Browsing by Author "Bandara, D."

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Alpha thalassaemia and extended alpha globin genes in Sri Lanka
(Elsevier-Academic Press, 2013) Suresh, S.; Fisher, C.; Ayyub, H.; Premawardhena, A.; Allen, A.; Perera, A.; Bandara, D.; Olivieri, N.; Weatherall, D.
The α-globin genes were studied in nine families with unexplained hypochromic anaemia and in 167 patients with HbE β thalassaemia in Sri Lanka. As well as the common deletion forms of α(+) thalassaemia three families from an ethnic minority were found to carry a novel form of α(0) thalassaemia, one family carried a previously reported form of α(0) thalassaemia, --(THAI), and five families had different forms of non-deletional thalassaemia. The patients with HbE β thalassaemia who had co-inherited α thalassaemia all showed an extremely mild phenotype and reduced levels of HbF and there was a highly significant paucity of α(+) thalassaemia in these patients compared with the normal population. Extended α gene arrangements, including ααα, αααα and ααααα, occurred at a low frequency and were commoner in the more severe phenotypes of HbE β thalassaemia. As well as emphasising the ameliorating effect of α thalassaemia on HbE β thalassaemia the finding of a novel form of α(0) thalassaemia in an ethnic minority, together with an unexpected diversity of forms of non-deletion α thalassaemia in Sri Lanka, further emphasises the critical importance of micro-mapping populations for determining the frequency of clinically important forms of the disease.
Blood transfusion therapy for β-thalassemia major and hemoglobin E β-thalassemia: adequacy, trends, and determinants in Sri Lanka.
(John Wiley, 2019) Mettananda, S.; Pathiraja, H.; Peiris, R.; Wickramarathne, N.; Bandara, D.; de Silva, U.; Mettananda, C.; Premawardhena, A.
BACKGROUND: Regular blood transfusion therapy still remains the cornerstone in the management of β-thalassemia. Although recommendations are clear for patients with β-thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E β-thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with β-thalassemia major and hemoglobin E β-thalassemia. METHODS/PROCEDURE: This cross-sectional study was performed among all regularly transfused patents with β-thalassemia aged 2 to 18 years attending three large thalassemia centers in Sri Lanka. Data were collected using an interviewer-administered questionnaire, perusal of clinical records, and physical examination of patients by trained doctors. RESULTS: A total of 328 patients (male 47%) were recruited; 83% had β-thalassemia major, whereas 16% had hemoglobin E β-thalassemia. Sixty-one percent of patients had low pretransfusion hemoglobin levels (< 9.0 g/dL) despite receiving high transfusion volumes (> 200 mL/kg/year) by a majority (56%). Median pretransfusion hemoglobin was significantly lower in patients with hemoglobin E β-thalassemia compared with β-thalassemia major (P < 0.001); however, there was no difference in requirement for high transfusion volumes over 200 mL/kg/year in both groups (P = 0.14). Hepatomegaly and splenomegaly were more common in hemoglobin E β-thalassemia and were associated with lower pretransfusion hemoglobin. Transfusion requirements were higher among patients with hepatitis C and in those who are underweight. CONCLUSIONS: Over 60% of regularly transfused patients with β-thalassemia have low pretransfusion hemoglobin levels despite receiving large transfusion volumes. Patients with hemoglobin E β-thalassemia are undertransfused and specific recommendations should be developed to guide transfusions in these patients.
Dietary goitrogens and prevalence of goitre in Sri Lanka
(The College of Surgeons of Sri Lanka, 2011) Pinto, M. D. P.; Fernando, R.; Pathmeswaran, A.; Premathilake, M.; Fernando, T.; Edirisinghe, D.; Bandara, D.; Athulugama, N.; Abeysinghe, S.
