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Browsing by Author "Premawardhena, A.P."

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    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.
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    Anemia in Sri Lanka: A literature review
    (Informa Healthcare, 2022) Amarasingha, A.A.D.S.; Silva, H.J.R.L.; Perera, P.S.; Premawardhena, A.P.
    Anemia is a global health problem. This paper reviews literature on the prevalence of anemia in Sri Lanka. We searched EBSCO (Elton Bryson Stephens Company), Cochrane Library, and Medline for articles on prevalence and molecular basis of anemia in Sri Lanka from January 2000 to May 2021. Forty articles were selected. Most of the studies were on prevalence of anemia among children and pregnant women. All the studies had restricted themselves to assess the contributing factors for anemia in limited age categories. Most articles had attempted to determine the overall prevalence of anemia and the contribution of iron deficiency to it. There were only a few studies on prevalence and molecular basis of hemoglobinopathies and even fewer on the prevalence of anemia of chronic disease. None of the studies had attempted to assess the national prevalence of red cell membranopathies and enzymopathies. The published data on prevalence of anemia in Sri Lanka are incomplete. This review emphasizes the value of a much broader survey on anemia covering all age categories including the elderly and conducting a national survey including anemia of chronic disease and on red cell membranopathies and enzymopathies in Sri Lanka.
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    Apical cardiomyopathy : an important differential diagnosis in ischaemic chest pain
    (Sri Lanka Medical Association, 1996) Seneviratne, S.L.; Premawardhena, A.P.; Ranasinghe, G.W.; Gunatilake, S.B.; de Silva, H.J.
    Reports the cases of 1) a 54 year old man admitted with angina like pain of 3 hours. ECG showed deep ngative T waves in the anterolateral leads. But no septal Q waves. 2) a seventy year old man admitted with retrosternal chest pain and dyspnoea of 6 hours. ECG was as above. There were further investigations. Both patients had symptoms suggestive of apical hypertrophic cardiomyopathy
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    Assessing liver fibrosis in patients with transfusion dependent beta thalassaemia - a predictive model
    (Sri Lanka Medical Association, 2021) Padeniya, A.G.P.M.; Ediriweera, D.; de Silva, A.; Niriella, M.A.; Premawardhena, A.P.
    Introduction and Objectives Liver fibrosis in β-thalassaemia major is mainly due to transfusion-related iron overload. Transient elastography (TE) is an imaging modality which measures liver stiffness/fibrosis non-invasively. TE is simple, safe and efficient. However, inaccessibility and high-cost hinders its routine use. We designed a predictive model to evaluate liver fibrosis using demographic, anthropometric, biochemical and imaging data. Methods Sixteen patients with transfusion dependent beta thalassaemia were recruited to the study. FBC, LFT, serum ferritin and Transient Elastography (TE) and FerriScan measurements were recorded at the baseline and after two years follow up. Multiple regression model was developed to predict liver fibrosis using demographic, anthropometric, biochemical and imaging data. [age, gender, body mass index (BMI), steatosis score, liver iron content, mean pre-Hb over the last year, no of blood transfusions (lifetime), amount of blood ingested over the last year(ml/kg), amount of elemental iron by transfusions over last year(mg/ kg), serum ferritin, SGOT, SGPT and compliance with iron chelation].Results Of 16, 8 (50%) were females, mean (SD) age, BMI and fibrosis scores were 21(4.3) years, 18.8 (2.8) kgm-2 and 9.7(5.7) kPa respectively. Gender, BMI, SGOT, SGPT, compliance, number of transfusions taken lifetime showed significant association with liver fibrosis. The final model showed a coefficient of determination (R2) of 0.859. According to the model, predicted liver fibrosis is given by;-26.18 - 4.38*male+1.01*BMI - 0.11*SGPT+0.32*SGOT+2.78*compliance (rps)+0.04*no. of transfusions. ConclusionThe suggested model is a reliable tool to predict liver fibrosis in transfusion-dependent β-thalassaemia major patients in resource poor settings.
