Medicine
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This repository contains the published and unpublished research of the Faculty of Medicine by the staff members of the faculty
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Item Deferoxamine, deferasirox, and deferiprone triple iron chelator combination therapy for transfusion-dependent β-thalassaemia with very high iron overload: a randomised clinical trial(Elsevier Ltd, 2024-10) Premawardhena, A.; Wanasinghe, S.; Perera, C.; Wijethilaka, M.N.; Rajakaruna, R.H.M.G.; Samarasinghe, R.A.N.K.K.; Williams, S.; Mettananda, S.BACKGROUND Many patients with β-thalassaemia die prematurely due to iron overload. In this study, we aim to evaluate the efficacy and safety of the triple combination of deferoxamine, deferasirox and deferiprone on iron chelation in patients with transfusion-dependent β-thalassaemia with very high iron overload.METHODS This open-label, randomised, controlled clinical trial was conducted at Colombo North Teaching Hospital, Sri Lanka. Transfusion-dependent β-thalassaemia patients with ferritin >3500 ng/mL were randomised 2:1 into intervention (deferoxamine, deferasirox and deferiprone) and control (deferoxamine and deferasirox) arms. Reduction in serum ferritin after six months was the primary outcome measure. Reduction in liver iron content, improvement in cardiac T2∗, and adverse effects were secondary outcome measures.FINDINGS Twenty-three patients (intervention-15, control-8) were recruited. 92% and 62% in the intervention and control arms showed a reduction in ferritin, respectively. The mean reduction of ferritin was significantly higher in intervention (−1094 ± 907 ng/mL) compared to control (+82 ± 1588 ng/mL) arm (p = 0.042). There was no statistically significant difference in the liver iron content in two arms. In the intervention arm, 67% improved cardiac T2∗ (mean change +6.72 ± 9.63 ms) compared to 20% in the control arm (mean change −3.00 ± 8.24 ms). Five patients discontinued deferiprone due to arthralgia, which resolved completely after stopping the drug.INTERPRETATION Triple combination therapy with deferoxamine, deferasirox and deferiprone is more efficacious in reducing iron burden measured by serum ferritin and showed a positive trend in reducing myocardial iron content in patients with transfusion-dependent β-thalassaemia with very high iron overload. Deferiprone has the disturbing side effect of reversible but severe arthropathy.Item Morbidity and mortality patterns in patients with thalassaemia during the COVID-19 pandemic in Sri Lanka;A single centre experience(Sri Lanka College of Internal Medicine, 2022) Nisansala, R.; de Silva, S.; Ediriweera, D.; Premawardhena, A.Introduction: Patients with thalassaemia syndromes (TS) affected with COVID-19 attending a thalassaemia centre in Sri Lanka situated in the region most affected with COVID-19 were studied over a 16-month period. Methods: To assess the collateral effects on overall thalassaemia care in the centre, data on transfusion, chelation and clinic attendance were analysed. Morbidity events and deaths recorded during the COVID-19 period and during a similar period before the beginning of COVID-19 infection in Sri Lanka were recorded in all clinic registrants. Results: Seven patients (of 502) with TS had developed COVID-19 during the 16-month period; all were minimally symptomatic and had recovered without complications. Number of monthly clinic visits reduced from 338 pre-COVID to 268 during COVID (p=0.004). Iron chelator usage too reduced during the pandemic period (p<0.001). Though admissions related to morbidity reduced during the pandemic (58 vs 16, p<0.001) there were more non-COVID deaths (8 vs 4). Conclusions: Numbers affected with COVID-19 were low and severity of infection was mild in this cohort of patients with TS. Collateral effect on the management of the unit and effects on mortality in this vulnerable population appears to have been substantial.Item Quality of life and psychological morbidity among children with transfusion dependent thalassaemia and their parents(Sri Lanka Medical Association, 2018) Mettananda, S.; Pathiraja, H.; Peiris, R.; Bandara, D.; de Silva, T.U.N.; Mettananda, K.C.D.; Premawardhena, A.INTRODUCTION AND OBJECTIVES: Thalassaemia is a chronic illness which requires life-long supportive care. We aim to describe quality of life and psychological morbidity among children with transfusion-dependent thalassaemia and their parents.METHODS: This case-control study was conducted from September to December 2017. All patients with transfusion-dependent thalassaemia aged 2-18 years attending three largest thalassemia centers of Sri Lanka (Kurunegala, Anuradhapura and Ragama) were recruited as cases Children without chronic diseases admitting to same hospitals for acute non-life threatening illnesses were recruited as controls. Data were collected using an interviewer-administered questionnaire with validated questionnaires to measure quality of life (PedsQL 4.0 Generic core scales) and psychological morbidity (Strengths and difficulties questionnaire) of children and depressive symptoms of parents (Centre for epidemiological studies depression scale). Ethical approval was obtained from Ethics Committee of University of Kelaniya. RESULTS: 321 (male-46.4%; mean age-9 9±4 2years) cases and 194 (male-47.4% mean age- 8.9±3 7years) controls were recruited Mean quality of life score was significantly lower in cases (72.6±12.0%) compared to controls (88.6±11.6%), (p<0 001) Prevalence of psychological issues were significantly higher among patients with thalassaemia compared to controls in emotional (17 6% vs 0%; 2=32.8, p<0 001), conduct (18.6% vs 0 6%;2=35.4, p<0 001), hyperactive (9 5% vs 0%; 2=18 9 p<0.001) and peer relationship (14.4% vs 2 5%; 2=15 7 p<0 001) domains Significantly higher proportion of mothers of cases reported depressive symptoms compared to controls (40 1% vs 8.9%; 2=57 0 p<0.001).CONCLUSION: Patients with transfusion-dependent thalassaemia experience a poor quality of life and are victims of psychological and behavioural problems compared to their peers. Similarly, mothers of children with thalassaemia have higher prevalence of depression Psychological support should be an essential component in management of thalassaemiaItem How Young adults with Thalassaemia major feel and what they want?(Sri Lanka Medical Association, 2012) Nishad, A.A.N.; Premawardhena, A.INTRODUCTION: During last few decades with introduction of regular blood transfusion and effective iron chelating therapy, life expectancy of Thalassaemia major patients has improved. There is little data regarding quality of life of these patients. METHODS: Prospective quantitative and qualitative study was carried out at Ragama and Kurunegala with transfusion dependent patients of 16 years or older during 2011. SF-36, Becks depression inventory, GHQ-30 and in-depth interviews were used. RESULTS: Of 37 patients (19 females) 64% had at least grade 11 education. Median age was 20(25ch=17, 75th=22] years. Forty percent girls and 33% of boys told illness had a negative impact on romantic relationships. Forty four percent boys, 10.5% girls wanted to marry. Thirty three percent boys and 5% girls wanted to have children. Seventy percent girls and 56% boys wanted to engage in an occupation. SF-36 (Cronbach alpha=0.88) analysis, found that 66% thought they get sick easily and 70% thought they were not healthy. But 70% thought their health would not get worse. Limitation of activities was seen (highly active=64%, moderately active=18%). But 81% did not limit routines and 61% had no routine work difficulty. Seventy seven percent had good life satisfaction although 39% felt a great tiredness. Becks depression inventory reported 31% (95%CI=19-48J had reduced sleep and 26% (95%CI=14-42) had disturbed sleep. Mild depression was seen in 5.4%. CONCLUSIONS: Patients with thalassaemia want to live a normal life like healthy people. They seem to have a satisfactory quality of life.