OBJECTIVE: To assess the relationship between dietary goitrogens and the prevalence of goiter. METHODS:A descriptive cross-sectional study on the prevalence of goitre was conducted involving all areas of the country, except North and Batticaloa district from December 2006 to June 2007. A pre-tested interviewer administered questionnaire was used on all participants as a screening tool. Consumption of 6 possible dietary goitrogens was assessed. Sufficient frequency of consumption was taken as consumption >3 times a week. Pearson's chi-square test was used to analyze the significance of dietary goitrogens to the prevalence of goiter. RESULTS: 5200 participants were assessed (n=5200) island wide and 426 were detected with goitre. Mean age for occurrence of goitre was 36.3(+_17.3) years. Frequency of consumption of these food items was low, none being higher than 10% among the 5200 participants. Consumption of each goitrogen was as follows; Cabbage "[2.9%], Lima beans [9.9%], Kurakkan (Millet) [4%], Turnip [2.9%], Manioc(Cassava) [2.2%] and Peanuts [5.5%]. All six dietary goitrogens showed no significant association with the occurrence of goitre (p<0 05).CONCLUSION:Dietary goitrogens do not appear to have an association with prevalence of goitre in this cohort. As this cohort is a representative sample, dietary goitrogens are unlikely to be associated with prevalence of goitre in Sri Lanka.
Epidemiology of goitres in Sri Lanka - results of a community based study
(Sri Lanka Medical Association, 2008) Fernando, R.; Pathmeswaran, A.; Edirisinghe, D.; Abeysinghe, S.; Atulugama, N.; Bandara, D.; Premathileke, M.
BACKGROUND: lodization of salt was made mandatory in Sri Lanka in 1995. Data on the current prevalence and geographic distribution of goitre among the general population is not available. Objectives: To describe the epidemiology of goitre in the population over the age of 10 years in Sri Lanka DESIGN, SETTING AND METHODS: The country (excluding the Northern Province) was divided into 6 ecological zones based on annual rainfall and altitude. From each zone, 18 Grama Niladhari (GN) areas were selected, using the probability proportionate to size (PPS) technique. First household to be sampled was selected at random from the list maintained by the midwife and all eligible people in the house were sampled. The closest household, next to the index household,, was visited and sampling carried out next. The process was repeated until 50 subjects were sampled in a GN area. Each subject was examined by at least two researchers for the presence of a goitre. RESULTS: Total of 5200 subjects were assessed. 66 %( 3,433) were females. Mean (SD) age 36.3(17.3) years. 425 subjects had a goitre. The distribution of goitres according to the WHO categorization - Grades 1, 2, 3 and 4 was 18.6, 68.0, 12.5 and 0.9 percent respectively. Adjusted (age and zone) prevalence among females and males was 11.2% and 2.3% respectively. Adjusted (age and sex) prevalence (95% CI) in the different zones varied between 5.34% in the intermediate zone-south to 7.93% in the wet zone-hills. Overall adjusted prevalence was 6.8% (6.0-7.6). CONCLUSIONS: The prevalence of goitres is 6.8%. There is definite female preponderance. No obvious goitre belt can be discerned.
FNAC in the 'field'- a new experience
(Sri Lanka Medical Association, 2008) Fernando, R.; Hewavisenthi, J.; Pathmeswaran, A.; Edirisinghe, D.; Abeysinghe, S.; Atulugama, N.; Bandara, D.; Premathileke, M.
BACKGROUND: Fine needle aspiration cytology (FNAC) is an important diagnostic test in thyroid diseases. It usually is undertaken at a hospital or laboratory setup. As part of an islandwide field based study, FNAC was undertaken in the field. Literature review revealed that FNAC accuracy rate was around 65-70% in most Centres with full-fledged facilities. OBJECTIVES: To determine the effectiveness of FNAC done in the field DESIGN, SETTING AND METHODS: Informed written consent was obtained and the goitre was delineated with a marker pen. FNAC was carried out by a trained team member in the standard manner. Four slides were prepared from each patient. All slides were processed and examined by a cytologist RESULTS: 308 FNAC were done. 226 FNAC were diagnostic (73.36%) and 82(26.64%) were non diagnostic. 62(23.8%) showed benign cells only. The pathological entities detected were: 112 (49.5%) auto immune thyroiditis, 32 (14.1%) colloid storing nodules, 14(6.1%) colloid storing nodules with autoimmune thyroiditis, 5 (2.2%) follicular lesions and one toxic lesion. There were no significant complications noted in any of the patients. CONCLUSIONS: Field FNAC has a high diagnostic accuracy if done by a trained person. It is a safe procedure in the field.
