Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis

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dc.contributor.authorCanda, Ebru
dc.contributor.authorKöse, Melis
dc.contributor.authorKağnıcı, Mehtap
dc.contributor.authorDondurmacı, Meral
dc.contributor.authorUçar, Sema Kalkan
dc.contributor.authorSözmen, Eser
dc.contributor.authorÇoker, Mahmut
dc.date.accessioned2020-12-01T12:36:39Z
dc.date.available2020-12-01T12:36:39Z
dc.date.issued2019
dc.departmentEge Üniversitesien_US
dc.description.abstractAim: Cardiovascular involvement is common in patients with mucopolysaccharidoses (MPS). in this study, we investigated the effects of the markers involved in vascular endothelial injury pathogenesis [transforming growth factor ?- (TGF-?)], interleukin-6 (IL-6), IL-10, high sensitive-C reactive protein (hs-CRP), vascular endothelial growth factor (VEGF), N-terminal pro-Natriuretic peptide (NT-proBNP) and the clinical, laboratory and echocardiographic findings of the patients. Materials and Methods: A total of 37 patients (5 MPS I, 4 MPS II, 2 MPS IIIa, 4 MPS IIIb, 14 MPS IVa, 8 MPS VI) and 32 controls with similar age and sex were included in the study. Results: Corneal clouding was seen in 29 (78%) patients. There were 23 (62%) patients with organomegaly, and 28 (75%) patients with hearing loss. When the groups were compared in terms of NT-proBNP, hs-CRP, TGF-?, IL-6, IL-10 and VEGF levels, there was a statistically significant increase in the patient group for NT-proBNP and VEGF (p=0.04, p=0.03, respectively). the carotid intima media thickness was statistically significantly higher in the patient group (p<0.001). the left ventricular diastolic diameter was significantly higher in the patient group (p=0.009), intraventricular septum thickness was significantly higher in the patient group (p<0.001). the E/A ratio was significantly lower in the patient group (p<0.001). Conclusion: Cardiac involvement in MPS patients is a major cause of mortality and morbidity. It is thought that cytokines, proinflammatory markers are elevated in patients with vascular damage like other lysosomal diseases. There is a need for further studies to determine biomarkers for vascular involvement.en_US
dc.identifier.doi10.4274/jpr.galenos.2018.21548
dc.identifier.endpage127en_US
dc.identifier.issn2147-9445
dc.identifier.issn2587-2478
dc.identifier.issue2en_US
dc.identifier.startpage121en_US
dc.identifier.urihttps://doi.org/10.4274/jpr.galenos.2018.21548
dc.identifier.urihttps://app.trdizin.gov.tr//makale/TXpZd05UWTJOZz09
dc.identifier.urihttps://hdl.handle.net/11454/67000
dc.identifier.volume6en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakTR-Dizinen_US
dc.language.isoenen_US
dc.relation.ispartofThe Journal of Pediatric Researchen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subject0-Belirleneceken_US
dc.titleEvaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosisen_US
dc.typeArticleen_US

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