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Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis

2018; Galenos Yayinevi; Linguagem: Inglês

10.4274/jpr.21548

2587-2478

Ebru Canda, Melis Köse, Mehtap Kağnıcı, Meral Dondurmacı, Sema Kalkan Uçar, Eser Yıldırım Sözmen, Ertürk Levent, Mahmut Çöker,

Apelin-related biomedical research

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-IL 6, IL 10, high sensitive C reactive protein-hs CRP, vascular endothelial growth factor-VEGF, N-terminal pro-Natriuretic peptide-NT-proBNP) and 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, hsCRP, TGF-β, IL6, IL10 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).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).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