Health related quality of life among children with transfusion dependent β-thalassaemia major and haemoglobin E β-thalassaemia in Sri Lanka: a case control study.
(BioMed Central, 2019) Mettananda, S.; Pathiraja, H.; Peiris, R.; Bandara, D.; de Silva, U.; Mettananda, C.; Premawardhena, A.
BACKGROUND:Thalassaemia is a chronic disease without an effective cure in a majority. The clinical management has improved considerably during recent years; however, minimal attempts are made to up lift the quality of life among patients, especially in developing countries. Here we aim to describe and compare and to determine factors associated with health related quality of life among patients with transfusion dependent β-thalassaemia major and haemoglobin E β-thalassemia in Sri Lanka.METHODS:A case control study was conducted in the three largest thalassaemia centres of Sri Lanka. All patients with transfusion dependent β-thalassaemia (β-thalassaemia major and haemoglobin E β-thalassaemia) aged 5-18 years were recruited as cases whilst a randomly selected group of children without chronic diseases were recruited as controls. Socio-demographic and clinical data were collected using an interviewer-administered questionnaire and health related quality of life was measured using the validated Paediatric Quality of Life Inventory Version 4.0.RESULTS:Two hundred and seventy one patients with transfusion dependent β-thalassaemia (male-49.1%; mean age- 10.9 ± 3.6 years) and 254 controls (male-47.2%; mean age- 10.4 ± 3.5 years) were recruited. Mean health-related quality of life scores were significantly lower in patients compared to controls (72.9 vs. 91.5, p < 0.001). Of the patients, 224 (84%) had β-thalassaemia major and 43 (16%) had haemoglobin E β-thalassaemia. Quality of life scores in psychological health (p < 0.05), emotional functioning (p < 0.05) and social functioning (p < 0.05) were significantly lower in patients with haemoglobin E β-thalassaemia compared to β-thalassaemia major. Splenectomy (p < 0.05), short stature (p < 0.05), under nutrition (p < 0.05) and longer hospital stays (p < 0.05) were significantly associated with lower quality of life scores.CONCLUSIONS:Despite improvements in management, the quality of life among patients with β-thalassaemia still remains low. This is more pronounced in the subset of patients with haemoglobin E β-thalassaemia. Splenectomy, short stature, undernutrition and longer hospital stays were significantly associated with poor quality of life. It is timely, even in developing countries, to direct emphasis and to take appropriate steps to improve standards of living and quality of life of patients with β-thalassaemia.
Hepatic and renal status of paediatric patients with thalassaemia
(Sri Lanka Medical Association, 2023) Wijenayake, W.; Pathiraja, H.; Thennakoon, R.; Fernando, M.; Bandara, D.; Mettananda, S.
INTRODUCTION: Thalassaemia is a chronic disorder affecting many organ systems. Although cirrhosis is a well-recognised complication, sub-clinical hepatic and renal dysfunction in thalassaemia are poorly studied. OBJECTIVES: We aimed to describe the hepatic and renal status and dysfunction of paediatric patients with thalassaemia. METHODS: A cross-sectional descriptive study was conducted at Kurunegala and Ragama Thalassaemia Centres during February and March 2023. All patients aged less than 16 years attending the thalassaemia centres were recruited. Data were collected using a data collection form by interviewing parents and perusal of clinical records and analysed using SPSS 27.0. Ethical approval was obtained from the Sri Lanka College of Paediatricians. RESULTS: Sixty-five patients (mean age-7.7; males-46%) were recruited. Of them 48(73%) had homozygous beta-thalassaemia and 17(26%) had HbE thalassaemia; 52(80%) were transfusion-dependent and 13(20%) were non-transfusion-dependent. Hepatomegaly and splenomegaly were found in 45(69%) and 30(46%), respectively. Regarding hepatic status, 34(52%) had high (>40IU/L) alanine transaminases, of which 8(12%) had >3-fold elevation of alanine transaminases. A higher proportion of children with HbE thalassaemia (71%) had elevated alanine transaminases compared to homozygous beta-thalassaemia (46%, χ2=3.0, p=0.07). Also, a higher proportion of children on deferasirox (57%) had elevated alanine transaminases compared to those who were not on the drug (27%, χ2=3.3, p=0.06). Four (5%) had <2+ proteinuria in urinalysis however, the urine protein: creatine ratio was normal in all. CONCLUSION: High alanine transaminases were noted in over 50% of paediatric patients with thalassaemia. HbE thalassaemia type and use of deferasirox were associated with high alanine transaminase levels.
