Evaluation of Cardiovascular Involvement and Cytokine Levels in Patients with Mucopolysaccharidosis
Journal Title: The Journal of Pediatric Research - Year 2019, Vol 6, Issue 2
Abstract
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.
Authors and Affiliations
Ebru Canda, Melis Köse, Mehtap Kağnıcı, Meral Dondurmacı, Sema Kalkan Uçar, Eser Sözmen, Reşit Ertürk Levent, Mahmut Çöker
Keywords
Related Articles
- EP ID EP604349
- DOI 10.4274/jpr.galenos.2018.21548
- Views 87
- Downloads 0
Effect of Fatigue on Quality of Life in Pediatric Oncology Patients
As a result of the medical progress in the field of pediatric oncology, childhood cancers are seen as a chronic life-threatening disease rather than an acute or terminal disease. While methods used in cancer treatment in...
Early-Onset Isolated Bilateral Pheochromocytoma As a Major Clinical Manifestation of von-Hippel Lindau Syndrome Type 2C
Pheochromocytoma is a rare disease that is characterized by the increased production and secretion of catecholamines from the adrenal medulla. The disease is autosomal dominant, and frequently sporadic and unilateral. Ph...
Congenital Penil Lymphedema in a Preterm Neonate
Congenital penile lymph edema is a rare anomaly of unknown cause of the lymphatic vessels. Lymphedema is often the shortage of lymphatic collecting ducts, lymphatic hypoplasia, hyperplasia, as a result of aphasia or lymp...
Pediatric Bilateral Pheochromocytoma and Experience of Laparoscopic Cortical Sparing Adrenalectomy
Pheochromocytomas are neuroendocrine tumors. In this report, we present a 15-year-old girl who had cerebral palsy and pheochromocytoma. She also had a diagnosis of hyperinsulinemic hypoglycemia in her history. She underw...
The Results of Simulation Training in Pediatric Nursing Students’ Education
Aim: Simulation training that started with low realistic models in health education has developed in recent years and is now widely used in medicine and nursing education. The purpose of the present research is to compar...