Conference Papers
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This collection contains abstracts of conference papers, presented at local and international conferences by the staff of the Faculty of Medicine
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Item Symptoms and signs of β thalassaemia trait: results of the first comparative study(Sri Lanka Medical Association, 2007) Premawardhena, A.P.; Weerasinghe, M.; Kottachchi, D.; Arambepola, M.; Katugaha, N.; Samarakoon, S.; Otivieri, N.F.; Weatherall, D.J.OBJECTIVE: Thalassaemia trait is considered a mild and asymptomatic condition. However patients with this disease may be symptomatic. Should these be attributed to the disease? Does pre-knowledge about the diagnosis affect patients' symptoms? Previous studies were affected by the lack of a control arm. This is the first controlled study which compares symptoms of (3 thalassaemia trait with matched controls and also looks at how prior knowledge of the disease affects symptoms. DESIGN, SETTING AND METHODS: We administered a questionnaire to 146 individuals who knew that they had thalasseamia trait (Group 1} and to 248 "normal" volunteers who did not know their "thalassaemic status". Eleven symptoms were assessed. All individuals were examined by the same investigator and had a full blood count from an automated analyzer and a thalassaemia screening with High Performance Liquid Chromatography (HPLC- Bio Rad). RESULTS: The FBC and HPLC data showed that of the 248 "normal" controls , 63 had p thalassaemia trait (Group 2), and a further 96 had MCV< 80 and MCH <27 without thalassaemia (Group 3), presumably due to iron deficiency. 89 had normal red cell indices and normal HPLC (Group 4). Comparison of the four groups showed that "anaemic symptoms" like headache, exercise intolerance and lethargy occurred in significantly higher numbers in Groups 1 and 2 compared to Group 4. Comparison of Group 1 and Group 3 did not show any significant difference suggesting a similar mechanism for symptoms. Group 2 did not differ significantly from Group 1 in anyway. CONCLUSIONS: The p thalasseamia trait can be a symptomatic disorder and the symptoms arc those of anaemia. Symptoms are not affected or caused by being aware of the diagnosis.Item Cardiac functions in older patients with haemoglobin E- β thalassaemia(Sri Lanka Medical Association, 2007) Premawardhena, A.P.; Wanninayake, S.; Dolapihilla, S.N.; Kapuruge, L.; Katugaha, N.; Olivieri, N.F.; Weatherall, D.J.INTRODUCTION: Cardiac disease accounts for most deaths in patients with thalassaemia. Little is known about cardiac functions in ageing patients with thalassaemia OBJECTIVE: To study cardiac functions in older patients with haemoglobin E - p thalassaemia. DESIGN, SETTING AND METHODS: All patients with haemoglobin E - p thaiassaemia over the age of 24 attending the Thalassaemia Centre at Kurunegala were studied. Data were collected on biographs, transfusion load, body iron loads, ischaemic heart disease risk factors and symptoms and signs of heart disease. Serum lipids, 2D-echo and exercise ECG in addition to basic biochemical investigations were assessed in all. Those with a positive exercise ECG underwent stress echocardiography. Data from age-sex matched "healthy" individuals from the same geographic area were used for comparison. RESULTS: Total of 26 patients and 26 controls were studied. The median age was 34.25 years (24-50). Fourteen females were present in either group. 24% of controls, but-none in thalassaemic families had a family history of ischaemic heart disease. Twelve patients with thalassaemia and one control had a positive exercise ECG. All 12 with positive exercise ECG had normal stress echocardiography. Severe hypocholesterolaemia (mean total cholesterol 90.5 vs 376.9mg/dl) was present in all patients with thalassaemia. Pulmonary hypertension was noted in 7 patients with thalassaemia (not in controls). Nine patients with thalassaemia but none of the controls showed diastolic dysfunction. CONCLUSION: The study shows unique cardiac abnormalities that occur in patients with haemoglobin E- β thalassaemia. The marked hypocholesterolaemia may reduce the risk of ischaemic heart disease but significant right heart damage seems to occur in these chronically anaemic patients.