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This repository contains the published and unpublished research of the Faculty of Medicine by the staff members of the faculty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 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.Item 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.Item 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.Item 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.Item A woman with unilateral thigh pain, moderate leukocytosis and extreme thrombocytosis (Case report 2)(The Sri Lanka College of Haematologists, 2019) Dilhani, M.N.; Jayaweera, W.M.N.M.; Williams, H.S.A.; Premawardhena, A.P.ABSTRACT: Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm (MPN) of hematopoietic stem cells characterized by the presence of the Philadelphia (Ph) chromosome (t (9;22) (q34.1;qll.2), in which granulocytes are the major proliferative component. Atypical presentations of chronic phase CML reported include extreme thrombocytosis with or without leukocytosis, marked megakaryocytic proliferation or significant marrow fibrosis. We describe a middle-aged woman who presented with nonspecific leg pain and abnormal FBC parameters. Moderate leukocytosis (WBC 55 x 103/ pl), mild anemia and marked thrombocytosis (1,615 xl03/pl) were noted without clinical or laboratory features of ongoing infection or inflammation. Clinical examination revealed mild pallor only. A prominent spleen was noted ultrasonically (13.5 cm). A leucoerythroblastic blood picture was seen with moderate neutrophil leukocytosis, tear drop poikilocytes and one percent blast cells. No characteristic myelocytes and neutrophil peaks were present, and dysplasia was absent. Mild eosinophilia was observed however basophilia was absent. Platelets were markedly increased with anisocytosis. An underlying MPN suspected. JAK2V617 F mutation was absent and BCR-ABL (transcript type- b3a2) mutation detected. Her bone marrow biopsy revealed markedly hyper- cellular fragments with suppressed erythropoiesis, markedly hypercellular granulopoiesis (blasts <5%) with prominent eosinophil precursors. Megakaryocyte numbers were markedly increased with numerous micro megakaryocytes. The trephine biopsy displayed a few loose clusters of megakaryocytes. A marked background fibrosis noted (Grade (3/3). The diagnosis of chronic phase- CML was made. She was commenced on Imatinib 400mg daily and she achieved and maintained all the desired milestones since diagnosis.Item Marriage patterns in Sri Lanka and the prevalence of parental consanguinity in patients with β-thalassaemia: a cross-sectional descriptive analysis(Cambridge University Press, 2020) Premawardhena, A.P.; de Silva, S.T.; Goonatilleke, M.D.D.C.; Ediriweera, D.S.; Mettananda, S.; Rodrigo, B.K.R.P.; Allen, A.; Weatherall, D.J.Consanguineous marriages potentially play an important role in the transmission of β-thalassaemia in many communities. This study aimed to determine the rate and socio-demographic associations of consanguineous marriages and to assess the influence on the prevalence of β-thalassaemia in Sri Lanka. Three marriage registrars from each district of Sri Lanka were randomly selected to prospectively collect data on all couples who registered their marriage during a 6-month period starting 1st July 2009. Separately, the parents of patients with β-thalassaemia were interviewed to identify consanguinity. A total of 5255 marriages were recorded from 22 districts. The average age at marriage was 27.3 (±6.1) years for males and 24.1 (±5.7) years for females. A majority (71%) of marriages were 'love' marriages, except in the Moor community where 84% were 'arranged' marriages. Overall, the national consanguinity rate was 7.4%. It was significantly higher among ethnic Tamils (22.4%) compared with Sinhalese (3.8%) and Moors (3.2%) (p < 0.001). Consanguinity rates were also higher in 'arranged' as opposed to 'love' marriages (11.7% vs 5.6%, p < 0.001). In patients with β-thalassaemia, the overall consanguinity rate was 14.5%; it was highest among Tamils (44%) and lowest among Sinhalese (12%). Parental consanguinity among patients with β-thalassaemia was double the national average. Although consanguinity is not the major factor in the transmission of the disease in the country, emphasis should be given to this significant practice when conducting β-thalassaemia prevention and awareness campaigns, especially in high-prevalence communities.Item A nationwide survey of hospital-based thalassemia