• Natural Product Sciences
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• 제27권2호
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• pp.78-85
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• 2021

Through bio-guided isolation, two natural iron chelators were isolated from Mangifera indica L. leaves, identified as mangiferin (1) and iriflophenone-3-C-𝛽-D-glucoside (2). Their iron-chelating activity was compared to that of Desferal^® using bipyridyl assay and EDTA as a standard. Mangiferin showed the highest activity with IC₅₀ value of 0.385 mM (162.85 ㎍/mL). Furthermore, two combinations of mangiferin with Desferal^® (M-D) and iriflophenone-3-C-𝛽-D-glucoside (M-I) were evaluated. The results showed that mangiferin potentiated the iron chelation activity of Desferal^® about 46%, also that M-I combination is a promising candidate formula for iron chelation therapy. In addition, mangiferin and Desferal-iron complexes were prepared and characterized by IR, UV, and Mass spectra to compare their mode of chelation to iron. Their structural stability was studied by DFT calculations. Furthermore, they displayed increased ABTS antioxidant activity when bound to iron as compared to their free form, which enhances their pharmacological importance.

• Toxicological Research
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• 제33권4호
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• pp.299-304
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• 2017

Thallium and its compounds are a class of highly toxic chemicals that cause wide-ranging symptoms such as gastrointestinal disturbances; polyneuritis; encephalopathy; tachycardia; skin eruptions; hepatic, renal, cardiac, and neurological toxicities; and have mutagenic and genotoxic effects. The present research aimed to evaluate the efficacy of the chelating agents deferasirox (DFX) and deferiprone (L1) in reducing serum and tissue thallium levels after the administration of thallium (III), according to two different dosing regimens, to several groups of Wistar rats for 60 days. It was hypothesized that the two chelators might be more efficient as a combined therapy than as monotherapies in removing thallium (III) from the rats' organs. The chelators were administered orally as either single or combined therapies for a period of 14 days. Serum and tissue thallium (III) and iron concentrations were determined by flame atomic absorption spectroscopy. Serum and tissue thallium (III) levels were significantly reduced by combined therapy with DFX and L1. Additionally, iron concentrations returned to normal levels and symptoms of toxicity decreased.

• 대한한방내과학회지
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• 제44권2호
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• pp.252-259
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• 2023

Objectives: This study identified the effect of Korean medicine treatment on a patient with iron overload who discontinued iron chelation therapy. Methods: A 64-year-old woman with iron overload was treated with Saenggangunbi-tang from November 14, 2022, to March 15, 2023, to reduce fatigue and improve laboratory findings. We observed changes in the symptoms and improvement of laboratory findings during the four-month treatment. Results: The approximately four-month treatment with Saenggangunbi-tang showed considerable improvement in laboratory findings and fatigue. In addition, no adverse effects, such as liver injury, were observed during Korean medicine treatment. Conclusion: This study suggests the availability of Saenggangunbi-tang as a therapeutic option for managing patients with iron overload.

• 대한임상검사과학회지
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• 제49권4호
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• pp.495-497
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• 2017

Thalassemia major is a genetic disorder with a defective synthesis of either the alpha or the beta chain of hemoglobin A. Blood transfusion is crucial for the survival in these patients. Unfortunately, endocrine dysfunction is a very common complication in these patients and is principally due to excessive iron overload as a result of frequent blood transfusions. Although regular blood transfusion may increase life expectancy, disturbances in growth and pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes, and disorderly bone growth are common side effects. We hereby present a case of a 23-year-old, unmarried woman with beta thalassemia major presenting with primary amenorrhea, poor development of secondary sexual character, and short stature. Thorough history, clinical examination, and laboratory investigation, including dynamic function test (insulin tolerance test) were conducted. These tests confirmed that she had multiple endocrinopathies, including hypogonadotropic hypogonadism, growth hormone deficiency, and subclinical adrenal insufficiency, which were caused by iron overload. She required hormone replacement therapy. Early recognition of possible deficiencies in hypothalamo-pituitary-end organ hormones caused by iron overload in thalassemia patients that undergo frequent blood transfusion procedures is essential. Appropriate treatments, including transfusion regimen and chelation therapy, as well as specific treatment of each complication are the crucial for the successful management and improvement of quality of life these patients.