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    Assessing reversibility of liver fibrosis in patients with transfusion-dependent beta thalassaemia following intensive chelation
    (Sri Lanka Medical Association, 2023) Padeniya, A.G.P.M.; Ediriweera, D.; Niriella, M.A.; de Silva, A.; Premawardhena, A.P.
    INTRODUCTION: Transfusion-related iron overload is a leading cause of hepatic fibrosis in transfusion-dependent thalassaemia (TDT). OBJECTIVES: This study aimed to evaluate the reversibility of liver fibrosis with intensive chelation therapy in TDT. METHODS: Forty-five patients were included. Serum ferritin, hepatic fibrosis & steatosis (assessed by Transient Elastography), and liver iron concentration/LIC (estimated by FerriScan) were recorded at recruitment and after 2 ½ years of intensive chelation. Compliance for iron chelators was monitored and recorded as good (gc), moderate compliance (mc), and poor (pc) compliance based on the number of days the iron chelators were used. RESULTS: 22/45 (49%) were males [mean age (SD)-19 (4.78) years]. There were 23 (51%), 12 (27%), and 10 (22%) patients with gc, mc, and pc with iron chelators, respectively. The LIC decreased in 36 (80%) patients. The median LIC reduction after 2 ½ years was as follows: gc group-13.5 to 5.1 mg Fe/g dw (P=0.0002); mc group-25.5 to 17.75 mg Fe/g dw (P=0.001). In the pc group, the LIC increased by 10.4 mg Fe/g dw (P =0.058). Liver fibrosis declined in 23 (51%) patients. The liver stiffness at recruitment and after 2 ½ years was 7.6 and 7.1 kPa (P=0.08) in the gc group. In both mc and pc groups, liver fibrosis increased on follow-up [significantly worsened in the pc group (P=0.04)]. CONCLUSION: The reduction of LIC in TDT was related to compliance with chelation therapy; substantial reductions were achieved in those with gc and mc. However, only those with gc managed to arrest the fibrosis progression.
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    Atlanto-axial subluxation:importance of early recognition
    (The Kandy Society of Medicine, 1996) Gunatilake, S.; Seneviratne, S.L.; Premawardhena, A.P.; de Silva, H.J.
    We report three cases which illustrate the importance of the early recognition of atlanto-axial subluxation by being aware of the disorders it is often associated with.
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    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.
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    Chracterisation of beta giobin mutations in Sri Lankan patients with betathalassaemia intermedia
    (Sri Lanka Medical Association, 2013) Perera, S.; Silva, D.P.S.I.; Hapugoda, M.; Wickramarathne, M.N.; Wijesirwardhena, I.; Efremove, D.G.; Fisher, C.A.; Weatherall, D.J.; Premawardhena, A.P.
    INTRODUCTION AND OBJECTIVES: Patients with beta thalassaemia intermedia account for a third of patients attending thalassaemia clinics in Sri Lanka. They show immense phenotypic diversity, the genetic basis for which has not been identified so far. Objective were to characterise beta globin gene mutations in Sri Lankan thalassaemia intermedia patients and to determine how it to influences disease severity. METHODS: We identified 64 thalassaemia intermedia patients from the five main thalassaemia centers; Anuradhapura (n= 6), Kuruncgala (n= 4), Ragama (n= 42), Badulla (n=7) and Chilaw (n=5). Their beta globin DNA sequences were analyzed using ABI PRISM 313lx genetic analyser. RESULTS: Of sixteen patients identified to be homozygous for beta mutations, eleven carried mild beta alleles, IVSI 5 G_C (n= 10) and a rare homozygous promoter mutation - 90 C_T (N=l). Other five were shown to have different types of severe iputations in homozygous state. Nearly half the sample (n=39) was heterozygous for beta mutations. Of them 33 showed mild to severe mutation in one of the alleles IVSI-5 G_C (n=12), IVSI-1 G_A (n= 11) were the commonest. Two patients who were hetcrozygones for beta mutation had a highly unstable Hb variant haemoglobin Mizuho causing severe haemolytic anacma. Hb variants Hb G-Szuhu and Hb G-Coushatta were identified in two patients. CONCLUSIONS: We identified types of beta mutations in some patients with thalassaemia intermedia, which account for the clinical severity.
