Severity of heart failure and health-related quality of life in beta-thalassemia patients: a cross-sectional study

Dimitroglou, Yannis; Anagnostopoulos, Fotios; Aggeli, Constantina; Delicou, Sophia; Xydaki, Aikaterini; Patsourakos, D; Tousoulis, Dimitris

doi:10.1007/s00277-020-04032-3

Cited by 6 publications

(9 citation statements)

References 37 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In Greece, the stage of heart failure in adult patients emerged as one of the most important factors that are negatively related to physical functioning, role-physical, general health, social functioning, and vitality ( 12 ).…”

Section: Resultsmentioning

confidence: 99%

“…Fatigue is an independent predictor of low HRQOL that has been associated with lower executive functioning and working memory of affected people (5,(8)(9)(10). Concurrently, regular blood transfusions and disease complications in beta-thalassemia patients affect HRQOL, and iron overload, comorbidities, and fatigue are the most represented etiologies of impaired life functioning (11)(12)(13). Furthermore, patients can encounter stigma for other reasons, such as race, disability, socioeconomic status, and delayed growth and puberty (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14).…”

Section: Introductionmentioning

confidence: 99%

“…Concurrently, regular blood transfusions and disease complications in beta-thalassemia patients affect HRQOL, and iron overload, comorbidities, and fatigue are the most represented etiologies of impaired life functioning (11)(12)(13). Furthermore, patients can encounter stigma for other reasons, such as race, disability, socioeconomic status, and delayed growth and puberty (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). The heterogeneous distribution of treatment opportunities and multidisciplinary management worldwide can also influence HRQOL perception in different settings (3,4).…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Health-related quality of life in hemoglobinopathies: A systematic review from a global perspective

2022

View full text Add to dashboard Cite

BackgroundSickle cell disease (SCD) and thalassemia are inherited blood disorders, which can lead to life-threatening events and chronic organ damage. Recent advances in treatments have increased life expectancy, and hemoglobinopathies have become chronic illnesses with social and emotional impairments. Thus, health-related quality of life (HRQOL) assessment has a fundamental role in disease management and treatment, and generic and disease-specific questionnaires are reliable and validated measures to estimate disease burden. The heterogeneous distribution of treatment opportunities worldwide influences physical, social, and emotional disease perception.ObjectivesTo review publications concerning HRQOL for SCD and thalassemia in different areas of the world in order to gather a global perspective of questionnaires used and outcomes evaluated.MethodsA systematic review of the literature was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The Medline databases were searched on 29 September 2021. Inclusion criteria were as follows: (1) studies of HRQOL assessment in SCD and thalassemia patients by using the PROMIS, the SF-36, the SCSES, the PedsQL-SCD, the PedsQOL generic core scale, the ASCQ-Me, and the TranQoL; and (2) every article type, including non-English studies. We excluded studies that were not limited to SCD or thalassemia and studies that were not specific to hemoglobinopathies, and not consistent with the topic of HRQOL assessment. We did not include the gray literature. A total of 102 out of 124 articles from PubMed, Cochrane Library, and Google Scholar were eligible for inclusion (66 SCD articles and 36 thalassemia articles). The quality of studies was assessed through Critical Appraisal tools for use in JBI Systematic Reviews. Data extraction was conducted using a standardized data collection form (authors, year and country of publication, study design, age and number of patients, HRQOL questionnaires, questionnaire language, and clinical outcomes).ResultsThe evaluation of HRQOL was conducted on all continents, but differences in the worldwide frequency of HRQOL assessment were observed. HRQOL of SCD patients was less investigated in Europe. HRQOL of thalassemia patients was less investigated in South-East Asia and Africa. Generic HRQOL questionnaires (PROMIS, SF-36, and PedsQL) were frequently adopted, while disease-specific ones (ASCQ-Me, SCSES for SCD, and TranQoL for thalassemia) were less used. Translation into local languages has been often performed.ConclusionHealth-related quality of life is a complex outcome that has been increasingly incorporated in clinical research and clinical practice worldwide, although with regional differences. Disease-specific outcomes (pain for SCD and transfusion burden for thalassemia) and healthcare system characteristics, particularly in low-income countries, have an impact on HRQOL and should be considered in healthcare plans.

show abstract

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Health-related quality of life in hemoglobinopathies: A systematic review from a global perspective

2022

View full text Add to dashboard Cite

show abstract

“…27 There were multiple publications where cardiac dysfunction caused by myocardial iron deposition in thalassemia patients has been reported, especially concerning the consequent HF. [2][3][4]28 Furthermore, the present study showed that men have a higher disease burden on both thalassemias and thalassemias combined with HF than women at the global and regional levels, and the gap is likely to expand further. These findings above were consistent with previous research done by Hahalis of both genders with similar serum ferritin a similar number of blood units infusion.…”

Section: Discussionmentioning

confidence: 56%

Changing trends in the prevalence of heart failure impairment with Thalassemias over three decades

