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 Impact of acalypha indica (Kuppamenia) phytochemicals on glucose-6-phosphate dehydrogenase deficiency: two clinical case studies(College of Chemical Pathologists of Sri Lanka, 2024) Fernando, K.; Fernando, N.; Dayanath, B.K.T.P.; Williams, S.; Premawardhena, A.INTRODUCTION Acalypha indica, known as “kuppamenia” in Sinhala, plays a significant role in ayurvedic medicine in Sri Lanka. Despite its medicinal use, certain phytochemicals within the plant have been linked to oxidative stress-induced haemolysis in individuals deficient in glucose-6-phosphate dehydrogenase (G6PD) enzyme activity. However, we know very little regarding those chemicals due to a dearth of literature. Here, we present two cases highlighting the impact of Acalypha indica ingestion on individuals with G6PD deficiency.CASE PRESENTATION Case 1: A 65-year-old man presented with jaundice, pallor, and haematuria after consuming “Kuppamenia mallum”. His peripheral blood picture showed bite cells and Heinz bodies. Positive Brewer’s test raised the clinical suspicion of G6PD deficiency. He received blood transfusions during the acute phase and was discharged upon improvement of symptoms. Low levels of G6PD enzyme at the follow-up visit confirmed the diagnosis. Case 2: A 61-year-old male presented with respiratory distress, jaundice and haematuria, accompanied by peripheral cyanosis and hypotension. He was admitted following four days of continuous ingestion of Acalypha indica. Peripheral oxygen saturation was low, and Heinz bodies were evident in the peripheral blood film. Blotting paper methaemoglobinaemia assessment revealed a level of 70%. Despite optimal medical management, he succumbed to a cardiac arrest on the fourth day of admission.DISCUSSION AND CONCLUSIONS Despite its antioxidant properties, Acalypha indica can precipitate acute haemolysis in G6PD-deficient individuals. Symptomatology and outcomes vary with the amount ingested, underscoring the necessity for research into identifying potentially toxic phytochemicals and their concentrations.Item Efficacy of hydroxyurea in reducing the erythropoietic stress of ineffective erythropoiesis in transfusion dependent beta thalassaemia: A randomised, double-blind placebo-controlled clinical trial(Sri Lanka Association for the Advancement of Science, 2021) Yasara, N.; Premawardhena, A.; Perera, P.; Manamperi, A.; Mettananda, S.The unbalanced synthesis and accumulation of a-globin chains due to impaired synthesis of 0- globin results in the destruction of red blood cells (RBC) and erythroid precursors of patients with P-thalassaemia. This leads to an increased erythropoietic activity and ineffective erythropoiesis in the bone marrow of these patients. Hydroxyurea is a licenced medication that decreases the RBC destruction of patients with p-thalassaemia. However, its effect on erythropoietic stress is unclear. In this study, our objective was to evaluate the effect of hydroxyurea on erythropoietic stress of ineffective erythropoiesis in transfusion-dependent (TD) p-thalassaemia. This experimental study was carried out at the Thalassaemia Unit of Colombo North Teaching Hospital as part of a randomised, double-blind placebo-controlled clinical trial that evaluates the efficacy of hydroxyurea. We recruited 24 patients with TD p-thalassaemia who were taking hydroxyurea IQ- 20 mg/kg/day and 16 patients who were receiving a placebo. The erythropoietic stress of ineffective erythropoiesis was assessed by measuring serum soluble transferrin receptor (sTfR) levels before and six months after taking either hydroxyurea or placebo. Levels of sTfR were measured using a validated enzyme-linked immunosorbent assay. Paired t-test was used in the statistical analysis. Nineteen (79%) out of 24 patients who received hydroxyurea showed a reduction in sTfR level, of which 8 (33%) and 6 (25%) showed >25% and 10-25% reductions, respectively. A significant reduction in mean sTfR level was observed after hydroxyurea treatment (72.3±SD25.9) compared to pre-treatment levels (89.6士SD22.9), (pv0.01). Conversely, no difference in sTfR levels was seen in patients who received the placebo pre・(91.9土SD24.7) and post-treatment (96.4±SD19.4), (p=0.17). In conclusion, oral hydroxyurea significantly reduced the erythropoietic stress of ineffective erythropoiesis in patients with TD p-thalassaemia showing a promise as a treatment modality.Item Elective cholecystectomy is associated with increased morbidity and mortality in patients with severe Thalassemia: A retrospective case control study.