Early detection of myocardial dysfunction in poorly treated pediatric thalassemia children and adolescents: Two Saudi centers experience

Ibrahim, Mohamed; Azab, Ahmed; Kamal, Naglaa M.; Ebrahim, S.; Shahin, Ashraf; Elsadek, Akram Elshafey; Abdulghany, Waleid E.; Sherief, Laila M.; Abdallah, Enas A.A.

doi:10.1016/j.amsu.2016.05.014

Cited by 10 publications

(8 citation statements)

References 52 publications

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“…[20] In addition, in 2016, a higher male predominance was reported among Saudi children with thalassemia as 70% were males, with a male to female ratio of 2.3:1. [28] Various other studies also reported that the male was slightly male predominance was detected in their study population. [21,[29][30][31] Al-Kherbash et al (2017) reported that the slight predominance was detected among as males represented 53.2% of the total studied cases, with a male to female ratio of 1.14:1.…”

Section: Discussionmentioning

confidence: 66%

Correlation of Cytogenetic, Molecular and Clinical Findings in Thalassemia Patients at a Tertiary Care Hospital

Nigam¹,

Verma²,

Agrawal³

et al. 2019

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BACKGROUND Thalassemia syndromes are most common monogenic disorders which can be inherited by children from their parents. This study aimed to evaluate the correlation of cytogenetic (1p36 deletion), molecular (common mutations) and clinical findings such as haematologic parameters, age at presentation, nutritional status and transfusion requirements with β-thalassemia patients. METHODS In total, 140 β-thalassemia patients were clinically classified into β-thalassemia major (TM) or intermedia (TI). The cytogenetic analysis for 1p36 deletion was carried out in suspected patients with phenotypic dysmorphic, developmental delay and mental retardation by karyotyping. ARMS-PCR was performed to identify the common mutations in β-thalassemia patients. All data were analyzed by SPSS software. RESULTS Karyotyping was performed in 10 β-Thalassemia patients for 1p36 del. based on their physical appearance and intelligence; but, none of them were found to be positive for 1p36 del. Total 101 (72.14%) patients were of thalassemia major and 39 (27.86%) were of thalassemia intermedia. Among the thalassemia major patients, 71 (70.30%) have common mutation. In our study the IVS I-5 G→C mutation was most common 62 (87.32%) in thalassemia major patients. The clinical parameters such as Hb (gm/dl), HbA2 (%), HbF (%), MCV (fL), MCH (pg) and MCHC (gm/dl) were significantly associated with β-Thalassemia major. CONCLUSIONS In β-Thalassemia patients, 1p36 deletion was not observed. In our study, the IVS I-5 G→C mutation was the most common [62(87.32%)]. There were significant differences on the age of first transfusion, Hb (gm/dl), HbA2 (%), HbF (%), MCV (fL), MCH (pg) and MCHC (gm/dl) in between β-Thalassemia major and intermedia.

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Section: Discussionmentioning

confidence: 66%

Correlation of Cytogenetic, Molecular and Clinical Findings in Thalassemia Patients at a Tertiary Care Hospital

Nigam¹,

Verma²,

Agrawal³

et al. 2019

jemds

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“…The assessment of systolic and diastolic left ventricular function using standard echocardiographic measures is subjected to several limitations [10] . Prior research has shown intriguing findings about the usefulness of TDI in the echocardiographic assessment of B-TM patients using derived indexes, such as the Tei index [29,30] .…”

Section: Discussionmentioning

confidence: 99%

Serum Copeptin as a Cardiomyopathy Predictor in Thalassemic Children: Relation to Tei Index

