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This repository contains the published and unpublished research of the Faculty of Medicine by the staff members of the faculty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 Parental awareness and cascade screening of thalassaemia(Sri Lanka Medical Association, 2010) Nishad, A.A.N.; Premawardhena, A.P.INTRODUCTION: A strategy to prevent new births with thalassaemia major in countries where abortion is illegal is to screen the at risk population for the carrier state and advocating "safe marriages". One of the highest risk groups for the thalassaemia gene are relatives of individuals with a child with 'thalassaemia major'. 'Cascade screening' of such relatives is an effective way to initiate and precede a much wider population screen. We wanted to identify the level of awareness of disease transmission and the extent to which cascade screening was already happening in Sri Lanka. METHODS: A self-administered questionnaire was used on 100 parents of children with transfusion dependent thalassaemia attending the thalassaemia units at Ragama and Kurunegala from 1st January to 31st December 2009. RESULTS: 73% fathers and 91% mothers knew their carrier state and 59 (64.1%) fathers and 98 (88.1%) mothers knew their carrier state bad caused the illness to the child. Mothers had 382 and fathers had 291 siblings. Those siblings had 714 and 610 children respectively. 40 (10.8%) of maternal siblings and 15 (5%) of the paternal siblings had been screened for thalassaemia. Only 48 (6.72%) of maternal side children and 23 (3.77%) of paternal side children had been screened. CONCLUSION: Screening of relatives of patients with thalassaemia in this group of people seems to be limited. A screening programme targeting those at high risk is necessary.Item Pulmonary arterial hypertension in thalassaemia patients- does splenectomy and disease severity increase the risk?(Sri Lanka Medical Association, 2011) Premawardana, N.P.; Nandasiri, A.S.D.; Ranaweera, A.G.R.M.A.; Nishad, A.A.N.; Silva, D.P.S.I.; Premawardhena, A.P.INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension (PAH) has been described in patients with thalassaemia. A causative association with splenectomy has been postulated. Our previous observations differed from this. We aimed to study these factors in our patients. METHODS: Pulmonary artery pressure (PAP) and other data of thaiassaemics attending the Thalassaemia Unit, Ragarna were studied using clinic records. PAP was measured using trans-thoracic 2D echocardiography. Four categories were separately analysed: thalassaemia major (TM) with splenectomy (A) and without (B), thalassaemia intermedia (TI) with splenectomy (C) and without (D). PAH was defined as PAP over 25 mm Hg. RESULTS: A total of 74 patients were studied, 60 (81%) with TM and 14 (19%) with TI. 25 of TMs (41%) and 10 of TIs (71%) had splenectomy (p <0.05). Mean ages (SD) of TM and TI were 20.71(8.4) and 32.6 (13.3) years respectively. Those of categories ABCD were 20.9 (7.4), 20.7 (9.1), 27 (9.6) and 46 (12.3) years. A total of 16 (21.6%) of all patients had PAH (95% CI13.8-32.3). This included 13 (21.7%) TM patients and three (21.4%) with TI (p-0.98). The prevalence of PAH was 17.1% (95 CI 8.4-33.5) in splenectomised thalassaemia patients and 25.6% (95% CI 16.5-43.8) in non splenectomised patients (p= 0.37), and in categories A,B,C,D were 16%, 23%, 20% and 25% respectively. Mean of PAP of splenectomised with PAH is 39 mmHg (SD=4.5) and in non splenectomised with PAH was 34.9 mmHg (SD=5.3) (p=0.8). CONCLUSIONS: PAH is a significant complication in patients with thalassaemia. However the severity of disease nor the splenectomy status were found to be causatively linked in our study.