A Perspective on Thalassaemia

Hussain, Zeeshan; Ansari, Saqib Hussain; Shamsi, Tahir

doi:10.21089/njhs.32.0036

Cited by 3 publications

(3 citation statements)

References 24 publications

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“…Annually 5000-6000 births with this autosomal disorder, and registered cases are approximately 100,000. The probability of birth of a child with β-Thalassemia major is 25%, carrier trait 50%, and normal 25% at every pregnancy when two carrier traits or thalassemia minor individuals tie a wedding knot 6 . BTM patients presented with common symptoms of progressive pallor and failure to thrive and need regular blood transfusions for their survival, splenomegaly (abdominal enlargement), growth retardation, and delayed puberty 7 .…”

Section: Introductionmentioning

confidence: 99%

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Effect of hydroxyurea on quality of life with a moderating role of healthcare professionals’ performance: A view from the beta-thalassemia patients.

Sheikh

Bibi

Siddiqui

et al. 2022

Int. j. endorsing health sci. res.

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Background: Transfusion-dependent (TDT) β –Thalassemia patients have lower HRQoL than healthy individuals. The impact of Hydroxyurea is always measured with the clinical investigations in terms of laboratory diagnosis. This study aimed to seek out the impact of the drug Hydroxyurea on improving physical health problems of Quality of Life (QoL) of β – Thalassemia patients with the moderating role of healthcare professionals. Methodology: In this research, a deductive approach was adopted whereby hypotheses were originated first, followed by organizing a well-structured questionnaire as a tool for measuring the data. The nature of the research was explanatory and based on primary quantitative data. The data was collected from 290 patients visited in the outpatient and daycare departments of the National Institute of Blood Diseases and Bone Marrow Transplantation Hospital. Results: This study revealed that the impact of Hydroxyurea therapy on improving the physical health problems of Beta Thalassemia is not evident as per the analysis (r=0.079; p=0.178). Similarly, a weak positive non-significant correlation exists between Hydroxyurea therapy and the performance of healthcare professionals in the hospital (r=0.016; p=0.782). Moreover, no significant improvement was observed in physical health problems and health domain of QoL of BT patients by moderating the role of healthcare professional performance (p=0.4471 and 0.4102, respectively). Conclusion: It is concluded that moderating the role of healthcare professionals' performance does not improve the impact of Hydroxyurea on physical health problems and domains of QoL of β – Thalassemia patients.

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Section: Introductionmentioning

confidence: 99%

“…Fetal Hb augmenting agents Hydroxyurea (HU) used in-vitro and in vivo increases fetal hemoglobin to fulfill the decreased level of adult hemoglobin. The success rate of HU has upsurged in past years, and responders showed transfusion independence to improve the life expectancy of patients 6 .…”

Section: Introductionmentioning

confidence: 99%

Effect of hydroxyurea on quality of life with a moderating role of healthcare professionals’ performance: A view from the beta-thalassemia patients.

Sheikh

Bibi

Siddiqui

et al. 2022

Int. j. endorsing health sci. res.

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“…Transfusion transmitted viral infections are another major problem faced by many developing countries. Above all, the overall cost of regular packed red cells (PRCs) transfusion and iron chelation in the developing world is ~3000 USD annually, 4 which poses an enormous burden on the patient's family and on the country's healthcare system. As far as allogeneic hematopoietic stem cell transplantation is concerned, high cost and technical difficulties, that is, unavailability of matched donor and so on, make allogeneic hematopoietic stem cell transplantation unattainable to the majority of the patients.…”

mentioning

confidence: 99%

A Pragmatic Scoring Tool to Predict Hydroxyurea Response Among β-Thalassemia Major Patients in Pakistan

Ansari

Hussain

Zohaib

et al. 2021

Journal of Pediatric Hematology/Oncology

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Despite high prevalence and incidence of β-thalassemia in Pakistan, there is very limited work on the use of hydroxyurea (HU) in thalassemia patients in the country. This is the first insight regarding genetic profiling of BCL11A and HU responses in Pakistani β-thalassemia. It correlates single-nucleotide polymorphisms on BCL11A (rs4671393, rs766432) and HBG2 (XmnI), age at first transfusion, and β-globin mutations with HU response in β-thalassemia major (BTM). Of 272 patients treated with HU, 98 were complete responders, 55 partial responders, and 119 nonresponders. Our analysis shows that HU response was significantly associated with patients having IVSI-1 or CD 30 mutation (P < 0.001), age at first transfusion > 1 year (P < 0.001), and with the presence of XmnI polymorphism (P < 0.001). The single-nucleotide polymorphisms of BCL11A were more prevalent among responders, but could not show significant association with HU response (P > 0.05). Cumulative effect of all 5 predicting factors through simple binary scoring indicates that the likelihood of HU response increases with the number of primary and secondary genetic modifiers (P < 0.001). Predictors scoring is a pragmatic tool to foresee HU response in patients with BTM. The authors recommend a score of ≥ 2 for starting HU therapy in Pakistani patients with BTM.

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Evaluation of the High Resolution Melting Approach for Detection of β-Thalassemia Gene Mutations

2021

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A Perspective on Thalassaemia

Cited by 3 publications

References 24 publications

Effect of hydroxyurea on quality of life with a moderating role of healthcare professionals’ performance: A view from the beta-thalassemia patients.

Effect of hydroxyurea on quality of life with a moderating role of healthcare professionals’ performance: A view from the beta-thalassemia patients.

A Pragmatic Scoring Tool to Predict Hydroxyurea Response Among β-Thalassemia Major Patients in Pakistan

Evaluation of the High Resolution Melting Approach for Detection of β-Thalassemia Gene Mutations

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