of pulmonary emphysema. The European AATD Research…”
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Protease-Specific Biomarkers to Analyse Protease Inhibitors for Emphysema Associated with Alpha 1-Antitrypsin Deficiency. An Overview of Current Approaches by Viglio, Simona, Bak, Elisabeth G, Schouten, Iris G M, Iadarola, Paolo, Stolk, Jan

“…As a known genetic cause of chronic obstructive pulmonary disease (COPD), alpha1-antitrypsin deficiency (AATD) can cause severe respiratory problems at a…”
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A quantitative method for detection of spliced X-box binding protein-1 (XBP1) mRNA as a measure of endoplasmic reticulum (ER) stress by van Schadewijk, Annemarie, van't Wout, Emily F. A., Stolk, Jan, Hiemstra, Pieter S.

“…Endoplasmic reticulum (ER) stress is increasingly recognized as an important mechanism in a wide range of diseases including cystic fibrosis, alpha-1…”
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Retinoic acid signaling balances adult distal lung epithelial progenitor cell growth and differentiation by Ng-Blichfeldt, John-Poul, Schrik, Anneke, Kortekaas, Rosa K., Noordhoek, Jacobien A., Heijink, Irene H., Hiemstra, Pieter S., Stolk, Jan, Königshoff, Melanie, Gosens, Reinoud

“…Despite compelling data describing pro-regenerative effects of all-trans retinoic acid (ATRA) in pre-clinical models of chronic obstructive pulmonary disease…”
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Therapeutic efficacy of α-1 antitrypsin augmentation therapy on the loss of lung tissue: an integrated analysis of 2 randomised clinical trials using computed tomography densitometry by Stockley, Robert A, Parr, David G, Piitulainen, Eeva, Stolk, Jan, Stoel, Berend C, Dirksen, Asger

“…Two randomised, double-blind, placebo-controlled trials have investigated the efficacy of IV alpha-1 antitrypsin (AAT) augmentation therapy on emphysema…”
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Treatment Effect of Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension Quantified by Automatic Comparative Imaging in Computed Tomography Pulmonary Angiography by Zhai, Zhiwei, Ota, Hideki, Staring, Marius, Stolk, Jan, Sugimura, Koichiro, Takase, Kei, Stoel, Berend C

“…OBJECTIVESBalloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) can have variable outcomes. To…”
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The Course of AαVal541 as a Proteinase 3 Specific Neo-Epitope after Alpha-1-Antitrypsin Augmentation in Severe Deficient Patients by Schouten, Iris G. M., Mumford, Richard A., Moes, Dirk Jan A. R., Hiemstra, Pieter S., Stolk, Jan

“…In alpha-1-antitrypsin deficiency (AATD), neutrophil serine proteases such as elastase and proteinase 3 (PR3) are insufficiently inhibited. A previous study in…”
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Modulation of Airway Epithelial Innate Immunity and Wound Repair by M(GM-CSF) and M(M-CSF) Macrophages by van Riet, Sander, van Schadewijk, Annemarie, de Vos, Steve, Vandeghinste, Nick, Rottier, Robbert J., Stolk, Jan, Hiemstra, Pieter S., Khedoe, Padmini

“…Airway epithelial cells and macrophages participate in inflammatory responses to external noxious stimuli, which can cause epithelial injury. Upon injury,…”
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The Relationship between Plasma Alpha-1-Antitrypsin Polymers and Lung or Liver Function in ZZ Alpha-1-Antitrypsin-Deficient Patients by Sark, Annelot D, Fromme, Malin, Olejnicka, Beata, Welte, Tobias, Strnad, Pavel, Janciauskiene, Sabina, Stolk, Jan

“…Alpha-1-Antitrypsin (AAT) is a protein of the SERPINA1 gene. A single amino acid mutation (Lys342Glu) results in an expression of misfolded Z-AAT protein,…”
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Regional lung densities in alpha-1 antitrypsin deficiency compared to predicted values by Stoel, Berend C, Stolk, Jan, Bakker, M Els, Parr, David G

“…We developed a method to calculate a standard score for lung tissue mass derived from CT scan images from a control group without respiratory disease. We…”
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Fibrosis-Related Gene Profiling in Liver Biopsies of PiZZ α1-Antitrypsin Children with Different Clinical Courses by Kamp, Jan C, Kappe, Naomi N, Moro, Carlos Fernández, Fuge, Jan, Kuehnel, Mark P, Wrenger, Sabine, Welte, Tobias, Hoek, Bart van, Jonigk, Danny D, Khedoe, Padmini P S J, Strnad, Pavel, Björnstedt, Mikael, Stolk, Jan, Janciauskiene, Sabina, Nemeth, Antal

“…PiZZ (Glu342Lys) α1-antitrypsin deficiency (AATD) is characterized by intrahepatic AAT polymerization and is a risk factor for liver disease development in…”
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Correction: Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry by Miravitlles, Marc, Turner, Alice M, Torres-Duran, María, Tanash, Hanan, Rodríguez-García, Carlota, López-Campos, José Luis, Chlumsky, Jan, Guimaraes, Catarina, Rodríguez-Hermosa, Juan Luis, Corsico, Angelo, Martinez-González, Cristina, Hernández-Pérez, José María, Bustamante, Ana, Parr, David G, Casas-Maldonado, Francisco, Hecimovic, Ana, Janssens, Wim, Lara, Beatriz, Barrecheguren, Miriam, González, Cruz, Stolk, Jan, Esquinas, Cristina, Clarenbach, Christian F

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Identification of Novel Short C-Terminal Transcripts of Human SERPINA1 Gene by Matamala, Nerea, Aggarwal, Nupur, Iadarola, Paolo, Fumagalli, Marco, Gomez-Mariano, Gema, Lara, Beatriz, Martinez, Maria Teresa, Cuesta, Isabel, Stolk, Jan, Janciauskiene, Sabina, Martinez-Delgado, Beatriz

“…Human SERPINA1 gene is located on chromosome 14q31-32.3 and is organized into three (IA, IB, and IC) non-coding and four (II, III, IV, V) coding exons. This…”
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Polymerization of misfolded Z alpha-1 antitrypsin protein lowers CX3CR1 expression in human PBMCs by Tumpara, Srinu, Ballmaier, Matthias, Wrenger, Sabine, König, Mandy, Lehmann, Matthias, Lichtinghagen, Ralf, Martinez-Delgado, Beatriz, Korenbaum, Elena, DeLuca, David, Jedicke, Nils, Welte, Tobias, Fromme, Malin, Strnad, Pavel, Stolk, Jan, Janciauskiene, Sabina

“…Expression levels of CX3CR1 (C-X3-C motif chemokine receptor 1) on immune cells have significant importance in maintaining tissue homeostasis under…”
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Pattern of emphysema distribution in α1-antitrypsin deficiency influences lung function impairment by PARR, David G, STOEL, Berend C, STOLK, Jan, STOCKLEY, Robert A

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