Quality of life in Sardinian patients with transfusion-dependent Thalassemia: a cross-sectional study

Floris, Francesco; Comitini, Federica; Leoni, G; Moi, Paolo; Morittu, Maddalena; Orecchia, Valeria; Perra, Maria Teresa; Pilia, Maria Paola; Zappu, Antonietta; Casini, Maria Rosaria; Origa, Raffaella

doi:10.1007/s11136-018-1911-7

Cited by 20 publications

(23 citation statements)

References 23 publications

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“…Negative correlation between serum ferritin level and the total QOL score had been demonstrated in this study. This is in agreement with other studies of thalassemia patients that demonstrated that higher serum ferritin levels were associated with lower total QOL scores . β‐TI patients develop iron overload, primarily because of the increased intestinal absorption of iron, in addition to the burden of the number of transfusions they receive throughout their life.…”

Section: Discussionsupporting

confidence: 91%

“…Patients with β‐TI usually present with milder clinical symptoms and a later onset of hemolytic anemia compared with those with β‐TM. QOL in β‐TM children has been widely investigated . However, there is limited knowledge regarding QOL and factors that affect it in β‐TI.…”

Section: Discussionmentioning

confidence: 99%

“…QOL in -TM children has been widely investigated. 16,17 However, there is limited knowledge regarding QOL and factors that affect it in -TI. In this case-control study, HRQOL and its related factors were evaluated in children and adolescents with -TI.…”

Section: Discussionmentioning

confidence: 99%

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The impact of illness perception and socio‐clinico‐demographic factors on perceived quality of life in children and adolescents with thalassemia intermedia

Atwa

Wahed

2019

Pediatric Blood & Cancer

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Background/objectives β‐Thalassemia intermedia (β‐TI) accounts for up to one‐fourth of β‐thalassemia patients. Evaluating and improving quality of life (QOL) should be a goal in β‐TI follow‐up and management strategies. Patients’ perceptions of their illness and its treatment may impact their QOL. This study aimed to evaluate QOL and the factors that affect it in children with β‐TI and to determine the impact of the patients’ and their mothers’ perceptions of the illness on patients’ QOL. Design/methods This was a case–control study. A total of 143 children and adolescents (71 β‐TI patients and 72 healthy controls) were enrolled. QOL, as perceived by the children and their mothers, was assessed using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scale. Perceptions of the illness by the mothers and children were assessed using the Brief Illness Perceptions Questionnaire (Brief IPQ). The patients’ clinical and sociodemographic data were extracted from their medical records. Results The controls had higher QOL scores in nearly all domains (P < 0.01). The patients and mothers who had higher illness perception scores had better QOL scores (P < 0.01). Patient age and serum ferritin levels correlated negatively with QOL, while mean hemoglobin levels correlated positively. The use of hydroxyurea in treatment and high illness perception were independent predictors of better QOL. Conclusion QOL is significantly affected in β‐TI patients; maintaining a suitable hemoglobin level and standard levels of body iron are associated with better QOL. Patients’ and their mothers’ perceptions of the illness play an important role in QOL.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

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The impact of illness perception and socio‐clinico‐demographic factors on perceived quality of life in children and adolescents with thalassemia intermedia

Atwa

Wahed

2019

Pediatric Blood & Cancer

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Section: Complicaciones De La β-Talasemiaunclassified

“…La aparición de métodos no invasivos para medir el hierro orgánico antes de la aparición de los síntomas clínicos junto con los nuevos quelantes de hierro han reducido la incidencia de complicaciones relacionadas con la sobrecarga de hierro [43]. Sin embargo, a pesar de la correcta aplicación de intervenciones diagnósticas y terapéuticas los pacientes talasémicos pueden experimentar enfermedades secundarias o complicaciones que han asumido un papel nuevo e importante en el curso de esta enfermedad en los últimos años [43,44]. La hipoxia, la eritropoyesis ineficaz y la sobrecarga de hierro contribuyen a producir las complicaciones en múltiples órganos; de los cuales el Universidad de Manizales -Facultad de Ciencias de la Salud hígado, el corazón y las glándulas endocrinas son los órganos afectados con mayor frecuencia y es en quienes finalmente se observan complicaciones cirrosis, insuficiencia cardíaca y defectos endocrinos (hipotiroidismo, hipopituitarismo, hipogonadismo, hipoparatiroidismo) [45].…”

Section: Complicaciones De La β-Talasemiaunclassified

Beta-Talasemia: Un mundo de complicaciones con nuevas alternativas de tratamiento.

Alzate

2019

archmed

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Introducción: β-talasemia, es causada por mutaciones en el gen de la globina HBB, que codifica la subunidad β de la HbA. La enfermedad es conocida por ser altamente prevalente en el área que se extiende desde África subsahariana, a través de la región mediterránea y Medio Oriente. En Colombia, se han reportado varios estudios independientes de hemoglobinopatías en ciudades como Cartagena, Buenaventura, Cali, San Andrés y Providencia debido a su gran población afrodescendiente sobre la cual las Talasemias y otras hemoglobinopatías tienen incidencia directa. Objetivo: recolectar datos acerca de las características clínicas, complicaciones y clasificaciones de β-talasemia con el fin de brindar una fuente de información que permita realizar un diagnóstico eficaz y en consecuencia un tratamiento que busque llegar a la curación completa de los pacientes que sufren esta condición, con el mínimo de complicaciones para los mismos. Conclusión: la β-Talasemia es una hemoglobinopatía estructural que tiene un porcentaje de prevalencia e incidencia importante en el mundo. En Colombia no se tiene claro cuál es la epidemiología real para esta condición puesto que no se han realizado estudios que abarquen una muestra adecuada y significativa. Esta enfermedad genera múltiples complicaciones en diferentes órganos, que no solo están asociadas al desarrollo de la enfermedad, sino también a la terapia de trasfusión a largo plazo. Por esta razón, los nuevos tratamientos están encaminas a lograr en un futuro la curación completa, reduciendo al máximo las complicaciones.

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

et al. 2020

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Quality of life in Sardinian patients with transfusion-dependent Thalassemia: a cross-sectional study

Abstract: Adult subjects with Thalassemia who live in Western countries have a good quality of life in accordance with the advances in the management of the disease.

Cited by 20 publications

References 23 publications

The impact of illness perception and socio‐clinico‐demographic factors on perceived quality of life in children and adolescents with thalassemia intermedia

The impact of illness perception and socio‐clinico‐demographic factors on perceived quality of life in children and adolescents with thalassemia intermedia

Beta-Talasemia: Un mundo de complicaciones con nuevas alternativas de tratamiento.

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

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