(Ferrata Storti Foundation, 2015) Premawardhena, A.; Fernando, R.; Kumarage, S.; Nishad, N.; de Silva, I.BACKGROUND: Haemoglobin disorders including thalassemia and sickle cell disease are often complicated with gall stone formation. The co-existence of Gilbert's syndrome together with these diseases further increases the risk of gall bladder disease. Some of these patients develop symptomatic disease which necessitates surgical intervention. At present the timing of cholecystectomy for thalassemia is no different from that of the general population with the exception of removal of the gall bladder at the time of splenectomy. This is no longer the case in sickle cell disease where, laparoscopic cholecystectomy is recommended even in asymptomatic patients. This practice however has not been extended to other types of haemoglobin disorders. AIM(S): 1.To assess the perioperative complications of patients with thalassaemia during cholecytetomy and to compare it with non thalassaemics who undergo the procedure. 2. To see if there is enough evidence to recommend elective cholecystectomy for thalassaemics. METHOD(S): We retrospectively studied case notes of thalassemia patients who had cholecystectomy (cases) in two of the biggest thalassaemia centres in Sri Lanka and also of 62 non-thalassaemics (controls) with gall bladder disease who had been scheduled to have gall bladder surgery in the same hospitals and looked at their peri-operative complications. RESULT(S): 98 out of 540 (18%) thalassaemics in the two centres had gall stones. Mean age of cases was 26.8 (SD 10.9) years and of controls 47.5 (SD 19.7) years. 19 (19%) thalassaemics with gall stones had undergone cholecystectomy. Ten patients had cholecystectomy simultaneously with splenectomy. The majority of non-thalassaemic "controls" had laparoscopic cholecystectomy 53/55 (96.3%) whilst the patients with thalassaemia were mostly operated with laparotomy 13/19 (68%). There was a significant excess complications occurring in both early (42.11 vs. 18.1%) and late (31.5 vs. 12.7%) phases in the thalassaemic patients compared with the controls. Among the early complications, sepsis (10.5% vs. 1.8%) and liver abscess formation (5.2 vs. 0%) was significantly different in the groups, adversely affecting the thalassaemics. Recurrent abdominal pain was more common among the thalassaemics as a late complication (P<0.05). Six thalassaemic patients with gall stone disease died during this study, 5(5%) while awaiting surgery and 1(1%) after surgery. There were no deaths among the controls. Out of the deaths, 3 (50%) were directly attributable to gallstone disease. In all three septicemia precipitated heart failure. We found a significant increase of both early and late post-surgical complications in the thalassemia group and also increased mortality most of which was related to severe sepsis. Higher perioperative mortality and morbidity were seen among symptomatic thalassaemic patients with gall stone disease undergoing cholecystectomy. This seems to suggest a strong case for supporting elective cholecystectomy in thalassemics before they develop symptoms. SUMMARY AND CONCLUSION(S): We suggest that laparoscopic elective cholecystectomy be considered for non-sickle, thalassemia patients too who have asymptomatic gall bladder disease, in an attempt to reduce this morbidity and mortality.Item Oxidative stress and antioxidant deficiency in the β-thalassaemia in Sri Lanka(Sri Lanka Medical Association, 2021) Perera, S.; Allen, A.; Mettananda, S.; Rodrigo, R.; Perera, L.; Darshana, T.; Moggach, F.; Jackson-Crawford, A.; Heirene, L.; Fisher, C.; Olivieri, N.; Rees, D.; Allen, S.; Premawardhena, A.Introduction In the β thalassaemias oxidative stress is potentially high and this could be further exacerbated in the absence of robust antioxidant