Academic Journal
Multimodal analysis of granulocytes, monocytes, and platelets in patients with cystic fibrosis before and after Elexacaftor–Tezacaftor–Ivacaftor treatment
| العنوان: | Multimodal analysis of granulocytes, monocytes, and platelets in patients with cystic fibrosis before and after Elexacaftor–Tezacaftor–Ivacaftor treatment |
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| المؤلفون: | Schmidt, Hanna, Höpfer, Larissa Melina, Wohlgemuth, Lisa, Knapp, Christiane Leonie, Mohamed, Adam Omar Khalaf, Stukan, Laura, Münnich, Frederik, Hüsken, Dominik, Koller, Alexander Sebastian, Stratmann, Alexander Elias Paul, Müller, Paul, Braun, Christian Karl, Fabricius, Dorit, Bode, Sebastian Felix Nepomuk, Huber-Lang, Markus, Messerer, David Alexander Christian |
| سنة النشر: | 2023 |
| المجموعة: | OPUS FAU - Online publication system of Friedrich-Alexander-Universität Erlangen-Nürnberg |
| مصطلحات موضوعية: | ddc:610 |
| الوصف: | Cystic fibrosis (CF) is a monogenetic disease caused by an impairment of the cystic fibrosis transmembrane conductance regulator (CFTR). CF affects multiple organs and is associated with acute and chronic inflammation. In 2020, Elexacaftor–Tezacaftor–Ivacaftor (ETI) was approved to enhance and restore the remaining CFTR functionality. This study investigates cellular innate immunity, with a focus on neutrophil activation and phenotype, comparing healthy volunteers with patients with CF before (T1, n = 13) and after six months (T2, n = 11) of ETI treatment. ETI treatment reduced sweat chloride (T1: 95 mmol/l (83|108) vs. T2: 32 mmol/l (25|62), p < 0.01, median, first|third quartile) and significantly improved pulmonal function (FEV 1 T1: 2.66 l (1.92|3.04) vs. T2: 3.69 l (3.00|4.03), p < 0.01). Moreover, there was a significant decrease in the biomarker human epididymis protein 4 (T1: 6.2 ng/ml (4.6|6.3) vs. T2: 3.0 ng/ml (2.2|3.7), p < 0.01) and a small but significant decrease in matrix metallopeptidase 9 (T1: 45.5 ng/ml (32.5|140.1) vs. T2: 28.2 ng/ml (18.2|33.6), p < 0.05). Neutrophil phenotype (CD10, CD11b, CD62L, and CD66b) and function (radical oxygen species generation, chemotactic and phagocytic activity) remained largely unaffected by ETI treatment. Likewise, monocyte phenotype and markers of platelet activation were similar at T1 and T2. In summary, the present study confirmed a positive impact on patients with CF after ETI treatment. However, neither beneficial nor harmful effects of ETI treatment on cellular innate immunity could be detected, possibly due to the study population consisting of patients with well-controlled CF. |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/pdf; image/tiff |
| اللغة: | English |
| Relation: | https://opus4.kobv.de/opus4-fau/frontdoor/index/index/docId/23626; urn:nbn:de:bvb:29-opus4-236261; https://nbn-resolving.org/urn:nbn:de:bvb:29-opus4-236261; https://doi.org/10.3389/fimmu.2023.1180282; https://opus4.kobv.de/opus4-fau/files/23626/DataSheet_1.pdf; https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282.pdf; https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282-g001.tif; https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282-g002.tif; https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282-g003.tif; https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282-g004.tif |
| DOI: | 10.3389/fimmu.2023.1180282 |
| الاتاحة: | https://opus4.kobv.de/opus4-fau/frontdoor/index/index/docId/23626 https://nbn-resolving.org/urn:nbn:de:bvb:29-opus4-236261 https://doi.org/10.3389/fimmu.2023.1180282 https://opus4.kobv.de/opus4-fau/files/23626/DataSheet_1.pdf https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282.pdf https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282-g001.tif https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282-g002.tif https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282-g003.tif https://opus4.kobv.de/opus4-fau/files/23626/fimmu-14-1180282-g004.tif |
| Rights: | https://creativecommons.org/licenses/by/4.0/deed.de ; info:eu-repo/semantics/openAccess |
| رقم الانضمام: | edsbas.86B8AE37 |
| قاعدة البيانات: | BASE |
| DOI: | 10.3389/fimmu.2023.1180282 |
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