Assessment of ventricular dysfunction in Egyptian children with Beta-thalassemia major

Deraz, Salem; Naby, Sameh A Abd El; Mahmoud, Asmaa A.

doi:10.1016/j.hemonc.2020.07.003

Cited by 7 publications

(15 citation statements)

References 25 publications

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“…Higher LV early diastolic filling and a high E/A ratio in this case study indicated a restricted [11]. This was also in line with a prior analysis that stated that the most common finding in individuals with TM is a high E/A ratio [12].…”

Section: Discussionsupporting

confidence: 92%

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Frequency of Cardiomyopathy in Children having Thalassemia Major: A Cross-Sectional Study

Jamali¹,

Mujeeb-u-Rehman

Chand

et al. 2022

JPRI

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Aim: To determine the frequency of cardiomyopathy in children having Thalassemia major diagnosed on the basis of Echocardiography. Study Design: A cross-sectional study. Place and Duration: Paediatric department at Peoples medical college hospital (PMCH), Nawabshah from September 2019 to March 2020. Methodology: A total of 236 Thalessemic children visiting as outpatients or inpatients meeting inclusion criteria were enrolled. Informed consent was taken from the patients or their attendants. All demographic variables that included age, gender, address, educational status, family history, age of diagnosis of the disease, start of treatment and number of transfusions graded as none, occasional, were included. Echocardiography findings were performed and then recorded. Results: Mean age of the patients was 10.60±4.064 (5-18) years, 110(49.1%) study subjects were female and 126(50.8%) were male patients. While 75(31.78%) study subjects have family history of thalassemia. A total of 87(36.9%) study subjects have left ventricular dysfunction, 13(14.9%) have global dysfunction, 11(12.6%) have isolated systolic dysfunction and 63(72.4%) have isolated diastolic dysfunction. Conclusion: In -thalassemia, heart disease is the key determinant of prognosis and survival. In this study we found a significant number of thalassemia children having cardiac involvement and ventricular dysfunction.

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Section: Discussionsupporting

confidence: 92%

“…In Table 1 descriptive statistics of all quantitative variables was calculated in term of mean and standard deviation. Mean age of the patients was 10.60±4.064 (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)…”

Section: Resultsmentioning

confidence: 99%

Frequency of Cardiomyopathy in Children having Thalassemia Major: A Cross-Sectional Study

Jamali¹,

Mujeeb-u-Rehman

Chand

et al. 2022

JPRI

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“…There was a significant (p<0.05) increase in plasma BNP levels in TDT patients than controls with normal EF in the current study. These results are also reported by Kremastinos et al 2007 31 and Deraz et al 2021, 32 who found significantly (p<0.05) high levels of BNP with no clinical presentation of heart failure (HF) nor abnormal EF.…”

Section: Discussionsupporting

confidence: 82%

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

Ahmed¹,

Salih²,

Omer³

et al. 2022

TCRM

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“…Currently, there is no effective treatment for severe beta thalassemia; however, regular blood transfusions and iron removal are used clinically to prolong the lives of children. Despite these interventions, it is still difficult to avoid direct death due to liver and kidney damage as well as heart failure before puberty [ 11 ]. The majority of children with thalassemia major require long-term drug treatment and medical care after they are discharged from the hospital because the disease has a prolonged treatment course, repeated conditions, and many complications.…”

Section: Discussionmentioning

confidence: 99%

Clinical Study of Mobile Application- (App-) Based Family-Centered Care (FCC) Model Combined with Comprehensive Iron Removal Treatment in Children with Severe Beta Thalassemia

Chen

Huang

et al. 2022

Applied Bionics and Biomechanics

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Background and Objective. Hemoglobinopathy is one of the most prevalent monogenic disorders in the world. Thalassemia is characterized by autosomal recessive deficiencies in hemoglobin production. The difficulties of iron overload caused by transfusions, which are the foundation of illness management in the majority of patients with severe thalassemia, may further worsen the clinical features. There are numerous obstacles and restrictions to the currently accessible conventional therapy for thalassemia. The purpose of the study is to investigate the clinical impact of the family-centered care (FCC) model based on a mobile application (app) in conjunction with comprehensive iron removal therapy for children with severe beta thalassemia. Methods. A retrospective study was conducted on the clinical records of 148 children diagnosed with severe beta thalassemia who were admitted to our hospital between October 2018 and September 2021. The patients were separated into two groups, a control group and an intervention group, with 74 cases in each group, according to the various care approaches. The basic treatment regimen was given to all of the children: deferoxamine mesylate combined with deferiprone. During treatment, the control group received routine care, and the intervention group adopted the FCC model based on a mobile app. The quality of life scale for children and adolescents (QLSCA) score, the family assessment device (FAD) score, the exercise of self-care agency scale (ESCA) score, and the medication compliance scale score were compared between the two groups. Results. The QLSCA score, ESCA score, and medication compliance scale score of the intervention group were significantly higher than those of the control group and showed a significant difference (intergroup effect: F = 198.400 , 259.200, and 129.800, all P < 0.001 ). Scores in both groups increased over time (time effect: F = 19.350 , 40.830, and 12.130, all P < 0.001 ), and there was an interaction effect between grouping and time (interaction effect: F = 3.937 , 12.020, and 5.028). The P values were 0.020, <0.001, and 0.007. The FAD score of the intervention group was significantly lower than that of the control group (intergroup effect: F = 177.200 , P < 0.001 ). The FAD scores of both groups decreased over time (time effect: F = 7.921 , P = 0.005 ). There was an interaction effect between groups and time (interaction effect: F = 5.206 , P = 0.006 ). Conclusion. The application effect of the mobile app-based FCC model combined with the comprehensive iron removal treatment program in children with severe beta thalassemia is significant, which can significantly improve the quality of life, family function, self-care ability, and medication compliance of children, and has high clinical application value.

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Assessment of ventricular dysfunction in Egyptian children with Beta-thalassemia major

Cited by 7 publications

References 25 publications

Frequency of Cardiomyopathy in Children having Thalassemia Major: A Cross-Sectional Study

Frequency of Cardiomyopathy in Children having Thalassemia Major: A Cross-Sectional Study

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

Clinical Study of Mobile Application- (App-) Based Family-Centered Care (FCC) Model Combined with Comprehensive Iron Removal Treatment in Children with Severe Beta Thalassemia

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