OPG and RANKL in patients with thalassemia major in the center of hemoglobinopathy Lushnja, Albania

Zoga, Jorida; Refatllari, Etleva; Zaka, Ariana; Allkanjari, Adelina; Zeneli, D.; Kuqi, I.; Lala, Eliane Raquel Peres; Hoxhallari, Erinda; Mema, A.

doi:10.18638/arsa.2016.5.1.825

Cited by 2 publications

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“…32 Serum Osteocalcin level was low in our study, while in study done by Zoga J etal, they found no significant change in the osteocalcin between patients and control. 33 In another study done by Ozturk O etal, osteocalcin and IL-6 were not changed with increasing number of blood transfusion, and this finding is comparable with our result. 34…”

Section: Discussionsupporting

confidence: 91%

Biochemical Status of Beta-Thalassemia Major Patients in Erbil City

Surchi

Ali

2018

EDJ

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Background and objectives: β-thalassemia major patient is one of the hereditary hemolytic diseases, which can cause many hematological and biochemical changes in the affected patient. And these changes can happen even when the patient is treated adequately. The objective was to study biochemical changes in the level of serum hepcidin, osteocalcin, calcium, ferritin, iron, PTH and IL-6 in patients with β-thalassemia major and to compare it with control subjects. Patients and method: In this research 40 patients with beta thalassemia major, 20 Female and 20 Male (age ranged from 10 to 38 years), and 40 control subjects 20 Female and 20 Male (age ranged from 9 to 33 years) were studied. Measurement of serum hepcidin, osteocalcin, calcium, ferritin, iron, PTH and IL-6 were done by the researcher for both cases and control groups. Results: Serum Ferritin and Iron were higher significantly in all thalassemic patients (P< 0.001), this increment was proportional with increasing number of units of blood transfusion and aging. Mean serum Hepcidin, PTH, Osteocalcin and IL6 were significantly lower in thalassemic patients in contrast to the control subjects (P< 0.001). Reduction in S-PTH was proportional to increasing number of blood transfusion and aging. But there was no significant difference in the level of serum Calcium in the majority of patients, only 9 patients had low serum calcium. Conclusion: Our study demonstrates that in B-thalassemia major patients Serum Ferritin and Iron were increased proportionally with increasing age and number of units of blood transfusion. Mean serum PTH, osteocalcin, hepcidin, and IL-6 were reduced, but mean serum calcium was remained normal.

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Section: Discussionsupporting

confidence: 91%

Biochemical Status of Beta-Thalassemia Major Patients in Erbil City

Surchi

Ali

2018

EDJ

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“…In this study, the OPG level was significantly lower in patients than control group, while s‐RANKL level in patients was lower than control group but without statistical significance. Previous studies detected high s‐RANKL level in thalassemic patients, but without statistical significance 5,24 . Other studies reported s‐RANKL levels varying within normal to significant higher levels in thalassemia patients than the healthy controls, but the previous studies were conducted in adult age groups 25–27 .…”

Section: Discussionmentioning

confidence: 97%

“…Previous studies detected high s-RANKL level in thalassemic patients, but without statistical significance. 5,24 Other studies reported s-RANKL levels varying within normal to significant higher levels in thalassemia patients than the healthy controls, but the previous studies were conducted in adult age groups. [25][26][27] Younger thalassemic patients have increased concentrations of vitamin D, 5 also the regular intake of the active form of vitamin D as in our patient's case can affect the differentiation of mesenchymal progenitors into osteoblastic cells, which result in the decrease of RANKL activity in osteoblastic cells and the number of RANKLexpressing osteoblasts.…”

Section: Discussionmentioning

confidence: 99%

Bone health in pediatric transfusion‐dependent beta‐thalassemia: Circulating osteoprotegerin and RANKL system

Youssry

Saad

Madboly

et al. 2021

Pediatric Blood & Cancer

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Introduction While the mechanism of bone disease in thalassemia is multifactorial and still under investigation, the receptor activator of nuclear factor kappa B (RANK), receptor activator of nuclear factor kappa B ligand (RANKL), and osteoprotegerin (OPG) have pivotal roles in regulating bone metabolism. This study aimed to measure RANKL and OPG serum levels, and to detect the incidence of RANKL rs9533156, OPG rs2073618, and OPG rs2073617 genotypes in pediatric β‐thalassemia patients and to assess their relation to bone mineral density. Methods Sixty patients with transfusion‐dependent β‐thalassemia (TBT) patients ages 5 to 14 years were included, and 60 healthy, age‐ and sex‐matched volunteers contributed as a control group. The patients were scanned for bone mineral density. Results The mean of spine dual‐energy X‐ray absorptiometry (DXA) Z‐score in patients was −1.66 ± 1.02 standard deviation (SD). Twenty‐four of them had low spine DXA Z‐scores. The patients showed significantly lower OPG levels and OPG/RANKLs ratios than the control group (3.28 ± 9.11 ng/ml and 11.38 ± 14.93 ng/ml, and 0.01 ± 0.03 and 0.07 ± 0.09, respectively). The RANKL SNP rs9533156 TC heterozygous genotype was detected more with statistical significance in patients than controls. The incidence of OPG rs2073618 and OPG rs2073617 genotypes were 2.3 times and 1.9 times more frequent in patients than controls, respectively. Conclusion The RANK/RANKL/OPG system may have an important role in regulating bone metabolism in TBT patients, although further studies are needed to clarify its role.

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OPG and RANKL in patients with thalassemia major in the center of hemoglobinopathy Lushnja, Albania

Cited by 2 publications

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Biochemical Status of Beta-Thalassemia Major Patients in Erbil City

Biochemical Status of Beta-Thalassemia Major Patients in Erbil City

Bone health in pediatric transfusion‐dependent beta‐thalassemia: Circulating osteoprotegerin and RANKL system

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