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Thalassaemia Major Patients (thalassaemia + major_patient)

Distribution by Scientific Domains

Medical Sciences	100%

Selected Abstracts

Correlation of echocardiography parameters with cardiac magnetic resonance imaging in transfusion-dependent thalassaemia major

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 1 2007
Athanassios Aessopos
Abstract Background and objective:,Heart iron load (cardiac Fe) can be indirectly quantified by cardiac magnetic resonance (CMR) T2*. CMR accessibility is limited, whereas echocardiography (Echo) is relatively inexpensive and readily available. The objective was to find Echo parameters that may be useful for predicting cardiac Fe. Design and methods:,We compared a number of parameters derived from Echo to cardiac Fe in 142 thalassaemia major patients who had undergone a CMR study. Results:,All patients with decreased left ventricular (LV) function had cardiac Fe. After removing those patients from the analysis, the total diameter index (Tdi) >5.57 cms/m2, left atrial diameter index >2.41 cm/m2, and the diastolic parameter E/A > 1.96 were highly specific (91.4%, 97.1% and 96.9% respectively) but had low sensitivity (31.8%, 20.45% and 21.8%) in predicting iron load. A right ventricular index >1.47 cm/m2, LV systolic index >2.26 cm/m2 or Tdi >6.26 cm/m2 discriminated between patients with no, or mild to moderate cardiac Fe from those with heavy load, with specificity of 91%, 98.5%, and 98.5%, respectively, but with low sensitivity. Interpretation and conclusions:,Echo parameters for cardiac Fe prediction have restricted value, whereas CMR is essential to assess cardiac Fe. However, patients with decreased LV systolic function should be considered a priori as having cardiac Fe, and chelation therapy should be intensified. This also applies to patients who have the above-described Echo criterion values, even if CMR is not available. Once a patient is found by CMR to have cardiac Fe, then the above Echo criterion values may be useful for ongoing monitoring. [source]

Pseudoxanthoma elasticum-like lesions in association with thalassaemia major

AUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 3 2009
Shirley Yu
ABSTRACT A 33-year-old woman with a background of thalassaemia major presented with a 3-month history of yellowish plaques on the back of her neck bilaterally and alterations in the texture of the skin in both axillae. Examination of these lesions showed yellowish cobblestone plaques and coalescing papules in both axillae. Pseudoxanthoma elasticum (PXE)-like lesions were confirmed histopathologically. PXE-like lesions in association with thalassaemia major are an uncommon occurrence. Due to the increase in the survival rate of thalassaemia major patients on treatment, the development of these lesions is likely to increase. The histopathological manifestation of these lesions mimics that of hereditary PXE and is associated with severe vascular complications. This case highlights the importance of recognising this rare disorder and the role dermatologists have in establishing the diagnosis and advising on appropriate screening for complications. [source]

Noninvasive assessment of liver fibrosis in thalassaemia major patients by transient elastography (TE) , lack of interference by iron deposition

BRITISH JOURNAL OF HAEMATOLOGY, Issue 3 2010
Vito Di Marco
Summary The correlation between liver stiffness, measured by transient elastography, liver fibrosis, using the histological METAVIR score, and iron overload, measured by atomic absorption spectrometry was evaluated in 56 homozygous-,-thalassaemics. Liver stiffness increased proportionally to liver fibrosis staging (r = 0·70; P > 0·001) independently of liver iron concentration (r = 0·01; P = 0·932). The area under the receiver-operating characteristic curve for prediction of cirrhosis was 0·997 (95% confidence interval [CI]: 0·925,1·000) with cut-off of 13 kPa with 100% sensitivity (95% CI: 69·0,100·0) and 95% specificity (95% CI: 84·2,99·3). Transient elastography is a reliable non-invasive tool for diagnosing advanced liver fibrosis in homozygous-,-thalassaemics, regardless of the degree of iron overload. [source]

Exercise capacity and cardiovascular changes in patients with ,-thalassaemia major

CLINICAL PHYSIOLOGY AND FUNCTIONAL IMAGING, Issue 6 2006
Filippo Tocco
Summary Despite the introduction of deferoxamine, 50% of thalassaemia major patients die before the age of 35 years predominantly from iron induced heart failure. Indeed, the assessment of myocardial performance may be of particular interest since it can reveal an early myocardial dysfunction. By using impedance cardiography and mass spectrometry, we studied the cardiac function and the oxygen extraction ratio (O2ER) of 14 thalassaemic patients and 15 control healthy subjects during an incremental cycle-ergometer test. The achieved mechanical power output and the relative O2 uptake did not reach any significant difference between groups. At the highest workload, O2ER reached significantly higher values in thalassaemic patients versus control subjects while the relationship between cardiac index (CI) and O2ER (CI/O2ER) decreased showing a lower contribution of cardiovascular system to maintain O2 uptake. Results of this study imply that CI/O2ER allows an early diagnosis of the iron induced myocardial dysfunction, whereas it is not clinically patent yet. To our knowledge, this is the first study revealing an O2ER pivotal role as compensatory mechanism to maintain a normal working capacity in subjects suffering from thalassaemia major. [source]