• Clinical and Experimental Pediatrics
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• 제50권12호
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• pp.1225-1230
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• 2007

목 적 : 만성 빈혈로 대량 수혈을 받아야 하는 환아들에서는 조직과 장기에 철이 축적될 수 있다. 이러한 환아들에서 효과적인 철킬레이트 치료의 목적은 충분한 양의 철을 제거하여 체내에서 철로 인한 장기 손상이 나타나지 않도록 하는데 있다. 본 연구는 후향적으로 대량 수혈을 받은 환아들에서 정맥 투여한 deferoxamine의 효과를 알아보고자 하였다. 방 법 : 2005년 3월부터 2007년 1월까지 15명의 대량 수혈을 받았던 환아들을 대상으로 하였으며 이들 중 수혈 의존성 환아들은 한 달에 1단위 이상의 농축 적혈구 수혈을 최근 6개월 이상 계속 받고 있는 환아들로 정의하였다. 7일 동안 deferoxamine을 10-30 mg/kg/day로 24시간 지속 정맥주입하였으며 투여 전, 후 그리고 3개월 후의 혈청 철, 총철결합능, ferritin을 수혈 의존성 환아들과 수혈 비의존성 환아들로 나누어 비교하였다. 결 과 : 6명의 남아와 9명의 여아가 있었으며 이들의 나이는 5.6-21.3(중앙값 8.3)세였고 수혈 의존성 환아들은 7명, 수혈 비의존성 환아들은 8명이었다. 수혈 의존성 환아들의 ferritin은 deferoxamine 투여 전과 후, 3개월 후에 의미 있는 차이를 보이지 않았으나 수혈 비의존성 환아들에서는 투여 전과 비교 시 투여 3개월 후에는 의미 있는 감소를 보였다(P=0.046). Deferoxamine 정맥주입과 연관된 이상 반응은 경미하였으며 1-2일 내에 소실되었다. 결 론 : 7일 동안 정맥주입하는 deferoxamine은 수혈 비의존성 환아들에서 단기간의 효과적인 치료로 사용될 수 있을 것으로 생각되지만 수혈 의존성 환아들의 경우에는 철의 축적을 막기 위해 유지 요법이 필요할 것으로 생각된다.

• BLOOD RESEARCH
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• 제53권4호
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• pp.314-319
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• 2018

Background Iron overload is a risk factor affecting all patients with thalassemia intermedia (TI). We aimed to determine whether there is a relationship of serum ferritin (SF) and alanine aminotransferase (ALT) with liver iron concentration (LIC) determined by R2 magnetic resonance imaging (R2-MRI), to estimate the most relevant degree of iron overload and best time to chelate in patients with TI. Methods In this cross-sectional study, 119 patients with TI (mean age years) were randomly selected and compared with 120 patients who had a diagnosis of thalassemia major (TM). Correlations of LIC, as determined by R2-MRI, with SF and ALT levels, were assessed in all participants. A P-value <0.05 was considered statistically significant. Results SF and LIC levels were lower in patients with TI than in those with TM; only ferritin values were significant. We found a statistically significant relationship between SF and LIC, with cut-off estimates of SF in patients with TI who had splenectomy and those who entered puberty spontaneously (916 and 940 ng/mL, respectively) with LIC >5 mg Fe/g dry weight (P<0.0001). A significant relationship was also found for patients with TI who had elevated ALT level (63.5 U/L), of 3.15 times the upper normal laboratory limit, using a cut-off for LIC ${\geq}5mg\;Fe/g\;dry\;weight$. Conclusion We determined the cut-off values for ALT and SF indicating the best time to start iron chelation therapy in patients with TI, and found significant correlations among iron overload, SF, and ALT.