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    The clinical effects of excessive a globin genes : two family studies
    (Sri Lanka Medical Association, 2003) Premawardhena, A.P.; Fisher, C.A.; Rugless, M.; de Silva, S.; Perera, A.W.V.S.; Olivien, N.F.; Weatherall, D.J.
    INTRODUCTION: Globin chain imbalance is the central pathogenic abnormality in the thalassaemias, a condition where globin gene expression is reduced. Conversely, the inheritance of excess globin genes too may affect the phenotype. However such examples are rarely found. OBJECTIVES: To describe two families in whom the co-existence of excess a genes was noted together with p - thalassaemia trait. METHODS: During the routine P - globin gene analysis in patients attending the Thalassaemia Unit of the Kurunegala Hospital, two patients were identified to have thalassaemia intermedia phenotype, but with just one (3 - thalassaemia mutation. The clinical details of these patients and their families were studied in detail as was their h'aematological and genetic data. RESULTS: We describe two families in which the propositus had inherited six and eight a - genes respectively together with a single p - thalassaemia mutation. Both patients had the thalassaemia intermedia phenotype. The family members who did not inherit any thalassaemic mutations too had varying, but often marked hypochromic microcytosis. DISCUSSION: We describe the first ever family study of a patient with the combination of 8 a - genes and p - thalassaemia trait. We also describe another family where a member had 6 a - genes together with p thalassaemia trait. This highlights yet another mechanism for the intermedia phenotype in patients with a solitary (3 - globin gene mutation. It also highlights the need for the study of a globin genes in patients with unexplained hypochromic microcytic anaemia.
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    Coagulopathy and fibrinoloysis following the bite of a hump-nosed viper (Hypnale hypnale)
    (Oxford University Press, 1996) Premawardhena, A.P.; Seneviratne, S.L.; Gunatilake, S.B.; de Silva, H.J.
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    Cushing syndrome due to an adrenal phaeochromocytoma
    (Sri Lanka Medical Association, 2006) Gunasekara, A.D.C.J.; Premawardhena, A.P.; Hettiarachchi, H.A.N.S.; Rathnasena, B.G.N.; de Silva, H.J.
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    A descriptive study of anaemia in the elderly at a tertiary care institute in Sri Lanka
    (The Sri Lanka Medical Association, 2022) Chathurangani, K.C.; Darshana, L.G.T.; Premathil, R.A.; Costa, Y.J.; Amarasekara, A.A.D.S.; Premawardhena, A.P.
    INTRODUCTION: Anaemia is a commonly encountered condition among the elderly population which calls for further evaluation to identify the cause and to prevent complications.OBJECTIVES: To determine the prevalence, causes and complications related to anaemia among elderly patients admitted to two medical wards (15/16) of Colombo North (Teaching) Hospital, Ragama, Sri Lanka.METHODS: Patients aged over>65 years admitted to the above wards between April –Sep 2020 and who had anaemia were included in the study. Clinical and nutritional data were collected using an interviewer-administered questionnaire. Laboratory findings were extracted from hospital records.RESULTS: The majority of the patients were females (63.2%; n = 129). The mean age was 72.5 years (65 – 92 years). Most of the patients (62.3%; n = 127) were symptomatic for anaemia at the time of hospital admission. The majority of the participants (75.5%; n = 154) did not demonstrate any complications related to anaemia. The severity of the anaemia was moderate among more than half of the patients (52.5%; n=107). Anaemia of chronic disease (54.4%; n=111) was the commonest etiological category detected. The majority of the cases with anaemia of chronic disease were due to chronic renal insufficiency (73.9%; n=82). The severity of the anaemia increased significantly with the presence of chronic disease (p 0.030).CONCLUSION: Most patients in the present study had moderate anaemia whilst anaemia of chronic disease was the leading aetiological class contributor. Community-based studies are needed to understand the true burden of anaemia in the ageing population in Sri Lanka.
