Early Detection of Iron Overload Cardiomyopathy in Transfusion Dependent Thalassemia Patients in Sulaimaniyah City, Iraq.

Background: Lifelong blood transfusion is recommended for patients with transfusion-dependent thalassemia (TDT) that lead to iron overload and results in cardiomyopathy (CM). Aim: To assess the accuracy of several electrocardiographic (ECG) data in patients at high risk of arrhythmia, early detection of structural and functional changes in left atrium and ventricle using ECG and echocardiography (two dimensional, M-mode echocardiography along with Doppler studies), and to observe the correlations between plasma B-type atrial natriuretic peptide (BNP)/serum ferritin and ECG with Doppler as well as echocardiographic changes in patients with TDT. Methods: The current prospective case-control study included 75 TDT patients and 74 control subjects with the mean age of 9.55 and 9.93 years, respectively. Participants were assessed for the socio-demographic, physical examinations, serum ferritin, plasma BNP, ECG, 2D echocardiography, and tissue/pulse wave Doppler. Results: The mean of serum ferritin and plasma BNP were significantly (p<0.001) higher in the cases (1475.19 ng/mL and 47.63 pg/mL, respectively) than controls (41.3 ng/mL and BNP 23.37 pg/mL, respectively). ECG findings showed significant higher P-wave dispersion, QRS duration, QT duration and dispersion, and JTc dispersion in cases than controls. Echocardiography findings revealed diastolic dysfunction with preserved ejection fraction (EF) in thalassemia cases, as well as significant higher left ventricular (LV) mass, LV internal diameters during systole and diastole (LVIDs and d) and LV posterior wall thickness during diastole (LVPWd) in cases than controls (p<0.05). Also, a significant (p<0.05) correlation between BNP and QT dispersion was found in patients. Conclusion: These findings suggest the usefulness of ECG, 2D echocardiographic, Doppler studies and plasma BNP, with no significant beneficial effect of serum ferritin level in detecting early cardiac changes in patients with TDT.

Corrigendum to "Beta-Thalassemia Intermedia: A Single Thalassemia Center Experience from Northeastern Iraq".

[This corrects the article DOI: 10.1155/2020/2807120.].

Beta-Thalassemia Intermedia: A Single Thalassemia Center Experience from Northeastern Iraq.

OBJECTIVE: To determine the molecular characterization and disease-associated complications of beta-thalassemia intermedia (ß-TI) patients in Sulaymaniyah province, northeastern Iraq. METHODS: A total of 159 ß-TI) patients in Sulaymaniyah province, northeastern Iraq. ß-TI) patients in Sulaymaniyah province, northeastern Iraq. RESULTS: Nineteen different ß-globin gene mutations arranged in 37 various genotypes were determined. The most frequent were IVS-II-I (G>A) (47.2%), followed by IVS-I-6 (T>C) (23.3%) and IVS-I-110 (G>A) (5%). Among disease-related morbidities documented, bone disease amounted to 53% (facial deformity and osteoporosis), followed by endocrinopathies 17.6% (growth retardation and subclinical hypothyroidism), cholelithiasis 13.8%, pulmonary hypertension 11.3%, and abnormal liver function test 7.5%, whereas venous thrombosis, extramedullary hemopoiesis, and leg ulcer were less frequently observed. Age ≥ 35 and female sex were risk factors for cholelithiasis, while age was an independent risk for hypothyroidism and female sex was associated with increased risk for osteoporosis. Mean serum ferritin of ≥1000 µg/L was associated with an increased risk of osteoporosis, whereas chelation therapy was protective for a multitude of other complications. Transfusion, on the other hand, increased the risk of osteoporosis, yet it was protective for cholelithiasis and hypothyroidism. Moreover, splenectomy was protective for cholelithiasis, although it was an independent risk for hypothyroidism. Finally, hydroxyurea was associated with an increased risk of osteoporosis, while it was protective for cholelithiasis. Discussion and Conclusion. ß +-thalassemia mutation had contributed to 41.25 of families with a less severe ß-thalassemia phenotype in the northeastern part of Iraq, justifying the need to investigate the contribution of genetic modifiers in ameliorating disease severity. In addition, the substantial number of ß-TI patients developed disease-related morbidities, which necessitates the need for more appropriate clinical management with earlier intervention.ß-TI) patients in Sulaymaniyah province, northeastern Iraq. µg/L was associated with an increased risk of osteoporosis, whereas chelation therapy was protective for a multitude of other complications. Transfusion, on the other hand, increased the risk of osteoporosis, yet it was protective for cholelithiasis and hypothyroidism. Moreover, splenectomy was protective for cholelithiasis, although it was an independent risk for hypothyroidism. Finally, hydroxyurea was associated with an increased risk of osteoporosis, while it was protective for cholelithiasis. Discussion and Conclusion. ß +-thalassemia mutation had contributed to 41.25 of families with a less severe ß-thalassemia phenotype in the northeastern part of Iraq, justifying the need to investigate the contribution of genetic modifiers in ameliorating disease severity. In addition, the substantial number of ß-TI patients developed disease-related morbidities, which necessitates the need for more appropriate clinical management with earlier intervention.Discussion and Conclusion. ß +-thalassemia mutation had contributed to 41.25 of families with a less severe ß-thalassemia phenotype in the northeastern part of Iraq, justifying the need to investigate the contribution of genetic modifiers in ameliorating disease severity. In addition, the substantial number of ß-TI patients developed disease-related morbidities, which necessitates the need for more appropriate clinical management with earlier intervention.ß-TI) patients in Sulaymaniyah province, northeastern Iraq. ß-TI) patients in Sulaymaniyah province, northeastern Iraq.