Correlation of OPG/RANKL in patients with thalassemia major at the center of Haemoglobinopathy Lushnje, Albania
Keywords:
Thalassemia, osteoporosis, OPG/RANKL
Abstract
Osteoporosis is an important cause of morbidity in hemoglobinopathy patients. It is characterized by low bone mass and disruption of bone architecture, resulting in reduced bone strength and increased risk of fractures. Osteoprotegerin (OPG) and receptor activator of NF-kappa-B ligand (RANKL) have recently been implicated in the pathogenesis of various types of osteoporosis. Our study aimed to determine the correlation between OPG/RANKL and patients affected by thalassemia major at the Center of Hemoglobinopathy in Lushnje. Methods: We measured serum OPG and RANKL levels in 70 patients with Thalassemia major and 67 healthy controls, determining correlations with BMD. Results: Serum OPG levels were significantly lower in thalassemic patients compared to the control group. Serum RANKL levels were higher in β-thalassemia patients compared to controls. 31.1% of our patients with Thalassemia major had osteoporosis and 21.6 % had osteopenia. We found a correlation between OPG-BMD (r=-0.768, p=0.000, and RANKL-BMD (r=0.468; p=0.000). Conclusion: OPG and RANKL in Thalassemia major patients should be considered as the main factors responsible for osteoclast activation.
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References
1. Refatllari E, Bulo A. The prevalence of beta-thalassemia in students screening in Lushnja district, Albania. BCLF, Antalya 2008
2. AbdAllah, N., Galal, A., Hamed, H. M., Adolf, S., Ragab, S. H., Mawgoud, S. A. el, & Rasheed, M. A. (2010). The roles of osteoprotegerin and RANKL in the pathogenesis of osteoporosis in Egyptian beta-thalassemia major patients. Journal of Applied Sciences Research, 6(8).
3. Amer, O. E., Wani, K., Ansari, M. G. A., Alnaami, A. M., Aljohani, N., Abdi, S., Hussain, S. D., Al-Daghri, N. M., & Alokail, M. S. (2022). Associations of Bone Mineral Density with RANKL and Osteoprotegerin in Arab Postmenopausal Women: A Cross-Sectional Study. Medicina (Lithuania), 58(8). https://doi.org/10.3390/medicina58080976
4. Çelik, T., Sangün, Ö., Ünal, Ş., Balcı, A., & Motor, S. (2022). Assessment of biochemical bone markers of osteoporosis in children with thalassemia major. Italian Journal of Pediatrics, 48(1). https://doi.org/10.1186/s13052-022-01290-x
5. Chuang, M. H., Chuang, T. L., Koo, M., & Wang, Y. F. (2019). Low Hemoglobin Is Associated With Low Bone Mineral Density and High Risk of Bone Fracture in Male Adults: A Retrospective Medical Record Review Study. American Journal of Men’s Health, 13(3). https://doi.org/10.1177/1557988319850378
6. di Paola, A., Marrapodi, M. M., di Martino, M., Giliberti, G., di Feo, G., Rana, D., Ahmed, S., Argenziano, M., Rossi, F., & Roberti, D. (2024). Bone Health Impairment in Patients with Hemoglobinopathies: From Biological Bases to New Possible Therapeutic Strategies. In International Journal of Molecular Sciences (Vol. 25, Issue 5). Multidisciplinary Digital Publishing Institute (MDPI). https://doi.org/10.3390/ijms25052902
7. Farmakis, D., Porter, J., Taher, A., Cappellini, M. D., Angastiniotis, M., Eleftheriou, A., Alassaf, A., Angastiniotis, M., Angelucci, E., Aydinok, Y., Bou-Fakhredin Rayan, R., Brunetta, L., Cappellini, M. D., Constantinou, G., Daar, S., de Sanctis, V., Dusheiko, G., Elbard, R., Eleftheriou, A., … Yardumian, A. (2022). 2021 Thalassaemia International Federation Guidelines for the Management of Transfusion-dependent Thalassemia. HemaSphere, 6(8). https://doi.org/10.1097/HS9.0000000000000732
8. Gaudio, A., Morabito, N., Catalano, A., Rapisarda, R., Xourafa, A., & Lasco, A. (2018). Pathogenesis of Thalassemia Major–Associated Osteoporosis: Review of the Literature and Our Experience. Journal of Clinical Research in Pediatric Endocrinology. https://doi.org/10.4274/jcrpe.0074
9. Hamidpour, M., Jafari, F., Mehrpouri, M., Azarkyvan, A., Bashash, D., & Maboudi, A. A. K. (2022). Evaluation of the relationship between biochemical parameters and osteoporosis in patients with β-thalassemia major. Iranian Journal of Pediatric Hematology and Oncology, 12(1). https://doi.org/10.18502/ijpho.v12i1.8360
10. Jafari, F., Azarkeivan, A., Bashash, D., Maboodi, A. A. K., & Hamidpour, M. (2019). Correlation between serum levels of RANKL with osteoporosis in patients with beta-thalassemia major. Koomesh, 21(1).
