Investigation of SCUBE-1 levels in pediatric patients with beta-thalassemia

Abstract

Aim: A thrombosis and inflammation marker, Signal peptide-CUB (complement C1r/C1 s)-EGF (epidermal growth factor)-like domain-containing protein 1 (SCUBE-1) is expressed in endothelial cells and activated platelets. We aimed to investigate the levels of this marker in patients with Thalassemia major (TM), a disease which causes a tendency to thrombosis.

Methods: Twenty patients with TM and age and gender-matched twenty healthy controls were included in this prospective case-control study. Other thrombosis markers such as D-dimer and homocysteine were studied in addition to SCUBE-1 in both groups.

Results: The SCUBE1 and D-dimer levels were higher in the TM group compared to the control group (P=0.004, P=0.023, respectively). A significant positive correlation was detected between platelet count and SCUBE1 (r=0.342, P=0.031).

Conclusion: We detected elevated SCUBE1 levels in children with TM compared to the healthy controls, which correlated with platelet count. SCUBE1 may be used as a thrombosis marker in TM in the early stages of the disease.

Keywords

• Beta-thalassemia major,Children,SCUBE-1,Thrombosis

Beta-talasemi majörlü pediatrik hastalarda SCUBE-1 düzeylerinin araştırılması

Öz

Amaç: Sinyal peptidi-CUB (tamamlayıcı C1r / C1s) -EGF (epidermal büyüme faktörü) benzeri alan içeren protein 1 (SCUBE-1), endotel hücreleri ve aktive olmuş trombositlerde eksprese edilen tromboz ve inflamasyon belirteci olarak kullanılan bir proteindir. Tromboza eğilim yaratan bir hastalık olan talasemi majörde bu belirtecin düzeylerini incelemeyi amaçladık.

Yöntemler: Bu kesitsel vaka-kontrol çalışmasına SCUBE-1 düzeylerini belirleyebilmek için 20 talasemi majorlü hasta ve kontrol grubu olarak da benzer yaş ve cinsiyette 20 sağlıklı çocuk dahil edildi. D-dimer, homosistein gibi diğer tromboz belirteçleri SCUBE-1 ek olarak her iki grupta çalışıldı.

Bulgular: Gruplar arasında SCUBE-1 ve D-dimer düzeyleri açısından anlamlı fark vardı (P=0,004, P=0,023, sırasıyla). Trombosit sayısı ile SCUBE-1 arasında anlamlı pozitif korelasyon tespit edildi (r=0,342, P=0,031).

Sonuç: SCUBE-1 seviyelerinin talasemi majorlü çocuk hastalarda sağlıklılara nazaran yüksek olduğunu ve trombosit sayısı ile korele olduğunu tespit ettik. SCUBE-1 belki erken evrelerde tromboz belirteci olarak kullanılabilir.

Anahtar Kelimeler

• Beta-talasemi major,Çocuklar,SCUBE-1,Tromboz

Kaynakça

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