Hepcidin is suppressed by erythropoiesis in hemoglobin E β-thalassemia and β-thalassemia trait
(American Society of Hematology, 2015) Jones, E.; Pasricha, S.R.; Allen, A.; Evans, P.; Fisher, C.A.; Wray, K.; Premawardhena, A.; Bandara, D.; Perera, A.; Webster, C.; Sturges, P.; Olivieri, N.F.; St Pierre, T.; Armitage, A.E.; Porter, J.B.; Weatherall, D.J.; Drakesmith, H.
Hemoglobin E (HbE) β-thalassemia is the most common severe thalassemia syndrome across Asia, and millions of people are carriers. Clinical heterogeneity in HbE β-thalassemia is incompletely explained by genotype, and the interaction of phenotypic variation with hepcidin is unknown. The effect of thalassemia carriage on hepcidin is also unknown, but it could be relevant for iron supplementation programs aimed at combating anemia. In 62 of 69 Sri Lankan patients with HbE β-thalassemia with moderate or severe phenotype, hepcidin was suppressed, and overallhepcidin inversely correlated with iron accumulation. On segregating by phenotype, there were no differences in hepcidin, erythropoiesis, orhemoglobin between severe or moderate disease, but multiple linear regression showed that erythropoiesis inversely correlated with hepcidin only in severe phenotypes. In moderate disease, no independent predictors of hepcidin were identifiable; nevertheless, the low hepcidin levels indicate a significant risk for iron overload. In a population survey of Sri Lankan schoolchildren, β-thalassemia (but not HbE) trait was associated with increased erythropoiesis and mildly suppressed hepcidin, suggesting an enhanced propensity to accumulate iron. In summary, the influence oferythropoiesis on hepcidin suppression associates with phenotypic disease variation and pathogenesis in HbE β-thalassemia and indicates that the epidemiology of β-thalassemia trait requires consideration when planning public health iron interventions.
Image Processing Approach for Ancient Brahmi Script Analysis
(University of Kelaniya, 2015) Warnajith, N.; Bandara, D.; Bandara, N.; Minato, A.; Ozawa, S.
Writing is one of the most important inventions of Humankind. There are few main languages in the ancient world. Among these ancient languages Sanskrit was used in Indo region. Brahmi script is one of the most important ancient letters in South Asia. It became the matrix of Debanagaricharacter used for Sanskrit and Hindi. And, it produced Burmese script, Khmer script, Thai alphabet, Laotian alphabet, Tibetan alphabet, etc., during the last two thousand years. The people of Sri Lanka are fortunate that some of the writing of their ancestors is preserved on various inscriptions found throughout the country. These inscriptions have been discovered under the drip ledges of caves, rocks, pillars and slabs. Inscribed gold, silver and copper plates have been discovered as well. On the decipherment of these inscriptions, historical, linguistic and paleographical conclusions could be arrived at. The source data of this study is already published grayscale images of early Brahmi script. These images are taken from the paper copies of inscriptions called as estampages (rubbed copy). Up to the present, reading and analyzing these inscriptions are done by manually. The aim of this study is to develop algorithms based on image data of these estampages and implement a digital data repository. The process of this study can be divided into few stages. Such as: (1). Producing precise alphabet fonts of early Brahmi scripts has been produced from photographic data, (2). Precise method of identification of ancient letters with the aid of the alphabet fonts, which leads to automatic reading of ancient inscriptions by computers, (3). Database implementation for the analysis, (4). Develop web based interface for scholars over KISSEL network to share the knowledge and experience among the KISSEL users.
Jaina Religion in Ancient Sri Lanka
(Centre for Asian Studies, University of Kelaniya, Sri Lanka, 2017) Bandara, N.; Bandara, D.; Harischandra, M.