11. Koohmanaee, S., Dabandi, B., Baghersalimi, A., Zare, R., Zoroufi, M. A., MirMonsef, S. G., Rad, A. H., Akhavan, A., Golshekan, K., Moamer, F., Medghalchi, A., & Dlili, S. (2021). The probability of indicating Osteoprotegerin as a biomarker for osteoporosis in patients with thalassemia major. Iranian Journal of Pediatric Hematology and Oncology, 11(1), 18–23. https://doi.org/10.18502/ijpho.v11i1.5002
12. Morabito, N., Gaudio, A., Lasco, A., Atteritano, M., Pizzoleo, M. A., Cincotta, M., la Rosa, M., Guarino, R., Meo, A., & Frisina, N. (2004). Osteoprotegerin and RANKL in the pathogenesis of thalassemia-induced osteoporosis: New pieces of the puzzle. Journal of Bone and Mineral Research, 19(5). https://doi.org/10.1359/JBMR.040113
13. Oğuz, S. H., Okay, M., Fedai, A. B., Ünal, Ş., Gümrük, F., Sayınalp, N., & Ünlütürk, U. (2023). Endocrine Disorders in Adult Beta-Thalassemia Patients: Insights from a Long-Term Follow-Up. Endocrinology Research and Practice, 27(4). https://doi.org/10.5152/erp.2023.23340
14. Pietrapertosa, A. C., Minenna, G., Colella, S. M., Santeramo, T. M., Renni, I. R., & D’Amore, M. (2009). Osteoprotegerin and RANKL in the pathogenesis of osteoporosis in patients with thalassemia major. Panminerva Medica, 51(1).
15. Schündeln, M. M., Goretzki, S. C., Hauffa, P. K., Wieland, R., Bauer, J., Baeder, L., Eggert, A., Hauffa, B. P., & Grasemann, C. (2014). Impairment of bone health in pediatric patients with hemolytic anemia. PLoS ONE, 9(10). https://doi.org/10.1371/journal.pone.0108400
16. Skordis, N., Efstathiou, E., Kyriakou, A., & Toumba, M. (2008). Hormonal dysregulation and bones in Thalassaemia - An overview. In Pediatric Endocrinology Reviews (Vol. 6, Issue SUPPL. 1).
17. Tari, K., Valizadeh Ardalan, P., Abbaszadehdibavar, M., Atashi, A., Jalili, A., & Gheidishahran, M. (2018). Thalassemia an update: molecular basis, clinical features and treatment. International Journal of Biomedicine and Public Health, 1(1). https://doi.org/10.22631/ijbmph.2018.56102
18. Tombak, A., Boztepe, B., Akbayir, S., Dogru, G., & Sungur, M. A. (2020). Receptor Activator of Nuclear Factor κ-Β Ligand/Osteoprotegerin Axis in Adults with Hb S/β-Thalassemia and β-Thalassemia Trait. Hemoglobin, 44(5). https://doi.org/10.1080/03630269.2020.1811116
19. Toumba, M., & Skordis, N. (2010). Osteoporosis Syndrome in Thalassaemia Major: An Overview. Journal of Osteoporosis, 2010, 1–7. https://doi.org/10.4061/2010/537673
20. Tsartsalis, A. N., Lambrou, G. I., Tsartsalis, D. N., Papassotiriou, I., Vlachou, E., Terpos, E., Chrousos, G. P., Kanaka-Gantenbein, C., & Kattamis, A. (2019). Bone Metabolism Markers in Thalassemia Major-Induced Osteoporosis: Results from a Cross-Sectional Observational Study. Current Molecular Medicine, 19(5). https://doi.org/10.2174/1566524019666190314114447
21. Youssry, I., Saad, N., Madboly, M., Samy, R. M., Hamed, S. T., Tawfik, H., Elbatrawy, S. R., Kaddah, N., & Abd Elaziz, D. (2022). Bone health in pediatric transfusion-dependent beta-thalassemia: Circulating osteoprotegerin and RANKL system. Pediatric Blood and Cancer, 69(1). https://doi.org/10.1002/pbc.29377
22. Yu, U., Chen, L., Wang, X., Zhang, X., Li, Y., Wen, F., & Liu, S. (2019). Evaluation of the vitamin D and biomedical statuses of young children with β-thalassemia major at a single center in southern China. BMC Pediatrics, 19(1), 375. https://doi.org/10.1186/s12887-019-1744-8
23. Rogers, A., & Eastell, R. (2005). Review: Circulating osteoprotegerin and receptor activator for nuclear factor κB ligand: Clinical utility in metabolic bone disease assessment. In Journal of Clinical Endocrinology and Metabolism (Vol. 90, Issue 11, pp. 6323–6331). https://doi.org/10.1210/jc.2005-0794
2. AbdAllah, N., Galal, A., Hamed, H. M., Adolf, S., Ragab, S. H., Mawgoud, S. A. el, & Rasheed, M. A. (2010). The roles of osteoprotegerin and RANKL in the pathogenesis of osteoporosis in Egyptian beta-thalassemia major patients. Journal of Applied Sciences Research, 6(8).
