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The predicting formula and scoring system for cardiac iron overload for thalassaemia children: Study from a
Syarif Rohimi1, Bambang Budi Siswanto2, Muchtaruddin Mansyur3
1Rumah Sakit Anak dan Bunda RSAB Harapan Kita, Jakarta, Indonesia.
Insights
A new, low-cost scoring system effectively predicts cardiac iron overload in children with thalassemia, using simple tests like hemoglobin and echocardiography. This tool aids early detection and risk stratification for better patient management.
Area of Science:
- Cardiology
- Hematology
- Medical Imaging
Background:
- Cardiac iron overload is a serious complication in thalassemia patients.
- Magnetic resonance imaging T2* is the gold standard but is costly and inaccessible in many regions, including Indonesia.
- There is a need for affordable methods to screen for cardiac iron overload.
Purpose of the Study:
- To develop and validate a predictive formula and scoring system for cardiac iron overload using low-cost investigations.
- To classify patients into risk categories for myocardial iron overload.
Main Methods:
- A cross-sectional study involving 80 children (aged 6-18) with thalassemia.
- Clinical examination, laboratory tests, ECG, echocardiography, tissue Doppler imaging, and MRI T2* were performed.
- Multivariate logistic regression was used to identify determinants and develop a predictive formula and scoring system.
Main Results:
- 8 out of 80 children (10%) had cardiac iron overload based on MRI T2*.
- Key predictors identified were hemoglobin, reticulocyte count, mitral deceleration time (DT), and tricuspid regurgitation (TR Vmax).
- A scoring system was developed with scores of 0 or 1 assigned based on thresholds for these parameters, categorizing risk as low, moderate, or high.
Conclusions:
- The developed formula and scoring system provide a low-cost, accessible method for predicting cardiac iron overload in pediatric thalassemia patients.
- This tool can aid in early risk stratification and management, especially where MRI T2* is unavailable.
- Further validation in larger cohorts is recommended.
Abstract:
Magnetic resonance imaging T2* screening is the gold standard for detecting cardiac iron overload in thalassemia, but its implementation in Indonesia is limited by the high costs. A predicting formula and scoring system based on low-cost investigations is needed. This cross-sectional study was conducted among thalassemia aged 6-18 years at Rumah Sakit Anak dan Bunda RSAB Harapan Kita Indonesia, during October 2017 to April 2019. All subjects were scheduled for clinical examination, laboratory tests, ECGs, echocardiography, tissue Doppler imaging, and MRIT2*. Multivariate logistic regression was used to identify the formula, simplifying to a scoring system, and risk classification for myocardial iron overload using odds ratio (OR) and 95% confidence interval (CI). Significance was set as p<0,05. We recruited 80 children, of those, 8 (10%) were classified as cardiac iron overload based on MRI T2* screening. Multivariate logistic regression showed determinant factors for cardiac iron overload were hemoglobin (95% CI:1.92-369.14), reticulocyte (95% CI:1.14-232.33), mitral deceleration time (DT) (95% CI:1.80-810.62,), and tricuspid regurgitation (TR Vmax) (95% CI:1.87-1942.56) with aOR of 26.65, 14.27, 38.22, and 60.27 respectively. The formula for cardiac iron overload was decided as 9.32 + 3.28 (Hb) + 2.9 (reticulocyte) + 3.64 (DT) + 4.1 (TR Vmax). A scoring system was defined by simplifying the formula of Hb ≤ 8.2 g/L, reticulocyte ≤0.33%, DT ≤ 114.5 cm/s, and TR Vmax ≥ 2.37 m/s were given a score of 1, while others were assigned 0. Total scores of 0 or 1, 2 and 3 or 4 were categorized as low, moderate, and high risk for iron cardiac overload. The cardiac iron overload formula was 9.32 + 3.28 (Hb) + 2.9 (reticulocyte) + 3.64 (DT) + 4.1 (TR Vmax). Variables of Hb ≤ 8.2 g/L, reticulocyte ≤0.33%, DT ≤ 114.5 cm/s, and TR Vmax ≥ 2.37 m/s were given a score of 1, while others were assigned 0. Total scores of 0 or 1, 2, and 3 or 4 were categorized as low, moderate, and high risk for iron cardiac overload.
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