Jaina religion was one of the most popular religions that existed in ancient India. (It is well known as jainasm or jaina Sharma which belongs to the Sramana tradition) The religious leader of Jainism is known as Nigankanatha putta or Mahavira. Buddhist literature too mentions about Jainism in certain occurrences. Jainism was the first ever religion which invaded the South Asian countries such as South India, Mysore and Ceylon. Significant evidence can be found that can prove Jainism held and important place in ancient Sri Lanka. Local and foreign literary sources confirm the above fact. The main objective of this research is to discuss about the background of Jainism in ancient Sri Lanka based on the above sources. There are no definite factors to confirm as to how and when Jainism commenced its practices in ancient Sri Lanka. But there is evidence that Jainism dominated an authentic position in ancient Sri Lanka during the period of king Pandukabhaya. It becomes clear from Mahawamsa records to the effect that monasteries had been constructed for Jaina religions nominees named Jothiyagiri and Kumbanda. The above statements proves that Jainaism was the first ever religion was accorded the royal patronage of ancient Sri Lanka. Arrival of Arahant Mahinda Thera in Sri Lanka caused to create a new chapter in the history of ancient Sri Lanka. Buddhist doctrinal teachings evolved in the entire island effecting great influence on moral, virtuous and cultural lives of the citizens. Advent of Buddhism may have been strong enough to cause the decline of the powers of Jainaism. King Valagamba destroyed the monastery named Girinigantarama and built up Abhayagiri Viharaya at the same place which act resulted in degrading the authority of Jainaism. But it could be assumed that Jainaism raised its head again from time to time due to occurrences that took place after the Kings of this country left for South India for protection with the invasions of Paundyas and Cholas in Sri Lanka. There is no doubt that such religions influences may have had an impact on the customs, literature and the language which proves by the addition of adverse and unpleasant words like nasty, gaudy and indecorous etc.
Leg ulcers: A report in patients with hemoglobin E beta thalassemia and review of the literature in severe beta Thalassemia
(Basel, Karger., 2022) Mehta, V.; Kirubarajan, A.; Sabouhanian, A.; Jayawardena, S.M.; Chandrakumaran, P.; Thangavelu, N.; Cader, R.; Mettananda, S.; Bandara, D.; Khan, S.; Weatherall, D.J.; Allen, A.; Premawardhena, A.P.; Olivieri, N.F.
BACKGROUND: Leg ulcers are a frequent complication in patients with the inherited hemoglobin disorders. In thalassemia, the literature is limited, and factors associated with the development of leg ulcers in HbE beta thalassemia, the most common form of severe beta thalassemia worldwide, have not previously been reported. METHODS: We reviewed all available medical records of patients with HbE beta thalassemia to document the onset of leg ulcers at the two largest treatment centres in Sri Lanka. We reviewed the literature to identify studies reporting outcomes of interventions for ulcers in severe thalassemia. RESULTS: Of a total of 255 actively registered patients with HbE thalassemia in the two centres, 196 patient charts were evaluable. A leg ulcer with a documented date of onset was recorded in 45 (22%) of 196 evaluable patients, aged (mean ± SEM) 22.2 ± 1.4 years. Most had been irregularly transfused; steady state hemoglobin was 6.4 ± 0.2 g/dL. Treatment achieving healing in 17 patients included transfusions, antibiotics, oral zinc, WOUND TOILETING AND SKIN GRAFTING. DISCUSSION/CONCLUSION: Leg ulcers may be more common in HbE beta thalassemia than in other forms of thalassemia. A systematic approach to treatment will be needed to document the prevalence and factors placing such patients at risk for leg ulcers. Controlled trials to evaluate the optimal treatment of this common complication are indicated.
Maternal knowledge on curative therapies and its impact on medical care and psychological health among children with thalassaemia in Sri Lanka
(Sri Lanka College of Paediatricians, 2022) Mettananda, S.; Pathiraja, H.; Peiris, R.; Bandara, D.; de Silva, U.; Mettananda, C.; Premawardhena, A.