defences, such as nutritional vitamin C and E deficiencies. We undertook a comprehensive assessment of oxidant, antioxidant and inflammatory status in patients with subtypes of β-thalassaemia to study these factors in detail. Methods Consenting patients attending the thalassaemia centre in Ragama, (between November 2017 and June 2018) were assessed for the following: methaemoglobin, plasma haemoglobin, heme and ferritin as sources of oxidants, plasma haptoglobin, hemopexin and vitamins C and E as markers of antioxidants, plasma interleukin-6 and C-reactive protein for inflammation. Fruit and vegetable intake was determined by dietary recall. Results 162 patients were recruited. (59 HbE β-thalassaemia, 50 β-thalassaemia major, 40 β-thalassaemia intermedia, 13 HbS β-thalassaemia. Median age was 26.0 years (IQR 15.3-38.8), 101 (62.3%) were female. Oxidants were frequently increased and antioxidants depleted with high levels of oxidant damage, hypoxia and inflammation. Abnormalities were most severe in HbE-β thalassaemia and least severe in β thalassaemia intermedia. Oxidative stress was also more severe in splenectomised patients. Plasma vitamin C concentration was below the lower level of quantitation in 86/160 (53.8%) patients and vitamin E in 130/160 (81.3%) patients. Less than 15% of patients ate fresh fruits or raw vegetables frequently. Conclusion Markedly increased oxidative stress and antioxidant deficiency were observed in this study group, especially in those with HbE β-thalassaemia. Vitamin C & E supplementation may have a role in the long-term management of thalassaemia syndromes.Item Hydroxyurea for transfusion dependent β-thalassaemia: A randomized double-blind placebo-controlled clinical trial(Sri Lanka Medical Association, 2021) Yasara, N.; Wickramarathne, N.; Silva, I.; Hameed, N.; Attanayaka, A.M.K.R.; Jayasinghe, V.L.; Gunathilaka, P.A.C.K.; Wickramasinghe, N.; Rodrigo, R.; Perera, L; Perera, P.S.; Mettananda, K.C.D.; Manamperi, A.; Premawardhena, A.; Mettananda, S.Introduction and objectives Hydroxyurea induces fetal haemoglobin in vitro however, its clinical usefulness in β-thalassaemia is unclear. Here, we aim to assess the efficacy and safety of oral hydroxyurea in patients with transfusion dependent β-thalassaemia. Methods A phase 3 randomized double-blind placebo-controlled clinical trial was conducted at Colombo North Teaching Hospital in 2019/20. Sixty patients with transfusion dependent β-thalassaemia were randomized into hydroxyurea (10-20mg/kg/day) or placebo groups. Transfused blood volume, pre-transfusion haemoglobin, fetal haemoglobin and adverse effects were monitored during 6-month treatment and post-treatment periods. The study was approved by the ethics committee of University of Kelaniya and registered in Sri Lanka Clinical Trials Registry (SLCTR/2018/024). Results Fifty-four (hydroxyurea-27; placebo-27) patients completed the trial. Mean pre-transfusion haemoglobin (8.2±0.8g/ dLvs8.0±0.88g/dL, p=0.43) and fetal haemoglobin levels (7.9±11.2%vs4.6±4.3%, p=0.17) were higher in hydroxyurea group compared to placebo. Also, transfused blood volume was lower in the hydroxyurea group (94±29ml/kgvs102±28ml/kg, p=0.34). However, none were statistically significant. Based on elevation of fetal haemoglobin (>1.5% from baseline), we identified 12/27 patients who respond well to hydroxyurea (hydroxyurea-responders). Hydroxyurea-responders required significantly lower blood volume (77±27ml/kg) compared to non-responders (108±24ml/kg, p<0.01) and placebo group (102±28ml/kg, p<0.05). HbE β-thalassaemia sub-type (p<0.01) and Xmn1 polymorphism of γ-globin gene (p<0.05) were significant predictors of response to hydroxyurea. No serious side effects due to hydroxyurea were reported. Conclusion Over 40% of patients with transfusion dependent β-thalassaemia- specifically those with HbE β-thalassaemia and Xmn1 polymorphism of γ-globin gene- responded to hydroxyurea and required 25% less blood compared to controls. No serious adverse effects were reported following hydroxyurea treatment.Item Efficacy and Safety of Oral Hydroxyurea in Patients with Transfusion Dependent β Thalassaemia: a Randomized Double-Blind Placebo-Controlled Clinical Trial(Sri