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This repository contains the published and unpublished research of the Faculty of Medicine by the staff members of the faculty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 An islandwide hospital based epidemiological survey of haemoglobinopathies and an assessment of standards of care in 23 centres(Sri Lanka Medical Association, 2017) Premawardhena, A.P.; Mudiyanse, R.M.; Jifri, M.N.; Nelumdeniya, U.B.P.; Silva, D.P.S.I.; Nizri, A.H.M.; Rajiyah, M.O.F.; de Silva, T.U.N.; Pushpakumara, K.R.C.; Dissanayake, D.M.R.; Jansz, M.; Rifaya, M.I.; Navarathne, N.M.U.S.B.; Arambepola, W.W.M.M.; Thirukumaran, V.; Mendis, D.; Weerasekara, K.P.; de Silva, N.N.; Vaidyanatha, U.S.de.S.; Mettananda, D.S.G.; Oliveri, N.F.; Weatherall, D.J.INTRODUCTION & OBJECTIVES: There is no database for haemoglobinopathy patients in Sri Lanka resulting in imprecise knowledge about the distribution and standards of care among patients managed in different centres in the island. The prevention programme commenced in 2007 is not centrally monitored. METHODS: We carried out centre-wise visitation and data gathering of patients with haemoglobinopathies in 2015. 23 centres were visited by researchers who gathered information from patient records. RESULTS: Data was obtained of 1768 patients. Three centres had over 200 patients each and another three centres had between 50-100 patients. There were 8 centres with less than 10 patients each. Beta thalassaemia major (BTM) accounted for 1207 (68.26%) patients. There were 363 patients (20.53%) with Haemoglobin E-β thalassaemia. Sickle cell-β thalassaemia accounted for 51 patients (2.88%). The mean age of BTM patients was 13 years (range 2-44). Ethnic distribution of the haemoglobinopathies (82.5% Sinhalese, 12% Muslims and 5.2% in Tamils) was discrepant to the national ethnic data. Islandwide mean number of new births of all thalassaemics recorded showed a reduction from 66/year between 2004 - 2009 to 48/year between 2010 to 2015.Clinical record keeping was not systematic in most units thus complication rates were hard to obtain. Death data were available only in two units. CONCLUSION: This study identified significant inconsistencies in haemoglobinopathy care between centres. Existence of small centres needs to be recognized by the Ministry of Health. A reducing trend of new births over the last decade was observed.Item “Silent” white matter changes in brain MRI in patients with haemoglobinopathies and their clinical significance(Sri Lanka Medical Association, 2016) Premawardhena, A.P.; Ranawaka, U.R.; Hapangama, A.; Pathmeswaran, A.; Hettiarachchi, S.; Salvin, K.A.; Pilapitiya, T.; Sanjaya, G.; Oilvieri, N.F.; Weatherall, D.J.INTRODUCTION AND OBJECTIVES: Increasing interest has been directed to the study of white matter changes and lacunar infarcts in patients with haemoglobinopathies which are thought to be clinically silent. Previous studies suggest an association with splenectomy and thrombocytosis. The objective was to assess the association of white matter changes in patients with haemoglobinopathies. METHOD: Older patients with haemoglobinopthies attending the Hemals Thalassaemia Unit were assessed by a specialist neurologist and simultaneously had MRI brain. Relevant clinical, biochemical and hematological data were collected. A non-thalassaemic control group (age and sex matched) too were assessed. RESULTS: There were 82 patients (25 Thalassaemia Major,24 Intermedia and 33 Haemoglobin E b thalassaemia) and 19 controls. Mean age of the study group was 32yrs. 21 patients (26%) had MRI changes (20%,29%,27% in the three groups respectively) (p=0.73). 10% of controls had MRI changes. 12 (60%) of the patients with MRI changes were asplenic whilst 53.2% without changes were asplenic (P=0.138). There was no difference in the two groups with platelet counts. (Mean platelet count 581 Vs. 452 (p= 0.709) 58% of patients and 53% of controls had headache as a symptom. Headache was commoner among those with MRI changes (85% vs 51.6%; p=0.008). CONCLUSIONS: The white matter changes in MRI occurred in all three sub groups of thalassaemic patients studied in high frequency. To our knowledge, this is the first report of the association of MRI changes with neurological symptoms in thalassaemic patients. The higher frequency of headache in those with MRI changes suggest that these changes may not be silent as previously believed.