Dergiler / The Journal of Pediatric Research / 2019 / Cilt: 6 - Sayı: 2

Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis

Sayfa
121–127
DOI
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⚡ TL;DR(1 Cümlede Hızlı Çıkarım)

There is a need for further studies to determinebiomarkers for vascular involvement.

📌 3 Maddede Bu Makale

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Amaç & Hipotez:Aim: Cardiovascular involvement is common in patients with mucopolysaccharidoses (MPS).
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Temel Bulgular & Veri:In this study, we investigated the effects ofthe markers involved in vascular endothelial injury pathogenesis [transforming growth factor β- (TGF-β)], interleukin-6 (IL-6), IL-10, highsensitive-C reactive protein (hs-CRP), vascular endothelial growth factor (VEGF), N-terminal pro-Natriuretic peptide (NT-proBNP) and theclinical, 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 ageand sex were included in the study.Results: Corneal clouding was seen in 29 (78%) patients.
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Sonuç & Katkı:There is a need for further studies to determinebiomarkers for vascular involvement.
🔬Tür:Araştırma Makalesi
📍Kapsam:Genişletilmiş Akademik Örneklem
⚙️Değişkenler:Alan Parametreleri

Özet

Aim: Cardiovascular involvement is common in patients with mucopolysaccharidoses (MPS). In this study, we investigated the effects ofthe markers involved in vascular endothelial injury pathogenesis [transforming growth factor β- (TGF-β)], interleukin-6 (IL-6), IL-10, highsensitive-C reactive protein (hs-CRP), vascular endothelial growth factor (VEGF), N-terminal pro-Natriuretic peptide (NT-proBNP) and theclinical, 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 ageand 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 withhearing 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 statisticallysignificant increase in the patient group for NT-proBNP and VEGF (p=0.04, p=0.03, respectively). The carotid intima media thickness wasstatistically significantly higher in the patient group (p<0.001). The left ventricular diastolic diameter was significantly higher in the patientgroup (p=0.009), intraventricular septum thickness was significantly higher in the patient group (p<0.001). The E/A ratio was significantlylower 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, proinflammatorymarkers are elevated in patients with vascular damage like other lysosomal diseases. There is a need for further studies to determinebiomarkers for vascular involvement.

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Bu Makaleye Atıf Yap

CANDA, E., KÖSE, M., KAĞNICI, M., DONDURMACI, M., UÇAR, S. K., SÖZMEN, E., LEVENT, R. E., ÇOKER, M. (2019). Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis. The Journal of Pediatric Research, Cilt: 6 - Sayı: 2, 121–127. https://ajindex.com/articles/362901

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Kaynakça (15)

  1. Wraith JE. The mucopolysaccharidoses: a clinical review and guide to management. Arch Dis Child 1995;72:263-7
  2. Ferrari S, Ponzin D, Ashworth JL, et al. Diagnosis and management of ophthalmological features in patients with mucopolysaccharidosis. Br J Ophthalmol 2011;95:613-9
  3. Lachman R, Martin KW, Castro S, Basto MA, Adams A, Teles EL. Radiologic and neuroradiologic findings in the mucopolysaccharidoses. J Pediatr Rehabil Med 2010;3:109-18
  4. Braunlin EA, Harmatz PR, Scarpa M, et al. Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management J Inherit Metab Dis 2011;34:1183-97
  5. Braunlin EA, Berry JM, Whitley CB. Cardiac findings after enzyme replacement therapy for mucopolysaccharidosis Type I. Am J Cardiol 2006;98:416-8
  6. Rentería VG, Ferrans VJ, Roberts WC. The heart in the Hurler syndrome: gross, histologic and ultrastructural observations in five necropsy cases. Am J Cardiol 1976;38:487-501
  7. Hishitani T, Wakita S, Isoda T, Katori T, Ishizawa A, Okada R. Sudden death in Hunter syndrome caused by complete atrioventricular block. J Pediatr 2000;136:268-9
  8. Latif N, Sarathchandra P, Taylor PM, Antoniw J, Yacoub MH. Localization and pattern of expression of extracellular matrix components in human heart valves. J Heart Valve Dis 2005;14:218-27
  9. Chen YL, Wu KH. Airway management of patients with craniofacial abnormalities: 10-year experience at a teaching hospital in Taiwan. J Chin Med Assoc. 2009;72:468-70
  10. Dangel JH. Cardiovascular changes in children with mucopolysaccharide storage diseases and related disordersclinical and echocardiographic findings in 64 patients. Eur J Pediatr 1998;157:534-8
  11. Brands MM, Frohn-Mulder IM, Hagemans ML, et al. Mucopolysaccharidosis: cardiologic features and effects of enzyme-replacement therapy in 24 children with MPS I, II and VI. J Inherit Metab Dis 2013;36:227-34
  12. Wang RY, Covault KK, Halcrow EM, et al. Carotid intima-media thickness is increased in patients with mucopolysaccharidosis. Mol Genet Metab 2011;104:592-6
  13. Yano S, Moseley K, Wong L, Castelnovi C, Azen C, Pavlova Z. Glycosaminoglycan metabolism defects and atherosclerosis: frequent association of endothelial dysfunction in patients with Mucopolysaccharidosis. J Inherit Metab Dis 2014;37:255-61
  14. Wippermann CF, Beck M, Schranz D, Huth R, Michel-Behnke I, Jüngst BK. Mitral and aortic regurgitation in 84 patients with mucopolysaccharidoses. Eur J Pediatr 1995;154:98-101
  15. Sahn DJ, De