Applicability of a clinical scoring criteria for disease severity of ß-thalassemia/hemoglobin E in Indonesia

Wahidiyat, Pustika Amalia; Sastroasmoro, Sudigdo; Fucharoen, Suthat; Setianingsih, Iswari; Putriasih, Siti Ayu

doi:10.13181/mji.v27i1.1779

Cited by 9 publications

(7 citation statements)

References 11 publications

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“…This essential micronutrient plays a role in regulating bone growth and development [ 76 ]. It has a positive effect on the proliferation and function of osteoblasts [ 14 , 77 ].…”

Section: Discussionmentioning

confidence: 99%

“…Children with normal BMD have higher serum Cu and Zn levels than those with low BMD and the incidence of normal BMD increases as the serum Cu and Zn increases [ 78 ]. Studies have suggested that the activation of lysyl oxidase, a Cu-containing oxygenase, induces the formation of collagen and elastin crosslinking [ 76 ]. What is more, decreased BMD is associated with many primary and secondary pediatric diseases [ 79 ].…”

Section: Discussionmentioning

confidence: 99%

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Copper/Zinc Ratio in Childhood and Adolescence: A Review

et al. 2023

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Both copper (Cu) and zinc (Zn) are crucial micronutrients for human growth and development. This literature review covered the last five years of available evidence on the Cu/Zn ratio in children and adolescents. We searched PubMed, Web of Science, Google Scholar, Cochrane Library, and Science Direct for publications between 2017 and 2022, especially in English, although publications in other languages with abstracts in English were included. The main terms used were “copper”, “zinc”, “copper-zinc”, and “zinc-copper” ratios. Cu and Zn determinations made in blood, plasma, or serum were included. This review comprises several cross-sectional and case–control studies with substantial results. The bibliographic search generated a compilation of 19 articles, in which 63.2% of the studies mostly reported a significantly higher Cu/Zn ratio, and 57.9% of them informed significantly lower levels of Zn. We conclude that children and adolescents with acute and chronic conditions are at greater risk of developing elevated Cu/Zn ratios, related to altered nutritional, infectious, and inflammatory status.

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“…This essential micronutrient plays a role in regulating bone growth and development [ 76 ]. It has a positive effect on the proliferation and function of osteoblasts [ 14 , 77 ].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Copper/Zinc Ratio in Childhood and Adolescence: A Review

et al. 2023

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“…Beta-thalassemia major (β-TM) is a hematologic disorder caused by absent or severely reduced synthesis of the β-globin chain in the hemoglobin A molecule resulting in damage to the erythrocyte membrane and subsequent anemia 1,2 . Patients with β-TM require lifelong blood transfusions to increase hemoglobin levels and minimize the detrimental effects of inefficient erythropoiesis 3 .…”

Section: Introductionmentioning

confidence: 99%

In Transfusion-dependent Thalassemia, Increased Iron Overload is Associated with Lower Serum Alpha-klotho, Which is Strongly Associated with Lower Total and Ionized Calcium Concentrations

Maes¹,

Moustafa²,

Al‐Hakeim³

et al. 2020

Preprint

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Patients with transfusion-dependent thalassemia (TDT) show disorders in calcium metabolism. The α-klotho protein is predominantly expressed in tissues that are involved in calcium homeostasis, and lowered levels are associated with bone disease. Aim of the study. To study the associations between low α-klotho status and calcium metabolism in relation to iron status in children with TDT. Methods. α-klotho, calcium, parathyroid hormone (PTH), calcyphosin, vitamin D3, phosphorous, fibroblast growth factor receptor 2 (FGFR2), as well as iron and erythron biomarkers were measured in 60 children with TDT and 30 healthy control children. Results. A meaningful part of TDT patients showed lowered α-klotho levels, and those children also showed low serum total and ionized calcium concentrations. TDT patients showed increased PTH, FGFR2, and calcyphosin and lowered vitamin D3 as compared with healthy children. The α-klotho levels were significantly correlated with total and ionized calcium (positively) and with iron overload biomarkers and the number of blood transfusions (inversely). Partial Least Squares path analysis showed that 40.1% of the variance in serum total calcium could be explained by the regression on α-klotho, vitamin D3 (both positively), and calcyphosin (inversely) and that the effects of the latter are mediated by iron overload and the number of blood transfusions. Conclusion. In TDT, iron overload and its consequences may induce lowered levels of α-klotho which in turn may lead to lower calcium thereby explaining at least in part the effects of TDT on bone metabolism including spontaneous pathological fractures, osteoporosis, osteopenia, and skeletal deformities.

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“…Beta-thalassemia major (β-TM) is a genetic disorder caused by mutations in the β-globin gene resulting in a lack or reduction of the β-globin chain synthesis leading to the globin chain imbalances (1). This imbalance is characterized by an excess of free α-globin chains in maturing red blood cells (RBCs) that lead to hemolysis with consequent anemia (2). Beta-TM patients require frequent blood transfusions to maintain normal hemoglobin levels and to suppress ineffective erythropoiesis (3).…”

Section: Introductionmentioning

confidence: 99%

Increased Zinc and Albumin but Lowered Copper in Children with Transfusion-dependent Thalassemia

Alhillawi

Al‐Hakeim

Moustafa

et al. 2020

Preprint

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Measurements of copper and zinc in transfusion-dependent thalassemia (TDT) show contradictory results.Aim of the study: To examine serum levels of these minerals in TDT in relation to iron overload indices and erythron variables. Methods: This study recruited 60 children with TDT and 30 healthy children aged 3-12 years old.Results: Zinc was significantly higher in TDT children than in control children, whilst copper and the copper to zinc ratio were significantly lowered in TDT. Serum zinc was significantly associated with the number of blood transfusions and iron overload variables (including serum iron and TS%) and negatively with erythron variables (including hemoglobin, mean corpuscular volume, mean corpuscular hemoglobin). Serum copper was significantly and negatively associated with the same iron overload and erythron variables. The copper to zinc ratio was significantly correlated with iron, TS%, ferritin, hemoglobin, mean corpuscular volume, and mean corpuscular hemoglobin. Albumin levels were significantly higher in TDT children than in control children. Conclusion: Our results suggest that the increase in zinc in children with TDT may be explained by iron loading anemia and hemolysis and the consequent shedding of high amounts of intracellular zinc into the plasma. Increased albumin levels and treatment with Desferral may further contribute towards higher zinc levels in TDT. We suggest that the elevations in zinc in TDT are a compensatory mechanism protecting against infection, inflammation, and oxidative stress. Previous proposals for prophylactic use of zinc supplements in TDT may not be warranted.

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Applicability of a clinical scoring criteria for disease severity of ß-thalassemia/hemoglobin E in Indonesia

Cited by 9 publications

References 11 publications

Copper/Zinc Ratio in Childhood and Adolescence: A Review

Copper/Zinc Ratio in Childhood and Adolescence: A Review

In Transfusion-dependent Thalassemia, Increased Iron Overload is Associated with Lower Serum Alpha-klotho, Which is Strongly Associated with Lower Total and Ionized Calcium Concentrations

Increased Zinc and Albumin but Lowered Copper in Children with Transfusion-dependent Thalassemia

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