Prise en charge thérapeutique des patients atteints de bêta-thalassémie majeure dans un service de pédiatrie du sud tunisien : à propos de 26 cas

Maâloul, I.; Laaroussi, O.; Jedidi, Inès; Sfaihi, Lamia; Kmiha, Sana; Kamoun, T.; Aloulou, Hajer; Hachicha, M.

doi:10.1016/j.tracli.2017.11.002

Cited by 4 publications

(2 citation statements)

References 14 publications

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“…Our study's median age at first transfusion was 12.00 (IQR: 7.00) months. Maaloul et al reported that the mean age at which patients with β-thalassemia major received their first blood transfusion was 11.5 months, which was similar to our study, as were the results of Ansari et al, who reported a median age of 9 (range: 6-15) months when their study sample received their first transfusion (Ansari et al, 2022;Maaloul et al, 2018). Requirements for transfusions at an early age translate into an early requirement for chelation therapy; patients of β-thalassemia major require adequate monitoring of iron status and chelation from an early age if complications secondary to iron overload are to be prevented.…”

Section: Discussionsupporting

confidence: 91%

Incidence of Dilated Cardiomyopathy in Patients of Thalassemia Major Receiving Deferasirox and Deferoxamine as Chelators

MUSTAFA,

NADEEM,

KHAN

et al. 2023

Biol Clin Sci Res J

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The objective of this study was to determine the frequency of dilated cardiomyopathy in patients with β-thalassemia major who received treatment with deferasirox and deferoxamine. A descriptive cross-sectional study was conducted in the Department of Paediatrics, Combined Military Hospital, Rawalpindi, from May 2022 to January 2023. All 746 paediatric patients aged between 4 to 18 years, of both genders, who had been diagnosed with β-thalassemia major and had received chelation with either deferasirox or deferoxamine for at least one continuous year were included. Patients who were non-compliant with treatment based on history or suffered from concurrent chronic cardiopulmonary, renal (including proteinuria), hepatic, or neoplastic disease, those who suffered from malabsorption syndromes, or those who had undergone gastrointestinal surgery were excluded. All patients underwent a 2D echocardiographic study to document cardiac dilatation and measurement of ejection fraction. Dilated cardiomyopathy was defined as dilating one or both heart ventricles with an ejection fraction of less than 40%. Patients were divided into two groups: those who had received deferasirox and those who had received deferoxamine. Data was analyzed using SPSS 26.0. The study population had a median age of 8.00 (IQR: 5.00) years, with a male majority of 384 (51.5%). The median time on chelation therapy was 23.00 (IQR: 10.00) months, while the median age at first transfusion was 12.00 (IQR: 7.00) months. The median number of lifetime transfusions was 60.50 (IQR: 33.00), while only 431 (57.8%) patients had received chelator therapy previously. Out of the total sample population, 36 (4.8%) patients had dilated cardiomyopathy, of whom 22 (5.9%) occurred with deferasirox while 14 (3.8%) were seen with deferoxamine (p=0.172). The study concluded that the frequency of dilated cardiomyopathy secondary to iron deposition in the myocardium of patients with β-thalassemia major on deferasirox for at least one year was similar to those using deferoxamine for the same minimum period.

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Section: Discussionsupporting

confidence: 91%

Incidence of Dilated Cardiomyopathy in Patients of Thalassemia Major Receiving Deferasirox and Deferoxamine as Chelators

MUSTAFA,

NADEEM,

KHAN

et al. 2023

Biol Clin Sci Res J

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“…Consanguinity therefore plays an important role in the occurrence of familial forms, with variable genotypes, as shown by our clinical cases 1 and 2, where three cases were found, two of them alive and the older brother probably dead from beta-thalassaemia major. Other authors, notably Barouni [12,13,14,15]. Barouni M et al also found in their study that 37.1% of thalassaemia patients had at least one brother or sister affected by the same disease [12].…”

Section: Clinical Diagnosismentioning

confidence: 80%

Beta-thalassaemia: Four cases at the mother and child centre of the Chantal Biya Foundation

Pondy Ongotsoyi AH,

Yemdji M,

Ahmed L

et al. 2024

Int. J. Sci. Res. Arch.

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Beta thalassaemia is one of the most common autosomal recessive diseases in the world. We present the clinical cases of four patients presenting with beta thalassaemia, of which only two cases confirmed. The aim of this case reports is to draw the clinician’s attention to the diagnosis of beta thalassaemia and to highlight the importance of a good interpretation of a haemoglobin electrophoresis and to clarify the difficulties in the management of beta thalassaemia in our context.

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Prise en charge thérapeutique des patients atteints de bêta-thalassémie majeure dans un service de pédiatrie du sud tunisien : à propos de 26 cas

Cited by 4 publications

References 14 publications

Incidence of Dilated Cardiomyopathy in Patients of Thalassemia Major Receiving Deferasirox and Deferoxamine as Chelators

Incidence of Dilated Cardiomyopathy in Patients of Thalassemia Major Receiving Deferasirox and Deferoxamine as Chelators

Beta-thalassaemia: Four cases at the mother and child centre of the Chantal Biya Foundation

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