Premature atherosclerosis in children with beta-thalassemia major: New diagnostic marker

Abstract

BackgroundEarly vascular alteration, atherosclerosis and coronary artery disease have emerged as important cardiovascular complications among beta-thalassemia major (B-TM) patients. The aims of the current study were to assess the prevalence of premature atherosclerosis among our B-TM patients, and to investigate the diagnostic value of serum Osteoprotegerin assay as an early biomarker for atherosclerosis.MethodsThis cross-sectional study was conducted at Hematology unit - Pediatric Department, Zagazig University Children Hospital- Egypt in the period from March 2014 to March 2015. A total of 115 children were enrolled in the current study; as sixty-five (65) children with beta thalassemia major aged 5–18 years, on regular blood transfusion regimen represented the patient group. While fifty (50) healthy children, with comparable age and gender, were assigned as control group. All participants were subjected to history taking, thorough clinical examination and laboratory investigations including; complete blood count, liver and kidney function tests, C- reactive protein, lipid profile, serum ferritin and serum Osteoprotegerin (OPG) assay. Also, carotid artery intima media thickness (CAIMT) was performed by duplex ultrasound for patients and controls.ResultsOur B-TM patients were transfusion-dependent for as long as 8.5 ± 3.8 years with significantly higher serum ferritin levels (2490 ± 1579 ng/dl vs 83 ± 32 ng/dl, p = 0.001), C-reactive protein (5.7 ± 5.7 vs 0.9 ± 0.9), liver enzymes and bilirubin when compared to controls. Significantly higher serum triglyceride (128 ± 20 vs 101 ± 7 mg/dL, p = 0.009) and atherogenic index of plasma (0.45 ± 0.12 vs 0.22 ± 0.04, p = 0.001) were recorded in patients than comparisons. On the contrary, total serum cholesterol (116 ± 16 vs 143 ± 5, p < 0.001), low density lipoprotein-cholesterol (LDL-C) (44 ± 9 vs 73 ± 6, p < 0.001) and high density lipoprotein cholesterol (HDL-C) (39 ± 2 vs 61 ± 5, p < 0.001), were significantly lowered in patients versus normal peers. Carotid arteries intima media thickness (CAIMT) of both side were significantly increased for patients (Rt 0.62 ± 0.2 vs. 0.29 ± 0.07 mm, p = 0.001 & Lt 0.66 ± 0.17 vs 0.29 ± 0.05 mm, p = 0.001) when compared with healthy controls, and showed positive correlation with, serum triglyceride, atherogenic index of plasma, and serum Osteoprotegerin levels. ELISA assay of serum Osteoprotegerin (OPG) revealed significantly higher levels for thalassemia patients than matched healthy controls (427 ± 102 vs. 324 ± 126 pg/ml, p = 0.02). Of particular interest is the obvious positive correlation between OPG levels and CAIMT of both sides (Rt r 0.54, p = 0.001 &Lt r 0.479, p = 0.001) and also with serum triglycerides (r 0.374, p = 0.03).ConclusionsSubclinical atherosclerosis started prematurely in children with beta- thalassemia. Carotid artery intima media thickness represented a simple, accurate and non-invasivemodality for early detection ofatherosclerosis. It was correlated well with serum Osteoprotegerin; this finding highlighte...

Assessment of Aortic Elasticity and the Doppler Tissue Echocardiography in Thalassemia Major Children

“…Prenner and Chirinos, 7 Zapolski et al, 8 and Sahin et al 9 found an increase of arterial stiffness parameters in diabetes mellitus, end stage renal and thalassemia major (TM) diseases compared with healthy in the order given. In addition, considering TM patients, Sherief et al 10 used the method of carotid artery intima media thickness and found a significant increase, while Cusmà Piccione et al 11 did not find. But the arterial stiffness was increased significantly.…”

“…2 Many studies have been conducted about the aortic elastic changes due to various diseases. [4][5][6][7][8][9][10][11] Bayar et al 4 found changes in arterial stiffness parameters in patients with celiac diseases compared with the healthy when Ülger et al 5 did not find any changes in asthma patients. In this regard, Bektaş et al 6 found a significant change in asthmatic patients compared with posttreatment asthmatic patients.…”

Modified conditioning regimen improves outcomes of unrelated donor peripheral blood stem cell transplantation for β‐thalassaemia major patients

“…β‐Thalassaemia major (β‐TM), a hereditary haemolytic anaemia induced by a dysfunction of β‐chains synthesis in haemoglobin, results from a β‐globin genetic mutation . It is prevalent in people of Mediterranean, central African, Southeast Asian, and South American descent.…”

Peningkatan Kadar Feritin Serum Memiliki Korelasi Negatif dengan Kadar High Density Lipoprotein pada Anak Penyandang Thalassemia Beta Mayor

“…3 Kadar zat besi yang tinggi di dalam darah akan menyebabkan peningkatan risiko terjadinya atherosklerosis melalui peningkatan zat radikal bebas. 4 Selain itu, anak dengan thalassemia beta juga memiliki risiko atherosklerosis prematur sebagai akibat dislipidemia. 3 Beberapa penelitian telah menunjukkan bahwa akumulasi zat besi di tubuh dapat menimbulkan gangguan profil lipid.…”

“…8 Dislipidemia pada thalassemia mayor disebabkan oleh berbagai faktor, antara lain dilusi plasma akibat anemia, meningkatnya aktivitas eritropoeisis yang disertai dengan pengambilan kolesterol oleh makrofag dan histiosit dalam sistem retikuloendotelial, gangguan fungsi hati karena penimbunan zat besi, gangguan hormonal, dan menurunnya aktivitas lipolisis di luar hati. 4 Gambaran profil lipid pada thalassemia memiliki korelasi dengan penumpukan zat besi di dalam darah. Penumpukan zat besi di dalam hati akan menimbulkan kerusakan hepatosit dan sel retikuloendotelial, yang ditandai oleh perkembangan fibrosis hingga terjadi sirosis.…”