Talasemi Major Hastalarında D Vitamini ile Sol Ventrikül Fonksiyonu Arasındaki İlişki

Year 2024, Volume: 21 Issue: 3, 440 - 443

Defne Ay Tuncel , Şule Çalışkan Kamış , Begül Yağcı-küpeli

https://doi.org/10.35440/hutfd.1566315

Abstract

Amaç: Talasemi Major (TM) hastalarında D vitamini düzeylerinin (VDL) sol ventriküler fonksiyon (LVF) ile ilişkisini değerlendirmeyi amaçladık.
Materyal ve Metod: 1 Haziran 2022 ve 1 Haziran 2023 tarihleri arasında Adana Tıp Fakültesi, Sağlık Bilimleri Üniversitesi, Adana Şehir Eğitim ve Araştırma Hastanesi'nde (AŞEAH) pediatrik hematoloji onkoloji kliniğine başvuran TM'li hastalar çalışmaya alınmıştır. Rutin tetkikleri yapılarak, 25-Hidroksivitamin D (25(OH)D) düzeyleri, ferritin değerleri, Ekokardiyografi (EKHO) Ejeksiyon Fraksiyonu (EF), Fraksiyonel Kısalma (FS) verileri hasta kayıtlarından elde edildi.
Bulgular: Çalışmaya 89 TM hastası dahil edildi. Kadın oranı %46,1 (n=41), %53,9'u erkek (n=48) idi. Vakaların yaşları bir ile yirmi dört yaş arasında değişiyordu ve ortalama on yaş idi. Vakaların 84'ü 0-18 yaş arasında (%94,4), 5'i (%5,6) 18 yaş üzerindeydi. Medyan ferritin düzeyi 1497 µg/L (68-11354), VDL 18 µg/L (3-52), EF 66 (37-77) ve FS 36 (21-67) olarak belirlendi. Anlamlı bir ters korelasyon TM hastala-rında ferritin düzeyleri ile VDL bulundu. (p=0,026, r=-0,235).
TM hastalarında VDL, EF ve FS düzeyleri arasında korelasyon saptanmadı (p=0,684, r_0,44; p=0,082, r=0,185).
Sonuç: TM tanısı alan hastalarda demir yükü ile VDL arasında ters ilişki gözlendi. Kardiyak disfonksiyon demir birikiminin bir sonucu olmasına rağmen, bu çalışma düşük VDL'li hastalarda anlamlı kalp bozuk-luğunu ortaya koymamıştır. Mevcut veriler bu eksikliğin kardiyak sistolik fonksiyonlar üzerindeki doğ-rudan etkilerini desteklememektedir. Bu bulgular Talasemi Major hastalarında D vitamini durumunun izlenmesinin ve demir yükünün kontrol edilmesinin önemini vurgulamaktadır.

Keywords

Transfüzyon bağımlı talasemi, d vitamini, kalp yetmezliği

Project Number

2639/08.06.2023

The Relationship Between Vitamin D and Left Ventricular Function in Thalassemia Major Patients

Abstract

Background: Our goal was to evaluate a correlation vitamin D levels(VDL) with left ventricular function (LVF) in Thalassemia Major (TM) patients.
Materials and Methods: Patients with TM who presented to the PHOC at Adana Faculty of Medicine, Health Sciences University, Adana City Training and Research Hospital(ACTRH) during June 1, 2022 and June 1, 2023, were eligible for the study. Routine investigations were performed, and data on 25-Hydroxyvitamin D (25(OH)D) levels, ferritin values, Echocardiography (ECHO) Ejection Fraction (EF), and Fractional Shortening (FS) were obtained from patient records .
Results: The study included 89 patients of TM. The female ratio was 46.1% (n=41), while 53.9% were male (n=48). The cases ranged in age from one to twenty-four years, with a median of ten. Out of the cases, 84 were between the ages of 0 and 18 (94.4%), with 5 over the age of 18 (5.6%). The median ferritin level was 1497 µg/L (68-11354), VDL was 18 µg/L (3-52), EF was 66 (37-77), and FS was 36 (21-67). A significant inverse correlation was found in VDL with ferritin levels in TM patients. (p=0.026, r=-0.235).
No correlation was found between VDL, EF, and FS levels in TM patients (p=0.684, r_0.44; p=0.082, r=0.185).
Conclusions: An inverse relationship was observed between iron burden and VDL in patients diagno-sed with TM. Although cardiac dysfunction is a consequence of iron accumulation, this study did not reveal significant cardiac impairment in patients with low VDL. Available data do not support the direct effects of this deficiency on cardiac systolic functions. These findings highlight the importance of monitoring vitamin D status and controlling iron load in Thalassemia major patients.

Keywords

vitamin D, thalassemia major, heart failure

Ethical Statement

ADANA ŞEHİR EĞİTİM VE ARAŞTIRMA HASTANES ETİK KURULUNDA GÖRÜŞÜLDÜ VE ÇALIŞMA KABUL EDİLDİ.

