Prevalence and Risk Factors for Cardiac and Liver Iron Overload in Adults with Thalassemia in Malaysia

Ngim, Chin Fang; Lee, Mei Y.; Othman, Norliza; Lim, Soo Min; Ng, Chen Siew; Ramadas, Amutha

doi:10.1080/03630269.2019.1599906

Cited by 8 publications

(13 citation statements)

References 27 publications

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“…The level of SF has been found to be strongly correlated with LIC in several studies 17–19 . In the current study, most of the patients having a reduction in SF from their baseline levels also saw a decrease in their LICs, especially in the case of those whose SF reduction was greater than 50%.…”

Section: Discussionsupporting

confidence: 60%

“…The level of SF has been found to be strongly correlated with LIC in several studies. [17][18][19] In the current study, most of the patients having a reduction in SF from their baseline levels also saw a decrease in their LICs, especially in the case of those whose SF reduction was greater than 50%. Therefore, favorable outcomes can be predicted for TDT patients who solely have liver siderosis and are treated with the DFO-DFX combination if they evidence a substantial decrease in their SF levels.…”

Section: Discussionsupporting

confidence: 48%

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Using of deferasirox and deferoxamine in refractory iron overload thalassemia

Takpradit

Viprakasit

Narkbunnam

et al. 2021

Pediatrics International

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Background: Iron overload is a major complication of transfusion-dependent thalassemia (TDT) and requires iron chelation (IC) therapy. However, a combination therapy may be required for patients responding poorly to monotherapy. Methods: Nine TDT patients previously treated with IC were enrolled; five patients were previously treated with deferasirox (DFX) twice daily. The dose of DFX was 20-40 mg/kg/day, while the dose of deferoxamine (DFO) was 18-40 mg/kg/day for 3-6 days/week. Results: At the 6-and 12-month time points, six and eight patients demonstrated decreased serum ferritin levels, with median reductions of 707 ng/mL (range, 1,653-5,444 ng/mL) and 1,129 ng/mL (range, 1,781-7,725 ng/mL) compared to the baseline, respectively. Eight patients also had a reduced liver iron concentration (LIC), with a median reduction of 3.9 mg/g dry wt (range, 8.3-11.1 mg/g dry wt). Of the five patients treated with DFX twice daily, four responded to combination therapy. All responsive patients could finally stop DFO after the decline in LIC. Moreover, there were no treatment-related complications. Conclusion:The combination of DFX and DFO proved to be effective and without significant toxicities for TDT patients who had been unresponsive to standard IC therapy. Further studies with a larger cohort size and long-term follow-up are warranted to elucidate the efficacy of the combination.

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Section: Discussionsupporting

confidence: 60%

Section: Discussionsupporting

confidence: 48%

Using of deferasirox and deferoxamine in refractory iron overload thalassemia

Takpradit

Viprakasit

Narkbunnam

et al. 2021

Pediatrics International

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“…Fe plays an essential role in catalytic processes involving enzymes such as peroxidase, cytochromes, and catalase [1]. Maintenance of a healthy state requires sufficient stores of this microelement, yet Fe excess is a factor leading to such detrimental states as intensified oxidative stress [2], inflammation [3], liver [4] and cardiac [5] dysfunction, kidney injury [6], neurodegeneration [2], adipose tissue dysfunction [7], glucose metabolism disorders [8], and disrupted gene expression [9]. On the other hand, Fe deficiency leads to anemia [10].…”

Section: Introductionmentioning

confidence: 99%

Hepcidin and Erythroferrone Correlate with Hepatic Iron Transporters in Rats Supplemented with Multispecies Probiotics