Background: b-thalassaemia is an inherited disorder of haemoglobin synthesis which results in severe transfusion-dependent anaemia from infancy. Although considered a life-limiting disease, it can be cured by allogeneic haematopoietic stem cell transplantation and gene therapy. However, many patients and their families in developing countries are unaware of these treatment options. Objectives: To assess the maternal knowledge on curative therapies and to determine its association with the adequacy of current medical treatment and psychological health among children with b-thalassaemia. Method: We conducted a cross-sectional study at the three largest thalassaemia centres of Sri Lanka. All patients with transfusion-dependent b-thalassaemia aged 2-18 years were eligible for the study. Data were collected using an interviewer-administered questionnaire by interviewing mothers and from medical records. The questionnaire contained questions to gather information on socio-demographic background, clinical details and maternal knowledge on curative therapies for thalassaemia. The psychological morbidity of children was assessed using the previously validated ‘strengths and difficulties questionnaire’. Binary logistic regression was used in the analysis. Results: A total of 304 patients (mean age 9.8years; females 54%) were recruited. A majority (86%) of mothers knew that b-thalassaemia can be cured by haematopoietic stem cell transplantation; however, only 1% were aware of gene therapy. Detailed knowledge on curative therapies was lacking in most mothers; only 22% could identify suitable donors for transplantation. Maternal knowledge on curative therapies was associated with higher educational level and income of parents. Accurate maternal knowledge on haematopoietic stem cell transplantation was significantly associated with lower rates of hepatomegaly, splenomegaly, emotional symptoms, conduct symptoms, hyperactive symptoms and abnormal peer relationships in patients. Conclusions: This study demonstrated that maternal knowledge on curative therapies among patients with b-thalassaemia is sub-optimal. It further demonstrated that having an accurate maternal knowledge is associated with improved medical care and a lower prevalence of psychological symptoms among patients.
Methemoglobinemia and ascorbate deficiency in hemoglobin E β thalassemia: metabolic and clinical implications.
(American Society of Hematology., 2012) Allen, A.; Fisher, C.; Premawardhena, A.; Bandara, D.; Perera, A.; Allen, S.; St Pierre, T.; Olivieri, N.; Weatherall, D.
ABSTRACT: During investigations of the phenotypic diversity of hemoglobin (Hb) E β thalassemia, a patient was encountered with persistently high levels of methemoglobin associated with a left-shift in the oxygen dissociation curve, profound ascorbate deficiency, and clinical features of scurvy; these abnormalities were corrected by treatment with vitamin C. Studies of erythropoietin production before and after treatment suggested that, as in an ascorbate-deficient murine model, the human hypoxia induction factor pathway is not totally dependent on ascorbate levels. A follow-up study of 45 patients with HbE β thalassemia showed that methemoglobin levels were significantly increased and that there was also a significant reduction in plasma ascorbate levels. Haptoglobin levels were significantly reduced, and the high frequency of the 2.2 haptoglobin genotype may place an additional pressure on ascorbate as a free-radical scavenger in this population. There was, in addition, a highly significant correlation between methemoglobin levels, splenectomy, and factors that modify the degree of globin-chain imbalance. Because methemoglobin levels are modified by several mechanisms and may play a role in both adaptation to anemia and vascular damage, there is a strong case for its further study in other forms of thalassemia and sickle-cell anemia, particularly when splenic function is defective.
Novel Approach for Analyzing Brahmi Scripts
(Department of Zoology, University of Kelaniya, Kelaniya, Sri Lanka, 2014-06) Bandara, D.; Warnajith, N.; Minato, A.; Ozawa, S.