Project Number

2639/08.06.2023

References

• 1. Pala M, Bhat KG, Manya S, Joseph N, Harish S. Vitamin D levels and left ventricular function in beta-thalassemia ma-jor with iron overload. Eur J Pediatr. 2023;182(4):1749-1754..
• 2. Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis. 2010 May 21;5:11.
• 3. Weatherall D J. Phenotype—genotype relationships in mo-nogenic disease: lessons from the thalassaemias. Nat Rev Genet. 2001; 2(4): 245-255.
• 4. Gulbis B, Ferster A, Vertongen F, Fabiola DER, Bruxelles RDL. Hemoglobinopathies in Belgium. Belgian Journal of Hematology. 2010; 1(2):50-56
• 5. Kattamis A, Forni G L, Aydinok Y, Viprakasit V. Changing patterns in the epidemiology of β‐thalassemia. Eur J Hae-ma. 2020; 105(6): 692-703.
• 6. Borgna-Pignatti C, Marsella M. Iron chelation in thalasse-mia major. Clinical therapeutics. 2015; 37(12): 2866-2877.
• 7. Betts M, Flight PA, Paramore LC, Tian L, Milenković D, Sheth S. Systematic Literature Review of the Burden of Disease and Treatment for Transfusion-dependent β-Thalassemia. Clin Ther. 2020;42(2):322-337.e2..
• 8. Shiae Ali, E Bakhshali, M A ShojaRazavi, S J Poorzand, H Layegh P. Cardiac MR images of thalassemia major pati-ents with myocardial iron overload: a data note. BMC Rese-arch Notes.2021; 14:1-3.
• 9. Tziomalos K, Perifanis V. Liver iron content determination by magnetic resonance imaging. World Journal of Gastro-enterology. 2010;16(13): 1587.
• 10. Pepe A, Meloni A, Rossi G, Midiri M, Missere M, Valeri G, Wood J C. Prediction of cardiac complications for thalasse-mia major in the widespread cardiac magnetic resonance era: a prospective multicentre study by a multi-parametric approach. European Heart Journal-Cardiovascular Imaging 2018; 19(3): 299-309.
• 11. Dejkhamron P, Wejaphikul K, Mahatumarat T, Silvilairat S, Charoenkwan P, Saekho S, Unachak K. Vitamin D deficiency and its relationship with cardiac iron and function in pati-ents with transfusion-dependent thalassemia at Chiang Mai University Hospital. Pediatric Hematology and Oncology. 2018;35(1):52-59.
• 12. Wood C. Cardiac complications in thalassemia ma-jor. Hemoglobin. 2009; 33(1): 81-86.
• 13. Ambarwati L, Rahayuningsih S E, Setiabudiawan B. Associa-tion between vitamin D levels and left ventricular function and NT-proBNP levels among thalassemia major children with iron overload. Annals of Pediatric Cardiology. 2016; 9(2):126.
• 14. Ergün T, Cansever M. Comparison of 25-OH vitamin D levels between children with upper and those with lower extre-mity fractures: A prospective case-control study. Acta Ort-hopaedica et Traumatologica Turcica.2022; 56(2):76.
• 15. Płudowski P, Kos-Kudła B, Walczak M, Fal A, Zozulińska-Ziółkiewicz D, Sieroszewski P, Misiorowski W. Guidelines for preventing and treating vitamin D deficiency: a 2023 up-date in Poland. Nutrients.2023; 15(3): 695.
• 16. Yeşiltepe-Mutlu G, Aksu E D, Bereket A, Hatun Ş. Vitamin D status across age groups in Turkey: Results of 108,742 samples from a single laboratory. Journal of clinical rese-arch in pediatric endocrinology.2020; 12(3): 248.
• 17. Zakaria N A, Bahar R, Abdullah W Z, Mohamed Yusoff A A, Shamsuddin S, Abdul Wahab R, Johan M F. Genetic Mani-pulation Strategies for β-Thalassemia: A Review. Frontiers in Pediatrics.2022; 920.
• 18. Mishra A K, Tiwari A. Iron overload in beta thalassaemia major and intermedia patients. Maedica. 2013; 8(4): 328.
• 19. Matta B N, Musalla K M, Maakaron J E, Koussa S, Taher A T. A killer revealed: 10-year experience with beta-thalassemia intermedia. Hematology, 2014;19(4):196-198.
• 20. Meloni A, Pistoia L, Gamberini M R, Cuccia L, Lisi R, Cecinati V, Cademartiri F. Multi-Parametric Cardiac Magnetic Reso-nance for Prediction of Heart Failure Death in Thalassemia Major. Diagnostics. 2023; 13(5): 890.
• 21. Yu U, Chen L, Wang X, Zhang X, Li Y, Wen F, Liu S. Evalua-tion of the vitamin D and biomedical statuses of young childrenwith β-thalassemia major at a single center in so-uthern China. BMC pediatrics. 2019; 19(1): 1-8.
• 22. Gaudio A, Morabito N, Catalano A, Rapisarda R, Xourafa A, Lasco, A. Pathogenesis of thalassemia major-associated os-teoporosis: a review with insights from clinical experien-ce. Journal of clinical research in Pediatric Endocrinology. 2019;11(2):110.
• 23. Santra S, Sharma K, Dash I, Mondal S, Mondal H, Santra Sr S. Bone mineral density, serum calcium, and vitamin D le-vels in adult thalassemia major patients: experience from a single center in eastern India. Cureus.2022; 14(7):e26688.
• 24. Pishgahi M, Niroomand M, Bozorgmehr R, Ghane-Fard S, Mousavi F, Tabary M, Khaheshi I. Association between Se-rum Vitamin D Level and Echocardiographic Abnormalities in Patients with Thalassemia Ma-jor. Cardiovascular&Haematological Disorders-Drug Targets (Formerly Current Drug Targets-Cardiovascular&Hematological Disorders).2020; 20(3): 214-220.
• 25. Kıdır V. D vitamininin kardiyovasküler ve metabolik etkileri. Journal of Clinical and Experimental Investigations. 2013;4(3), 398-404.