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    Do thalassaemic patients, their parents and caregivers support termination of pregnancy as a method for thalassaemia prevention?
    (Sri Lanka Medical Association, 2011) Nandasiri, A.S.D.; Dissakaruna, A.M.D.D.; Silva, D.P.S.I.; Nishad, A.A.N.; Premawardhena, A.P.
    INTRODUCTION AND OBJECTIVES: The only proven way to prevent thalassaemia, the commonest monogenic disease in Sri Lanka, is premarital and ante natal counselling followed by pre-natal diagnosis (PND) and termination of affected fetuses. Termination of pregnancy (TOP) of a thalassaemic fetus is illegal in Sri Lanka. This study assesses the attitudes towards termination of thalassaemic fetuses among the patients themselves, their immediate family members and hospital staff. METHODS: A single operator administered a pre-formed questionnaire to test the knowledge and attitudes towards TOP among patients, parents, immediate family members and hospital staff associated with the thalassaemia unit; over a two month period from Is' of March 2010, at the Thalassaemia Unit, NCTH, Ragama. RESULTS: A total of 24 adult patients with thalassaemia major (TM) and 15 with thalassaemia intermedia (TIM) 39 parents and 25 hospital staff were interviewed. 39.1% of TMs, 60% TIMs, 60% hospital staff, 54.5% parents were aware of PND. 78.8% of TMs, 87% TIMs, 60% hospital staff, 85.3% parents knew that abortion of a thalassaemic fetus was illegal. Only 39.1% of TM supported TOP, if thalassaemia was diagnosed antenatally whilst 73.3% TIMs, 80% hospital staff and 88.57% parents were supportive (pO.OQl). 78.2% of TMs, 100% in all other groups support premarital screening and making partner screening compulsory. CONCLUSIONS: Though there is strong support for TOP from parents and hospital staff, this is not so with patients with TM. However partner selection prior to marriage seems to have more uniform support.
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    Excessive fibrinolysis: the coagulopathy following Merrem's hump-nosed viper( Hypnale hypnale) bites
    (1998) Premawardhena, A.P.; Seneviratne, S.L.; Gunatilake, S.B.; de Silva, H.J.
    In 56 patients with proven hump-nosed viper (Hypnale hypnale) bites, 12 (21.4 percent) developed continued oozing of blood from the site of the bite and a prolonged clotting time. Further investigations showed low fibrinogen levels and increased fibrinogen degradation products in plamsa. The bleeding time, platelet count, prothrombin time, and partial thromboplastin time with kaolin were normal. The bite of this snake can be complicated with a coagulopathy in which excessive fibrinolysis seems to be the main abnormality
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    Familial thrombocytopaenia in three male siblings
    (Sri Lanka Medical Association, 2017) Amarasena, P.; Premawardhena, A.P.; Herath, H.R.B.M.; Seneviratne, S.L.
    INTRODUCTION & OBJECTIVES: Familial thrombocytopaenia is uncommon. Specific molecular defects have been identified in some families. We describe three male siblings with thrombocytopaenia and other immune related findings. The parents are healthy and non-related. METHODS: Clinical and investigative findings were obtained from the brothers and their parents. RESULTS: Case 1: A 15 year old male presented with spontaneous ecchymotic patches and oral mucosal bleeding. Platelet count was 1000/mm3, mild hepatomegaly was present on ultrasonography and IgA was raised. As the response to IV methyl-prednisolone was poor, IVIG, prednisolone and azathioprine were used. He has had recurrent RT infections and as his latest platelet counts are suboptimal, Rituximab is being considered. Case 2: A 19 year old male was found to have thrombocytopaenia whilst being investigated for a large scalp haematoma aged 2 years. He had been treated with oral prednisolone and needed pulse IV dexamethasone. Presently he is off steroids and the platelet count is 54000/mm3. Serum IgM is reduced and IgA is raised. He has chronic bilateral lower limb eczema, an atrio-fascicular accessory pathway and gets recurrent RT infections. Case 3: A 12 year old male had fever, cervical lymphadenopathy and hepatosplenomegaly aged 3 years. He then developed AIHA and thrombocytopaenia. The thrombocytopaenia persisted and was treated with prednisolone and cyclosporine. Aged 9 years, he developed SLE and a year later, class IV lupus nephritis was found on renal biopsy. CONCLUSION: An AR or XR genetic cause is likely in this family. The identification of the exact molecular defect may help with selecting appropriate medications to target abnormal immune pathways.
