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 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 Techniques for primary screening for haemoglobinpathies in Sri Lanka: a comparison of single tube osmotic fragility (STOP) and dye test {DCIP) vs full blood count (FBC)(Sri Lanka Medical Association, 2007) Premawardhena, A.P.; Williams, S.; Idirisinghe, A.; Allen, A.; Olivieri, N.F.; Weatherall, D.J.INTRODUCTION: Techniques best suited for haemoglobinopathy screening of the Sri Lankan population need to be determined. Full blood count is used to detect p thalassaemia trait but is affected by iron deficiency. Its ability to detect haemoglobin (Hb) E is less certain. Alternative tests for screening include single tube osmotic fragility (STOP) test (for [5 thalassaemia) and a dye test (DCIP) for HbE. However there is no consensus regarding their usage in population surveys. OBJECTIVES: To decide the validity of STOP and DCIP test as screening tests for haemoglobin disorders in Sri Lanka. DESIGN, SETTING AND METHODS: Blood samples of volunteers attending an educational campaign held at the Faculty of Medicine, Ragama were tested using STOP, DCIP and FBC, and the results compared with the "gold standard" HPLC (high performance liquid chromatography) Results: 1062 individuals participated. HPLC identified 27 p traits and 3 HbE carriers. A total of 508 subjects had either MCV < 80 or MCH <27 on FBC, which included 26 of the p traits and all with HbE. The rest were presumably iron deficient. Only 56 subjects had a positive STOF test including 26 with p trait. 34 had a positive DCIP test including all three HbE carriers. One person with HbA2 of 3.5% had a normal MCV, MCH, negative STOF and negative DCIP. STOF test had a sensitivity and specificity of 96% and 97% for p trait. The DCIP had a sensitivity and specificity of 100% and for the detection of Hb E. CONCLUSIONS: Both STOF and DCIP are excellent tests for primary screening. The STOF is a superior test for screening thalassaemias than FBC in populations with high prevalence of iron deficiency.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.