3. Amer, O. E., Wani, K., Ansari, M. G. A., Alnaami, A. M., Aljohani, N., Abdi, S., Hussain, S. D., Al-Daghri, N. M., & Alokail, M. S. (2022). Associations of Bone Mineral Density with RANKL and Osteoprotegerin in Arab Postmenopausal Women: A Cross-Sectional Study. Medicina (Lithuania), 58(8). https://doi.org/10.3390/medicina58080976
4. Çelik, T., Sangün, Ö., Ünal, Ş., Balcı, A., & Motor, S. (2022). Assessment of biochemical bone markers of osteoporosis in children with thalassemia major. Italian Journal of Pediatrics, 48(1). https://doi.org/10.1186/s13052-022-01290-x
5. Chuang, M. H., Chuang, T. L., Koo, M., & Wang, Y. F. (2019). Low Hemoglobin Is Associated With Low Bone Mineral Density and High Risk of Bone Fracture in Male Adults: A Retrospective Medical Record Review Study. American Journal of Men’s Health, 13(3). https://doi.org/10.1177/1557988319850378
6. di Paola, A., Marrapodi, M. M., di Martino, M., Giliberti, G., di Feo, G., Rana, D., Ahmed, S., Argenziano, M., Rossi, F., & Roberti, D. (2024). Bone Health Impairment in Patients with Hemoglobinopathies: From Biological Bases to New Possible Therapeutic Strategies. In International Journal of Molecular Sciences (Vol. 25, Issue 5). Multidisciplinary Digital Publishing Institute (MDPI). https://doi.org/10.3390/ijms25052902
7. Farmakis, D., Porter, J., Taher, A., Cappellini, M. D., Angastiniotis, M., Eleftheriou, A., Alassaf, A., Angastiniotis, M., Angelucci, E., Aydinok, Y., Bou-Fakhredin Rayan, R., Brunetta, L., Cappellini, M. D., Constantinou, G., Daar, S., de Sanctis, V., Dusheiko, G., Elbard, R., Eleftheriou, A., … Yardumian, A. (2022). 2021 Thalassaemia International Federation Guidelines for the Management of Transfusion-dependent Thalassemia. HemaSphere, 6(8). https://doi.org/10.1097/HS9.0000000000000732
8. Gaudio, A., Morabito, N., Catalano, A., Rapisarda, R., Xourafa, A., & Lasco, A. (2018). Pathogenesis of Thalassemia Major–Associated Osteoporosis: Review of the Literature and Our Experience. Journal of Clinical Research in Pediatric Endocrinology. https://doi.org/10.4274/jcrpe.0074
9. Hamidpour, M., Jafari, F., Mehrpouri, M., Azarkyvan, A., Bashash, D., & Maboudi, A. A. K. (2022). Evaluation of the relationship between biochemical parameters and osteoporosis in patients with β-thalassemia major. Iranian Journal of Pediatric Hematology and Oncology, 12(1). https://doi.org/10.18502/ijpho.v12i1.8360
10. Jafari, F., Azarkeivan, A., Bashash, D., Maboodi, A. A. K., & Hamidpour, M. (2019). Correlation between serum levels of RANKL with osteoporosis in patients with beta-thalassemia major. Koomesh, 21(1).
