Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis
Aim: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 β- (TGF-β)], interleukin-6 (IL-6), IL-10, high sensitive-C reactive protei...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Galenos Yayinevi
2019-06-01
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| Series: | Journal of Pediatric Research |
| Subjects: | |
| Online Access: |
http://jpedres.org/archives/archive-detail/article-preview/evaluation-of-cardiovascular-nvolvement-and-cytoki/27902
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| _version_ | 1828015975981645824 |
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| author | Ebru Canda Melis Köse Mehtap Kağnıcı Meral Dondurmacı Sema Kalkan Uçar Eser Sözmen Reşit Ertürk Levent Mahmut Çöker |
| author_facet | Ebru Canda Melis Köse Mehtap Kağnıcı Meral Dondurmacı Sema Kalkan Uçar Eser Sözmen Reşit Ertürk Levent Mahmut Çöker |
| author_sort | Ebru Canda |
| collection | DOAJ |
| description | Aim: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 β- (TGF-β)], 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-β, 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. |
| first_indexed | 2024-04-10T10:27:02Z |
| format | Article |
| id | doaj.art-ded71b00331b4eb4900d1363c422fb05 |
| institution | Directory Open Access Journal |
| issn | 2147-9445 2587-2478 |
| language | English |
| last_indexed | 2024-04-10T10:27:02Z |
| publishDate | 2019-06-01 |
| publisher | Galenos Yayinevi |
| record_format | Article |
| series | Journal of Pediatric Research |
| spelling | doaj.art-ded71b00331b4eb4900d1363c422fb052023-02-15T16:21:20ZengGalenos YayineviJournal of Pediatric Research2147-94452587-24782019-06-016212112710.4274/jpr.galenos.2018.2154813049054Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with MucopolysaccharidosisEbru Canda0Melis Köse1Mehtap Kağnıcı2Meral Dondurmacı3Sema Kalkan Uçar4Eser Sözmen5Reşit Ertürk Levent6Mahmut Çöker7 Ege University Faculty of Medicine, Department of Pediatrics, Division of Metabolism and Nutrition, İzmir, Turkey Ege University Faculty of Medicine, Department of Pediatrics, Division of Metabolism and Nutrition, İzmir, Turkey Ege University Faculty of Medicine, Department of Pediatrics, Division of Metabolism and Nutrition, İzmir, Turkey Ege University Faculty of Medicine, Department of Biochemistry, İzmir, Turkey Ege University Faculty of Medicine, Department of Pediatrics, Division of Metabolism and Nutrition, İzmir, Turkey Ege University Faculty of Medicine, Department of Biochemistry, İzmir, Turkey Ege University Faculty of Medicine, Department of Pediatrics, Division of Cardiology, İzmir, Turkey Ege University Faculty of Medicine, Department of Pediatrics, Division of Metabolism and Nutrition, İzmir, Turkey Aim: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 β- (TGF-β)], 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-β, 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. http://jpedres.org/archives/archive-detail/article-preview/evaluation-of-cardiovascular-nvolvement-and-cytoki/27902 Mucopolysaccharidosescarotis intima media thicknessvascular involvementcytokines |
| spellingShingle | Ebru Canda Melis Köse Mehtap Kağnıcı Meral Dondurmacı Sema Kalkan Uçar Eser Sözmen Reşit Ertürk Levent Mahmut Çöker Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis Journal of Pediatric Research Mucopolysaccharidoses carotis intima media thickness vascular involvement cytokines |
| title | Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis |
| title_full | Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis |
| title_fullStr | Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis |
| title_full_unstemmed | Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis |
| title_short | Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis |
| title_sort | evaluation of cardiovascular involvement and cytokine levels in patients with mucopolysaccharidosis |
| topic | Mucopolysaccharidoses carotis intima media thickness vascular involvement cytokines |
| url |
http://jpedres.org/archives/archive-detail/article-preview/evaluation-of-cardiovascular-nvolvement-and-cytoki/27902
|
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