2024

The Egyptian Journal of Hospital Medicine

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Background: Among the most dangerous side effects for persons with beta thalassemia major (B-TM) are cardiac problems. Early recognition of cardiomyopathy guides early management and therefore minimize mortality and morbidity.Objective: This study aimed to assess copeptin levels as an early predictor of myocardial dysfunction in children with B-TM and to determine its relationship to tissue doppler derived Tei index in those patients. Subjects and methods:The study was conducted on 42 children with B-TM without known heart disease and 40 age -and sex-matched healthy controls. Serum copeptin was assayed by a commercial ELISA kit and left ventricular Tei index was measured using tissue Doppler imaging. Results: Copeptin levels were significantly higher in thalassemic patients compared with controls (P=0.014).Copeptin was correlated positively with age, disease duration, transfusion index, lactate dehydrogenase, indirect bilirubin, serum ferritin, end systolic and diastolic diameters and tricuspid regurgitant jet velocity. Copeptin, on the other hand, showed a negative correlation with fractional shortening and ejection percent. Copeptin and Tei index showed a substantial link in the investigation of correlation (p=0.001). Conclusion:Copeptin in B-TM may be regarded as an early indicator of cardiomyopathy. It ought to be included in thalassemic follow-up in order to detect and treat subclinical problems correctly, lowering the risk of detrimental heart failure (HF).

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“…Several studies showed varied EF values in TDT patients, but on average, they showed a decrease in the mean EF when compared to controls who did not suffer from TDT. [18,19] EF describes the fraction of the volume of blood pumped by the heart in each contraction. EF is mostly used to assess left heart systolic function, and although rarely it can also be used to assess systolic function of the right heart to the pulmonary circulation [20].…”

Section: Discussionmentioning

confidence: 99%

The correlation between serum ferritin levels and impaired heart function in children with transfusion dependent thalassemia

Dewanti¹,

Utamayasa²,

Ugrasena³

2021

GSC Biol. and Pharm. Sci.

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Background: Paediatric patients with transfusion dependent thalassemia require regular lifelong red cell transfusions. Blood transfusions increase the risk of iron overload, which can lead to cardiac hemosiderosis. Serum ferritin can be a parameter for evaluating systemic hemosiderosis. Objective: To evaluate the correlation between serum ferritin levels and impaired heart function in children with transfusion dependent thalassemia Methods: A cross sectional study was conducted with transfusion-dependent thalassemia who attended a cardiology and hemato-oncology outpatient clinic from January to December 2018 and had undergone more than 10 transfusion periods. Serum ferritin levels were taken from the mean of 3 measurements before echocardiography. Echocardiography examination was performed by a cardiologist using a 3D Doppler tool to evaluate ejection fraction (EF), tricuspid annular plane systolic excursion (TAPSE) and E / A ratio by calculating the initial diastole (E wave) and atrial contraction (wave A). Data analysis used the Spearman correlation with p <0.05. Results: There were 45 children with a median age of 10 (2-17) years and had received a median of 18 (10-51) blood transfusions. The mean serum ferritin level was 4,321 (1,168-15,233) ng / mL. Blood transfusion frequency was associated with an increase in serum ferritin (rho 0.74, P <0.005). From echocardiography examination, approximately 3/45 children had a feature of dilated cardiomyopathy. The mean value of EF 70 (SD 14.32), TAPSE 1.97 (SD 0.57) cm and an E / A ratio 1.68 (SD 0.46). Serum ferritin was negatively correlated with the ejection fraction (rho = -0.78, P <0.001), TAPSE (rho = -0.65, P <0.001) but positively correlated with the E / A ratio (rho = 0.67, P <0.001). Conclusion: Paediatric patients with TDT have decreased cardiac systolic and diastolic function, and serum ferritin correlates with the decreased cardiac function.

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Early detection of myocardial dysfunction in poorly treated pediatric thalassemia children and adolescents: Two Saudi centers experience

Cited by 10 publications

References 52 publications

Correlation of Cytogenetic, Molecular and Clinical Findings in Thalassemia Patients at a Tertiary Care Hospital

Correlation of Cytogenetic, Molecular and Clinical Findings in Thalassemia Patients at a Tertiary Care Hospital

Serum Copeptin as a Cardiomyopathy Predictor in Thalassemic Children: Relation to Tei Index

The correlation between serum ferritin levels and impaired heart function in children with transfusion dependent thalassemia

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