Lanka Medical Association, 2020) Yasara, N.; Wickramarathne, N.; Silva, I.; Hameed, N.; Attanayaka, A.M.K.R.; Jayasinghe, V.L.; Wickramasinghe, N.; Rodrigo, R.; Perera, L.; Mettananda, K.C.D.; Manamperi, A.; Premawardhena, A.; Mettananda, S.INTRODUCTION AND OBJECTIVES: Patients with β- thalassaemia require blood transfusions and iron chelation for life. Hydroxyurea is a licenced medication for sickle cell disease but its usefulness in transfusion dependent β-thalassaemia is unclear. Here, we aim to assess the efficacy and safety of oral hydroxyurea in patients with transfusion dependent β-thalassaemia. METHODS: A phase III randomized double-blind placebo-controlled clinical trial was conducted at Thalassaemia Unit of Colombo North Teaching Hospital in 2019. Forty-one patients with transfusion dependent β-thalassaemia were randomized into hydroxyurea (10-20mg/kg/day) or placebo (pharmaceutically inert capsule identical to hydroxyurea) groups. Transfused blood volume, pre-transfusion haemoglobin, haemoglobin F level and side effects were monitored monthly during 6- month treatment and 6-month follow-up periods. Adverse events were assessed by trained medical officers. The study was approved by ethics committee of University of Kelaniya and registered in Sri Lanka Clinical Trials Registry (SLCTR/ 2018/024). RESULTS: Of the 41 (hydroxyurea-20; placebo-21) patients, three discontinued treatment due to thrombocytopenia (hydroxyurea-2) and rash (placebo-1). Baseline characteristics of two groups were similar. Mean pre-transfusion haemoglobin (8.52+0.57 vs 8.38+0.55, p=0.45) and haemoglobin F levels (4.3+7.1% vs 3.1+1.9%, p=0.48) were higher in hydroxyurea group compared to placebo. Also, transfused blood volume was lower in hydroxyurea group (102+24ml/kg vs 111+27ml/kg, p=0.3). However, none were statistically significant. Based on elevation of haemoglobin F (>1.5% from baseline), we identified 6/18 patients as hydroxyurea responders. Hydroxyurea responders required significantly lower blood volume (87+13ml/kg) compared to non-responders (110+25ml/kg, p=0.05) and placebo group (111+27ml/kg, p<0.05) while maintaining higher pre-transfusion haemoglobin level (8.6+0.5 vs 8.4+0.5 and 8.3+0.5). No serious side effects were reported. CONCLUSIONS: One-third of patients with transfusion dependent β-thalassaemia responded to hydroxyurea treatment requiring 20% less blood compared to controls. No serious side effects were reported following hydroxyurea treatment.Item Identifying haemoglobinopathy traits and iron deficiency in Sri Lanka using the dtchlorophenolindophenol (DCIP) and one- tube osmotic fragility (OF) tests and measurement of red cell zinc protoporphyrin (ZPP)(Sri Lanka Medical Association., 2019) Perera, P. S.; Premawardhena, A.; Mettananda, S.; Rodrigo, R.; Perera, L.; Weatherall, D. J.; Allen, S.; Allen, A.INTRODUCTION & OBJECTIVES: Effective, low-cost, point-of-care tests are needed for haemoglobinopathy traits and iron deficiency throughout Southeast Asia. The objective of this study is to evaluate a combination of simple, low cost, easy-to-interpret and rapid tests as a "one-stop" screening procedure for identifying carriers of haemoglobin variants and iron deficiency in Sri Lanka. METHODS: Between November 2017 and May 2018, the KKU-OF and KKU modified DCIP tests (KKU, Thailand) were compared with High Performance Liquid Chromatography (HPLC) in a cross-sectional survey of 1324/1332 (99.4%) children attending secondary schools in Gampaha district, Sri Lanka. Iron deficiency was indicated in children with a raised red cell ZPP (Helena Biosciences), and confirmed by measurement of low serum ferritin and normal C- reactive protein (CRP). RESULTS: Median age was 17 years (IQR 16-18), all were Sinhalese and 814/1324 (61.5%) were female. HPLC identified 26 students with β-thalassaemia trait and 3 with HbE trait. The KKU-OF test correctly identified all 26 students with β-thalassaemia trait (sensitivity=100%) but was also positive in 288 samples with a normal HPLC result (specificity= 1007/1295; 85.5%). The KKU-DCIP test was positive only in the 3 students with HbE (100% sensitive and 100% specific). Iron deficiency was present in 148/1318 (11.2%) students. CONCLUSION: This one-stop approach may be an effective and affordable procedure in screening for haemoglobinopathy traits and identifying iron deficiency. We are investigating the causes of the false positive OF test results and planning large scale studies to assess cost-effectiveness.Item Body iron status of children with transfusion dependent thalassaemia: Trends of serum ferritin and associations of optimal body iron control(Sri Lanka Medical Association, 2018) Suriapperuma, T. N. P.; Peiris, K. R. R.; Mettananda, K.C.D.; Premawardhena, A.; Mettananda, S.INTRODUCTION AND OBJECTIVES: Iron overload due to regular transfusions is one of the most troublesome complications ofthalassaemia. Here we aim to describe body iron status, trends of serum ferritin and associations of optimal body iron control among patients with transfusion dependent thalassaemia. METHODS: A cross sectional descriptive study was conducted at Paediatric and Adolescent Thalassaemia Centres of Colombo North Teaching Hospital from October to December 2017. All children with transfusion dependent thalassaemia aged 16 years and below attending for blood transfusions were recruited. Data was collected using an interviewer-administered questionnaire by interviewing patients and perusing medical records and analysed using SPSS. Ethical approval was obtained from Ethics Committee of University of Kelaniya. RESULTS: Fifty-four children were recruited; 52% were males. Age groups were; <2 years:3.7%, 2-5 years:9.3%,6-10 years:29.6% and 11-16 years:57.4%. Majority (80%) were diagnosed with thalassaemia within the first year of life; 83% had thalassaemia major while 13% had HbE/thalassaemia. Serum ferritin levels were;4999ng/ml:3.7%. Trend of mean serum ferritin at yearly intervals showed gradual rise until 5 years and plateauing thereafter. Children with serum ferritin Rs.25000/=) was significantly associated with optimal body iron control (OR-4.81;95%Cll.17-19.67; p<0.05).CONCLUSION: Mean serum ferritin level gradually rose until 5-years of age and plateaued off in this sample. Optimal body iron control was positively associated with older age at diagnosis of thalassemia and higher family income.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 Microcytic anaemia in children: Do we really know the cause?(Sri Lanka Medical Association, 2018) Paranamanna, S.; Fernando, V. R.; Suranjan, P. D. M.; Rodrigo, R.; Perera, L.; Vipulanayake, U. K. T.; Fernando, A. G. L. N. P.; Fernando, A.M.; Costa, Y.; Dayanath, D.K.T.P.; Premawardhena, A.; Mettananda, S.INTRODUCTION & OBJECTIVES: Microcytic anaemia is common among children and is often attributed to iron deficiency. Other causes are given less priority. We aimed to describe the aetiology of microcytic anaemia among children aged between 6-59 months.METHODS: A descriptive study was conducted at Teaching Hospital, Ragama from March 2016 to February 2017. All newly diagnosed patients with microcytic anaemia (haemoglobin <11 g/dL and mean corpuscular volume <80 Fl) were recruited. Data was collected using an interviewer-administered questionnaire and 5ml of venous blood was obtained for full blood count, serum ferritin, capillary electrophoresis and alpha-globin genotype during a period free from any acute illness. A therapeutic trial with oral iron (6mg/kg/day) was given to all children and response was assessed after one month. Ethical approval was obtained from Ethics Committee of University of Kelaniya and data were analysed using SPSS. RESULTS: Sixty-six children (male-54.5%; mean age-20.5±13.9 months) were recruited. Severity of anaemia was mild-38%, moderate-61% and severe- I%. Reported clinical features were: irritability (26%), loss of appetite (6%), fatigue (5%), pica (3%), brittle hair (26%), dry skin (23%), angular stomatitis (2%) and glossitis (2%). Aetiologically, 28 (42.4%) had iron deficiency (ferritinlg/dl after 1 month) was observed in 27 (40 9%).CONCLUSION: Less than half of children with microcytic anaemia had low serum ferritin and only 41 % of children demonstrated a response to a trial of oral iron Thalassaemia trait, especially alpha-thalassaemia is an important cause for microcytic anaemia in asymptomatic children.