et al. 2020

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The influence of probiotic supplementation on iron metabolism remains poorly investigated. However, a range of studies, especially on Lactobacillus plantarum 299v (Lp229v), have indicated a possible positive impact of probiotics on iron absorption. The aim of the study was to determine the effect of multistrain probiotic supply on iron balance. Thirty Wistar rats were randomized into three groups: placebo (KK group), and multistrain probiotic per os in a daily dose of 2.5 × 109 colony forming units (CFU) (PA group) or 1 × 1010 CFU (PB group). Multistrain probiotic consisted of nine bacterial strains: Bifidobacterium bifidum W23, B. lactis W51, B. lactis W52, Lactobacillus acidophilus W37, L. brevis W63, L. casei W56, L. salivarius W24, Lactococcus lactis W19, and Lc. lactis W58, in equal proportions. After six weeks, blood and organ samples were collected. No differences were found between the three groups in terms of serum concentrations of hepcidin (HEPC), lactoferrin (LTF), homocysteine (HCY), ferritin (Ft), or erythroferrone (ErFe), or in liver content of divalent metal transporter 1 (DMT1), transferrin receptors 1 and 2 (TfR), or ZRT/IRT-like protein 14 (ZIP14) proteins. In the overall sample, positive correlations were noted between the serum concentrations of hepcidin and lactoferrin, and hepcidin and ferritin; serum concentration of hepcidin and DMT1 and TfR1 in the liver; and serum concentration of erythroferrone and TfR2 in the liver. The correlations of serum hepcidin and erythroferrone with liver DMT1 and TfR represent significant mechanisms of Fe homeostasis. Our study has shown that multistrain probiotic supplementation used in the experiment did not disrupt the biochemical and hepatic regulatory processes of Fe balance and did not demonstrate significant influence on selected parameters of Fe metabolism.

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“…Nevertheless, the accuracy of these tests is often negatively affected by inflammatory states and renal and liver conditions, which are more common in the context of HIV-1 infection ( 11 ). However, studies have shown that these circulating biomarkers are poor indicators of myocardial iron levels ( 12 , 13 ).…”

Section: Introductionmentioning

confidence: 99%

Association of myocardial iron deficiency based on T2* CMR with the risk of mild left ventricular dysfunction in HIV-1-infected patients

Yan

Zhang

et al. 2023

Front. Cardiovasc. Med.

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ObjectivesThis study sought to noninvasively determine myocardial iron levels in HIV-1-infected patients using CMR and explore the association between T2* values and mild left ventricular systolic dysfunction (LVSD).MethodsThis prospective study was conducted from June 2019 to July 2021. HIV-1-infected adults and healthy controls were consecutively enrolled for CMR exam. CMR exam included the assessment of myocardium iron content (T2*), cardiac function (cine), inflammation (T2), and fibrosis (through extracellular volume fraction [ECV] and late gadolinium enhancement [LGE]) measurements. Mild LVSD is defined as a left ventricular ejection fraction (LVEF) between 40% and 49%.ResultsOf 47 HIV-1-infected patients enrolled, 12 were diagnosed with mild LVSD (HIV-1+/LEVF+) and 35 were diagnosed with preserved LV function (HIV-1+/LEVF−). Compared with healthy controls, HIV-1-infected patients displayed higher T2*, T1, T2, ECV values and lower global circumferential strain (GCS) and global radial strain (GRS) (all P < 0.05). However, between patients with and without mild LVSD, only the T2* values and ECV (all P <0.05) were different. The association between increased T2* values (>26 ms) and mild LVSD remained significant after adjusting for the established univariate predictors (ECV >32.9%, T1 values >1336 ms) of mild LVSD (odds ratio [OR], 10.153; 95% confidence interval [CI] 1.565–65.878, P = 0.015).ConclusionsMyocardial T2* values were elevated in HIV-1-infected patients, supporting the notion that ID was associated with mild LVSD. Our findings highlight the potential for ID in HIV-1-infected patients as an auxiliary biomarker to monitor the course of LVSD.

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Prevalence and Risk Factors for Cardiac and Liver Iron Overload in Adults with Thalassemia in Malaysia

Cited by 8 publications

References 27 publications

Using of deferasirox and deferoxamine in refractory iron overload thalassemia

Using of deferasirox and deferoxamine in refractory iron overload thalassemia

Hepcidin and Erythroferrone Correlate with Hepatic Iron Transporters in Rats Supplemented with Multispecies Probiotics

Association of myocardial iron deficiency based on T2* CMR with the risk of mild left ventricular dysfunction in HIV-1-infected patients

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