Tang,

Zhang,

Deng

et al. 2023

Eur J Clin Investigation

View full text Add to dashboard Cite

BackgroundTo assess the prevalence trend and contributing factors of heart failure (HF) impairment with thalassemias at global, regional and national levels.MethodsData on HF impairment with thalassemias was collected from the Global Burden of Disease study. The absolute number and prevalence of the disease were systematically collected for each year, and the estimated annual percentage changes (EAPC) in HF impairment were calculated by gender, region and country to measure temporal trends.ResultsThalassemias have caused a significant global burden since 1990, and the case number of HF related to thalassemias has been steadily increasing. The highest case number of HF impairments with thalassemias is observed in China (7739 cases) and the highest prevalence is in Pakistan (1.61 per 100,000) currently. Besides, the middle sociodemographic index (SDI) region carries the highest burden of comorbid disease yet exhibits the most evident trend for improvement across the five regions (EAPC = −.98). The burden of thalassemias and comorbid HF is generally higher in males than females with the gender gap growing chasm in the future. Besides, the hotspots of HF impairment with thalassemias have gradually shifted to low SDI regions, though middle SDI regions still hold a relatively higher prevalence (.37 per 100,000) across different regions.ConclusionsThe burden of thalassemias and accompanying HF, as well as their temporal trends, vary greatly across countries and regions. These findings can improve understanding of these conditions and guide policymakers in developing appropriate policies to address disparities between countries.

show abstract

“…2 Apesar do avanço terapêutico, a mortalidade nos adultos continua alta mesmo nos países desenvolvidos, com média de idade abaixo dos 50 anos. 3 Ainda que as melhorias nos protocolos de transfusão sanguínea e de uso de agentes quelantes de ferro aumentaram a sobrevida dos pacientes com talassemia, a principal causa de morbidade e mortalidade nesses pacientes é a doença cardíaca, 4 responsável por 75% das mortes. 5 No Brasil, 10 a 20% dos indivíduos com talassemia dependentes de transfusão apresentam sobrecarga de ferro severa, com incidência de cardiopatias de 5%.…”

Section: Introductionunclassified

Prevalência das Complicações Cardiovasculares nos Indivíduos com Anemia Falciforme e Outras Hemoglobinopatias: Uma Revisão Sistemática

Lopes¹,

Dantas²,

Ladeia³

2022

Arquivos Brasileiros De Cardiologia

View full text Add to dashboard Cite

Fundamento: A anemia falciforme (AF) é uma doença hereditária cujas complicações cardiovasculares são a principal causa de morte, o mesmo sendo observado em outras hemoglobinopatias. A identificação precoce dessas alterações pode modificar favoravelmente o curso da doença. Objetivo: Comparar a prevalência de complicações cardiovasculares entre indivíduos com AF e indivíduos com outras hemoglobinopatias. Métodos: Seguindo recomendações do protocolo PRISMA, realizou-se revisão sistemática da literatura com buscas nas bases de dados PubMed/Medline, associadas à busca manual. Incluídos estudos que analisaram a prevalência das alterações cardiovasculares nas hemoglobinopatias (AF, traço falciforme, hemoglobinopatia SC, alfatalassemia e betatalassemia). A qualidade metodológica dos artigos foi realizada pela escala de Newcastle-Ottawa. Resultados: Foram selecionados para análise quatro estudos, resultando em um tamanho amostral de 582 participantes: 289 portadores de AF, 133 possuem hemoglobinopatia SC, 40 com betatalassemia, 100 indivíduos saudáveis e nenhum com alfatalassemia ou traço falcêmico. Dilatação das câmaras cardíacas, hipertrofia ventricular esquerda e direita, hipertensão pulmonar, disfunção diastólica, insuficiência mitral e insuficiência tricúspide são mais prevalentes na AF do que nas demais hemoglobinopatias consideradas. A sobrecarga miocárdica de ferro é mais frequente na talassemia maior do que na AF. A função sistólica foi similar entre as hemoglobinopatias. Conclusão: Verificou-se maior comprometimento cardiovascular nos indivíduos com AF do que naqueles com as demais hemoglobinopatias, reforçando a necessidade de acompanhamento cardiovascular regular e frequente nos pacientes falcêmicos. Palavras-chave: Anemia Falciforme; Complicações Cardiovasculares na Gravidez; Hemoglobinopatias.

show abstract

Severity of heart failure and health-related quality of life in beta-thalassemia patients: a cross-sectional study

Cited by 6 publications

References 37 publications

Health-related quality of life in hemoglobinopathies: A systematic review from a global perspective

Health-related quality of life in hemoglobinopathies: A systematic review from a global perspective

Changing trends in the prevalence of heart failure impairment with Thalassemias over three decades

Prevalência das Complicações Cardiovasculares nos Indivíduos com Anemia Falciforme e Outras Hemoglobinopatias: Uma Revisão Sistemática

Contact Info

Product

Resources

About