Writing is one of the most important inventions of humankind. There are few main languages in the ancient world. Among these ancient languages Sanskrit was used in the Indo region. Brahmi script is one of the most important ancient letters in South Asia. It became the matrix of Debanagari character used for Sanskrit and Hindi. The people of Sri Lanka are fortunate that some of the writing of their ancestors is preserved on various inscriptions found throughout the country. These inscriptions have been discovered under the drip ledges of caves, rocks, pillars and slabs. Inscribed gold, silver and copper plates have been discovered as well. On the decipherment of these inscriptions, historical, linguistic and paleographical conclusions could be arrived at. The source data of this study is already published as gray scale images of early Brahmi script. These images are taken from the paper copies of inscriptions called as estampages (rubbed copy). Up to the present, reading and analyzing these inscriptions are done manually. The aim of this study was to develop algorithms based on image data of these estampages and implement a digital data repository. The process of this study can be divided into several stages as follows: (1). Producing precise alphabet fonts of early Brahmi scripts from photographic data, (2). Development of a precise method of identification of ancient letters with the aid of the alphabet fonts, which leads to automatic reading of ancient inscriptions by computers, (3). Database implementation for the analysis, (4). Development of web based interface for scholars over KISSEL network to share the knowledge and experience among the KISSEL users.
Psychological morbidity among children with transfusion dependent β-thalassaemia and their parents in Sri Lanka
(Public Library of Science, 2020) Mettananda, S.; Peiris, R.; Pathiraja, H.; Chandradasa, M.; Bandara, D.; de Silva, U.; Mettananda, C.; Premawardhena, A.
BACKGROUND: Thalassaemia is a chronic disease which requires lifelong treatment in a majority. Despite recent advances in the medical care, minimal attempts are made to improve psychological health in these patients. In this study, we aim to describe the psychological morbidity in patients with transfusion dependent β-thalassaemia and their mothers in Sri Lanka. METHODS: This case control study was conducted in the three largest thalassaemia centres of Sri Lanka. All patients with transfusion dependent β-thalassaemia aged 4-18 years were recruited as cases whilst a randomly selected group of children without chronic diseases were recruited as controls. Psychological morbidity of children was assessed using the Strengths and Difficulties Questionnaire and depressive symptoms of mothers was assessed by the Centre for Epidemiological Studies Depression Scale. RESULTS: 288 transfusion dependent β-thalassaemia patients and equal number of controls were recruited. Abnormal emotional, conduct, hyperactivity and peer relationship symptom scores were reported by 18%, 17%, 9% and 14% of patients with thalassaemia respectively. Prevalences of abnormal psychological symptom scores in all domains were significantly higher among patients compared to controls. Abnormal conduct symptoms were significantly more prevalent among patients with HbE β-thalassaemia and those with suboptimal pretransfusion haemoglobin levels, lower transfusion volumes, hypothyroidism and undernutrition. Short stature was associated with abnormal emotional and hyperactivity scores. Depressive symptoms were significantly higher among mothers of patients with thalassaemia. Higher depressive symptom scores in mothers were significantly associated with abnormal emotional, conduct and peer relationship symptom scores in children. CONCLUSIONS: A higher proportion of patients with transfusion dependent β-thalassaemia had abnormal psychological symptom scores. Abnormal conduct symptoms were more prevalent among patients with HbE β-thalassaemia, those who were inadequately transfused and having hypothyroidism and undernutrition. Mothers of the children with transfusion dependent β-thalassaemia had significantly higher depressive symptoms which were significantly associated with psychological symptoms among children.
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 thalassaemia
Sickle cell disease in Sri Lanka: clinical and molecular basis and the unanswered questions about disease severity
(BioMed Central., 2020) Darshana, T.; Bandara, D.; Nawarathne, U.; de Silva, U.; Costa, Y.; Pushpakumara, K.; Pathirage, S.; Basnayake, S.; Epa, C.; Dilrukshi, P.; Wijayawardena, M.; Anthony, A. A.; Rodrigo, R.; Manamperi, A.; Smith, F.; Allen, A.; Menzel, S.; Rees, D.; Premawardhena, A.
