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This repository contains the published and unpublished research of the Faculty of Medicine by the staff members of the faculty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 Assessing liver fibrosis in patients with transfusion dependent beta thalassaemia - a predictive model(Sri Lanka Medical Association, 2021) Padeniya, A.G.P.M.; Ediriweera, D.; de Silva, A.; Niriella, M.A.; Premawardhena, A.P.Introduction and Objectives Liver fibrosis in β-thalassaemia major is mainly due to transfusion-related iron overload. Transient elastography (TE) is an imaging modality which measures liver stiffness/fibrosis non-invasively. TE is simple, safe and efficient. However, inaccessibility and high-cost hinders its routine use. We designed a predictive model to evaluate liver fibrosis using demographic, anthropometric, biochemical and imaging data. Methods Sixteen patients with transfusion dependent beta thalassaemia were recruited to the study. FBC, LFT, serum ferritin and Transient Elastography (TE) and FerriScan measurements were recorded at the baseline and after two years follow up. Multiple regression model was developed to predict liver fibrosis using demographic, anthropometric, biochemical and imaging data. [age, gender, body mass index (BMI), steatosis score, liver iron content, mean pre-Hb over the last year, no of blood transfusions (lifetime), amount of blood ingested over the last year(ml/kg), amount of elemental iron by transfusions over last year(mg/ kg), serum ferritin, SGOT, SGPT and compliance with iron chelation].Results Of 16, 8 (50%) were females, mean (SD) age, BMI and fibrosis scores were 21(4.3) years, 18.8 (2.8) kgm-2 and 9.7(5.7) kPa respectively. Gender, BMI, SGOT, SGPT, compliance, number of transfusions taken lifetime showed significant association with liver fibrosis. The final model showed a coefficient of determination (R2) of 0.859. According to the model, predicted liver fibrosis is given by;-26.18 - 4.38*male+1.01*BMI - 0.11*SGPT+0.32*SGOT+2.78*compliance (rps)+0.04*no. of transfusions. ConclusionThe suggested model is a reliable tool to predict liver fibrosis in transfusion-dependent β-thalassaemia major patients in resource poor settings.Item 20 year follow up and survival analysis in a cohort of patients with Haemoglobin E beta Thalassaemia.(Sri Lanka Medical Association., 2019) Olivieri, N.F.; Premawardhena, A.P.; Amir-Arsalan, S.; Ediriweera, D.; Mettananda, S.; Bandara, W.D.; Arambepola, M.; de Silva, S.; Refai, M.A.C.M.; Allen, A.INTRODUCTION & OBJECTIVES: Haemoglobin E beta thalassaemia (EBT) is the commonest beta thalassaemia syndrome in the world and is extremely phenotypically variable. Unlike for transfusion dependent thalassaemia (TDT) there are no clear guidelines for the management of this disease. We have followed up a cohort of 109 patients with EBT for 20 years. Objective of the study was to study the 20-year survival and factors that affect survival. METHODS: Study was conducted at Kurunegala Thalassaemia centre. Transfusions were stopped in 1997 in all 109 patients. Since then they were assessed every three months by the clinical team for the next 20 years. Relevant haematological, biochemical, radiological assessments were done periodically. RESULTS: 32 (30%) of patients were dead at 20 years. Kaplan Meir survival curve identified the median survival to be 51 years. Splenectomy had been done in 73/109 (67%) patients. Splenectomy allowed 66% to be off transfusions even 9.7± 1.3 years post- surgery. However, 33% had to return to transfusions. The commonest cause of death in the cohort was infections (34.3%). Most (72%) infective deaths happened in those who were splenectomised. Transfusions needed to be restarted in 60%, of whom 33% went back to (>8 per year) regular transfusions at a mean 8.4 ±0.8 years after stopping transfusions. CONCLUSION: In this first ever long term follow up study of EBT, significantly shortened survival is observed. Though splenectomy allows prolonged transfusion free phases in many it increases risk of infective deaths. Overall the disease is far less benign than previously thought with a high prevalence of morbidity and mortality.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.