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    Genetic determinants of jaundice and gallstones in haemoglobin E beta thalassaemia
    (2001) Premawardhena, A.P.; Fisher, C.A.; Fathihu, F.; de Silva, S.; Perera, W.; Peto, T.E.; Olivieri, N.F.; Weatherall, D.J.
    Chronic hyperbilirubinaemia, gallstone formation, and gall bladder disease are unusually common in people with haemoglobin E beta thalassaemia in Sri Lanka. To determine whether this has a genetic basis we compared the bilirubin levels and frequency of gallstones in patients with different alleles of the UGT*1 gene. There was a significantly higher bilirubin level in those with the 7/7 genotypes compared with 6/6 and 6/7 genotype (p=0.032 and 0.0015 respectively), who also appeared more prone to gallstone formation. These results suggest that the UGT*1 genotpe is of importance in the genesis of gallstones in this population of patients.
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    Genetic origin and clinical variability of sickle cell disease in Sri Lanka
    (Sri Lanka Medical Association, 2018) Darshana, L.G.T.; Bandara, W.D.M.S.; Nawaratna, U.S.B.; Costa, Y. J.; Nizri, A.H.M.; Silva, D.P.S. l.; de Silva, T.U.N.; Pushpakumara, K.P.C.; Pathirage, S.P.; Manamperi, A.; Premawardhena, A.P.
    INTRODUCTION AND OBJECTIVES: Sickle cell disease (SCD) is found at a low prevalence In Sri Lanka. A recent hospital based survey identified 60 patients in the country The clinical spectrum of SCD in Sri Lanka has not been studied and its genetic origin remains unknown.We envisaged to study the genetic origin and to carry out a clinical description ofSCD in Sri Lankan patients. METHODS: Patients were recruited from Ragama, Anuradhapura Hambantota and Kurunegala thalassaemia centres. All patients were examined and clinical details recorded. Genetic analyses were performed to identify the haplotype of HbS, Xmn I polymorphism gene deletions and mutations. RESULTS: A total of 49 SCD patients were studied Ages ranged from 5 - 47 years (mean 20.4) 91.8% of the patients were Sinhalese and the rest were Muslims. 87.7% of the patients had sickle-thalassaemia (SBT) and 6 were homozygous (HbSS) Joint pains were the commonest symptom in patients with SBT Clinical presentations in SBT varied from those with none to frequent crises Clinical management varied with only 42.86% of patients being on hydroxyurea. Most patients had not inherited disease ameliorating genetic factors with 95 9% not having gene deletions and 89 8% not having Hb F up-regulators Three Sickle haplotypes were identified including; Arab• Indian, Benin and Bantu.CONCLUSION: There appears to be at-least three genetic origins of HbS in Sri Lanka SCD is extremely clinically variable in Sri Lanka. The reason for this variation needs further study as most patients seem not to have common inherited modifiers
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    The Genetic Origins and Molecular Characterization of Sickle Cell Disease in Sri Lanka.
    (In: Proceedings of the International Postgraduate Research Conference 2017 (IPRC – 2017), Faculty of Graduate Studies, University of Kelaniya, Sri Lanka., 2017) Darshana, L.G.T.; Manamperi, A.; Premawardhena, A.P.