BACKGROUND: Though case reports and limited case series of Sickle cell disease in Sri Lanka have been reported previously, no attempt has been made hitherto to undertake a comprehensive genotypic-phenotypic analysis of this "rare" group of patients. RESULTS: All accessible Sickle cell disease patients, totaling 60, including, 51 Sickle β-thalassaemia and 9 homozygous sickle patients were enrolled from seven thalassaemia treatment centres between December 2016-March 2019. The majority of patients were of Sinhalese ethnicity (n = 52, 86.67%). Geographically, two prominent clusters were identified and the distribution of Sickle haemoglobin in the island contrasted markedly with the other haemoglobinopathies. 3/ 9 homozygous sickle patients and 3/ 51 Sickle β-thalassaemia patients were receiving regular transfusion. Joint pain was the commonest clinical symptom among all sickle cell disease patients (n = 39, 65.0%). Dactylitis was significantly more common in homozygous sickle patients compared with the Sickle β-thalassaemia groups (p 0.027). Two genetic backgrounds sickle mutation were identified namely, Arab Indian and Benin. Among the regulators of Foetal hemoglobin in Sickle patients of the present study rs1427407 G > T seemed to be the most prominent modifier, with a significant association with Foetal haemoglobin levels (p 0.04). CONCLUSIONS: Overall, the clinical course of the Asian version of Sickle cell disease in Sri Lanka appears to be milder than that described in India. KEYWORDS: Clinical; Genetic; Severity; Sickle cell; Sri Lanka.
Survival and complications in patients with haemoglobin E thalassaemia in Sri Lanka: a prospective, longitudinal cohort study.
(Elsevier Ltd, 2022) Premawardhena, A.P.; Ediriweera, D.S.; Sabouhanian, A.; Allen, A.; Rees, D.; de Silva, S.; Perera, W.; Katugaha, N.; Arambepola, M.; Yamashita, R.C.; Mettananda, S.; Jiffry, N.; Mehta, V.; Cader, R.; Bandara, D.; St Pierre, T.; Muraca, G.; Fisher, C.; Kirubarajan, A.; Khan, S.; Allen, S.; Lamabadusuriya, S.P.; Weatherall, D.J.; Olivieri, N.F.
Background: Worldwide, haemoglobin E β-thalassaemia is the most common genotype of severe β-thalassaemia. The paucity of long-term data for this form of thalassaemia makes evidence-based management challenging. We did a long-term observational study to define factors associated with survival and complications in patients with haemoglobin E thalassaemia. Methods: In this prospective, longitudinal cohort study, we included all patients with haemoglobin E thalassaemia who attended the National Thalassaemia Centre in Kurunegala, Sri Lanka, between Jan 1, 1997, and Dec 31, 2001. Patients were assessed up to three times a year. Approaches to blood transfusions, splenectomy, and chelation therapy shifted during this period. Survival rates between groups were evaluated using Kaplan-Meier survival function estimate curves and Cox proportional hazards models were used to identify risk factors for mortality. Findings: 109 patients (54 [50%] male; 55 [50%] female) were recruited and followed up for a median of 18 years (IQR 14-20). Median age at recruitment was 13 years (range 8-21). 32 (29%) patients died during follow-up. Median survival in all patients was 49 years (95% CI 45-not reached). Median survival was worse among male patients (hazard ratio [HR] 2·51, 95% CI 1·16-5·43), patients with a history of serious infections (adjusted HR 8·49, 2·90-24·84), and those with higher estimated body iron burdens as estimated by serum ferritin concentration (adjusted HR 1·03, 1·01-1·06 per 100 units). Splenectomy, while not associated with statistically significant increases in the risks of death or serious infections, ultimately did not eliminate a requirement for scheduled transfusions in 42 (58%) of 73 patients. Haemoglobin concentration less than or equal to 4·5 g/dL (vs concentration >4·5 g/dL), serum ferritin concentration more than 1300 μg/L (vs concentration ≤1300 μg/L), and liver iron concentration more than 5 mg/g dry weight of liver (vs concentration ≤5 mg/g) were associated with poorer survival. Interpretation: Patients with haemoglobin E thalassaemia often had complications and shortened survival compared with that reported in high-resource countries for thalassaemia major and for thalassaemia intermedia not involving an allele for haemoglobin E. Approaches to management in this disorder remain uncertain and prospective studies should evaluate if altered transfusion regimens, with improved control of body iron, can improve survival.
Trends shaping the accounting profession and its future outlook
(Department of Accountancy, Faculty of Commerce and Management Studies, University of Kelaniya, Sri Lanka, 2017) Lakdeva, V.; Silva, T.; Bandara, D.
පල්ලව අභාසය ලැබූ පැරණි සිංහල පිල්ලම්
(University of Kelaniya, 2006) Bandara, D.