11. Koohmanaee, S., Dabandi, B., Baghersalimi, A., Zare, R., Zoroufi, M. A., MirMonsef, S. G., Rad, A. H., Akhavan, A., Golshekan, K., Moamer, F., Medghalchi, A., & Dlili, S. (2021). The probability of indicating Osteoprotegerin as a biomarker for osteoporosis in patients with thalassemia major. Iranian Journal of Pediatric Hematology and Oncology, 11(1), 18–23. https://doi.org/10.18502/ijpho.v11i1.5002
12. Morabito, N., Gaudio, A., Lasco, A., Atteritano, M., Pizzoleo, M. A., Cincotta, M., la Rosa, M., Guarino, R., Meo, A., & Frisina, N. (2004). Osteoprotegerin and RANKL in the pathogenesis of thalassemia-induced osteoporosis: New pieces of the puzzle. Journal of Bone and Mineral Research, 19(5). https://doi.org/10.1359/JBMR.040113
13. Oğuz, S. H., Okay, M., Fedai, A. B., Ünal, Ş., Gümrük, F., Sayınalp, N., & Ünlütürk, U. (2023). Endocrine Disorders in Adult Beta-Thalassemia Patients: Insights from a Long-Term Follow-Up. Endocrinology Research and Practice, 27(4). https://doi.org/10.5152/erp.2023.23340
14. Pietrapertosa, A. C., Minenna, G., Colella, S. M., Santeramo, T. M., Renni, I. R., & D’Amore, M. (2009). Osteoprotegerin and RANKL in the pathogenesis of osteoporosis in patients with thalassemia major. Panminerva Medica, 51(1).
15. Schündeln, M. M., Goretzki, S. C., Hauffa, P. K., Wieland, R., Bauer, J., Baeder, L., Eggert, A., Hauffa, B. P., & Grasemann, C. (2014). Impairment of bone health in pediatric patients with hemolytic anemia. PLoS ONE, 9(10). https://doi.org/10.1371/journal.pone.0108400
16. Skordis, N., Efstathiou, E., Kyriakou, A., & Toumba, M. (2008). Hormonal dysregulation and bones in Thalassaemia - An overview. In Pediatric Endocrinology Reviews (Vol. 6, Issue SUPPL. 1).
17. Tari, K., Valizadeh Ardalan, P., Abbaszadehdibavar, M., Atashi, A., Jalili, A., & Gheidishahran, M. (2018). Thalassemia an update: molecular basis, clinical features and treatment. International Journal of Biomedicine and Public Health, 1(1). https://doi.org/10.22631/ijbmph.2018.56102
18. Tombak, A., Boztepe, B., Akbayir, S., Dogru, G., & Sungur, M. A. (2020). Receptor Activator of Nuclear Factor κ-Β Ligand/Osteoprotegerin Axis in Adults with Hb S/β-Thalassemia and β-Thalassemia Trait. Hemoglobin, 44(5). https://doi.org/10.1080/03630269.2020.1811116
19. Toumba, M., & Skordis, N. (2010). Osteoporosis Syndrome in Thalassaemia Major: An Overview. Journal of Osteoporosis, 2010, 1–7. https://doi.org/10.4061/2010/537673
20. Tsartsalis, A. N., Lambrou, G. I., Tsartsalis, D. N., Papassotiriou, I., Vlachou, E., Terpos, E., Chrousos, G. P., Kanaka-Gantenbein, C., & Kattamis, A. (2019). Bone Metabolism Markers in Thalassemia Major-Induced Osteoporosis: Results from a Cross-Sectional Observational Study. Current Molecular Medicine, 19(5). https://doi.org/10.2174/1566524019666190314114447
21. Youssry, I., Saad, N., Madboly, M., Samy, R. M., Hamed, S. T., Tawfik, H., Elbatrawy, S. R., Kaddah, N., & Abd Elaziz, D. (2022). Bone health in pediatric transfusion-dependent beta-thalassemia: Circulating osteoprotegerin and RANKL system. Pediatric Blood and Cancer, 69(1). https://doi.org/10.1002/pbc.29377
22. Yu, U., Chen, L., Wang, X., Zhang, X., Li, Y., Wen, F., & Liu, S. (2019). Evaluation of the vitamin D and biomedical statuses of young children with β-thalassemia major at a single center in southern China. BMC Pediatrics, 19(1), 375. https://doi.org/10.1186/s12887-019-1744-8
23. Rogers, A., & Eastell, R. (2005). Review: Circulating osteoprotegerin and receptor activator for nuclear factor κB ligand: Clinical utility in metabolic bone disease assessment. In Journal of Clinical Endocrinology and Metabolism (Vol. 90, Issue 11, pp. 6323–6331). https://doi.org/10.1210/jc.2005-0794
Published
2024-06-30
How to Cite
Zoga, J., Kallco, M., & Refatllari, E. (2024). Correlation of OPG/RANKL in patients with thalassemia major at the center of Haemoglobinopathy Lushnje, Albania. European Scientific Journal, ESJ, 20(18), 13. https://doi.org/10.19044/esj.2024.v20n18p13
Section
ESJ Natural/Life/Medical Sciences
Copyright (c) 2024 Jorida Zoga, Majlinda Kallco, Etleva Refatllari
This work is licensed under a Creative Commons Attribution 4.0 International License.
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