    Sickle cell disease (SCD) is globally the commonest monogenic disease. Although the incidence is not as common as in India, it is found in Sri Lanka too. A recent hospital based survey identified around 60 patients in the country but no detailed study of SCD have been done to-date. The genetic origin of Haemoglobin (Hb) S found in Sri Lanka is not yet known.
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    The Global distribution of length polymorphisms of the promoters of the gucuronosyltransferase I gene(UGTIAI): hematologic and evolutionary implications
    (Academic Press, 2003) Premawardhena, A.P.; Fisher, C.A.; Liu, Y.T.; Verma, I.C.; de Silva, S.; Arambepola, M.; Clegg, J.B.; Weatherall, D.J.
    The promoter region of the UDP glucuronosyltransferase 1 gene (UGT1A1) contains a run of thymine-adenine (TA) repeats, usually six (TA)(6). As well as its relationship to Gilbert's syndrome, homozygosity for the extended sequence, (TA)(7) (TA)(7), has been found to be an important risk factor for hyperbilirubinemia and gallstones in patients with hemoglobin E-beta-thalassemia and other intermediate forms of beta thalassemia. To assess the importance of this polymorphism in these common disorders a wide-scale population study of the relative frequency of the size alleles of the UGT1A1 promoter has been carried out. Homozygosity for the (TA)(7) allele occurs in 10-25% of the populations of Africa and the Indian subcontinent, with a variable frequency in Europe. It occurs at a much lower frequency in Southeast Asia, Melanesia, and the Pacific Islands, ranging from 0 to 5%. African populations show a much greater diversity of length alleles than other populations. These findings define those populations with a high frequency of hemoglobin E-beta-thalassemia and related disorders that are at increased risk for hyperbilirubinemia and gall bladder disease and provide evolutionary insights into how these polymorphisms have arisen and are so unequally distributed among human populations.
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    HCV and HBV infection among a cohort of Sri Lankan thalassaemic patients
    (Sri Lanka Medical Association, 2015) Perera, P.S.; Niriella, M.A.; Peries, M.A.C.; Nelumdeniya, U.B.; Dissanayake, D.M.R.; de Silva, D.S.I.; de Silva, H.J.; Premawardhena, A.P.
    INTRODUCTION AND OBJECTIVES: Previous studies suggest that prevalence of hepatitis C (HCV) and hepatitis B (HBV) virus infections is low in Sri Lanka. Patients with severe thalassaemia are at risk of developing blood borne infections like HBV and HCV. While HBV can be prevented by vaccination, safe blood donor screening practices is the best preventive strategy for HCV. Nationwide HCV blood donor screening was commenced in Sri Lanka in 2009. We studied tne prevalence of HBV and HCV Infections among a Sri Lankan cohort of thaiassaemic patients. Method: All consenting patients with J^JJJfusion dependent thalassaemia in Anuradhapura, Ragama, Baduila, Chilaw centers were screened for HBV and HCV by HBsAg and Anti-HCV antibodies respectively. Those positive during screening for HBV and HCV were confirmed by HBV-DNA and HCV-RNA PCR respectively. Results: A total of 513 patients were tested (Anuradhapura-210, Ragama-184, Badualla-70, Chilaw-49). There were no cases of HBV infection. Anti-HCV antibodies were positive in 97(45.2%), 14(7.6%), 5(7.1%), 0 in the four centres respectively, HCV was confirmed in 32 {15.2%}, 4 (2.2%), 1 (1.4%), 0 patients in the four centres. 2/4 patients from Ragama and the patient from Badualla had blood transfusions from Anuradhapura prior to changing care to present centre. HCV positive patients age ranged from 5-21 years (mean 12.5). Total transfusions ranged from 49-312. CONCLUSION: This is the first report of high HCV prevalence in a specific group to be reported from Sri Lanka, The high prevalence from a single centre (Anuradhapura} is alarming and reasons for this needs urgent investigation.
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