patients and standards of care and a preliminary assessment of the national prevention program in Sri Lanka(Public Library of Science, 2019) Premawardhena, A.P.; Mudiyanse, R.; de Silva, S.T.; Jiffry, N.; Nelumdeniya, U.; de Silva, U.; Lamabadusuriya, S.P.; Pushpakumara, K.; Dissanayaka, R.; Jansz, M.; Rifaya, I.; Navarathne, U.; Thirukumaran, V.; Arambepola, M.; Bandara, W.D.; Vaidyanatha, U.; Mendis, D.; Weerasekara, K.; de Silva, N**.; Kumara, D.K.S.; Amarasena, S.D.; Hemantha, K. K.; Refai, M.A.C.M.; Silva, I.; Hameed, N.; Rajiyah, F.; Mettananda, S.; Allen, A.; Weatherall, D. J.; Oliveri, N. F.OBJECTIVES:Our aim was to describe the numbers and distribution of patients with different types of thalassemia and to assess the standards of care in all thalassemia treatment centers throughout Sri Lanka and the success of the ongoing prevention programme.METHODS:This cross-sectional island-wide survey was conducted by two trained medical graduates, who visited each thalassemia center to collect data from every patient, using a standardized form. Data was collected through review of patient registers and clinical records.RESULTS: We collected data on 1774 patients from 23 centers. 1219 patients (68.7%) had homozygous β-thalassemia, 360 patients (20.3%) had hemoglobin E β-thalassemia, and 50 patients (2%) had sickle β-thalassemia. There were unacceptably high serum ferritin levels in almost all centers. The annual number of births of patients with β-thalassaemia varied between 45-55, with little evidence of reduction over 19 years. CONCLUSIONS:Central coordination of the treatment and ultimately prevention of thalassemia is urgently needed in Sri Lanka. Development of expert centers with designated staff with sufficient resources will improve the quality of care and is preferred to managing patients in multiple small units.Item Stroke in sickle cell beta thalassemia - a case report highlighting pitfalls in management in a low prevalence country(Kandy Society of Medicine, 2017) Premathilaka, L.H.R.A.; Lakmini, M.S.; Thamal Dharshana, L.G.; Nawaratne, S.B.; Mettananda, S.; de Silva, S.T.; Premawardhena, A.P.Stroke in Sickle cell disease is a devastating complication. As Sickle cell disease is uncommon in Sri Lanka many clinicians may be unfamiliar with management of the disease and its complications. A 10-year-old boy presented with a transient ischaemic attack. He has had a silent large parietal infarct previously. He had been managed with transfusions and had undergone a splenectomy. However, he had not received hydroxyurea or undergone trans-cranial Doppler assessment.Item Iron status and anaemia in Sri Lankan secondary school children: A cross-sectional survey(Public Library of Science, 2017) Allen, A.; Allen, S.; Rodrigo, R.; Perera, L.; Shao, W.; Li, C.; Wang, D.; Oliviery, N.; Weatherall, D.J.; Premawardhena, A.P.BACKGROUND: Iron deficiency, the most common micronutrient disorder and cause of anaemia globally, impairs growth, cognition, behaviour and resistance to infection. METHODS/RESULTS: As part of a national survey of inherited haemoglobin variants in 7526 students from 72 secondary schools purposefully selected from the 25 districts of Sri Lanka, we studied 5912 students with a normal haemoglobin genotype. Median age was 16.0 (IQR 15.0-17.0) years and 3189 (53.9%) students were males. Most students were Sinhalese (65.7%), with fewer Tamils (23.1%) and Muslims (11.2%). Anaemia occurred in 470 students and was more common in females (11.1%) than males (5.6%). Haemoglobin, serum ferritin, transferrin receptor and iron were determined in 1196 students with low red cell indices and a structured sample of those with normal red cell indices (n = 513). The findings were weighted to estimate the frequencies of iron deficiency and iron deficiency anaemia classified according to WHO criteria. Iron depletion (serum ferritin <15ug/ml) occurred in 19.2% and cellular iron deficiency (low serum ferritin and transferrin receptor >28.1 nmol/l) in 11.6% students. Iron deficiency anaemia (cellular iron deficiency with low haemoglobin) occurred in only 130/2794 (4.6%) females and 28/2789 (1.0%) males. Iron biomarkers were normal in 83/470 (14.6%) students with anaemia. In multiple regression analysis, the odds for iron depletion and cellular iron deficiency were about one-third in males compared with females, and the odds for iron deficiency anaemia were about one fifth in males compared to females. Tamil ethnicity and age <16 years increased the risk of all three stages of iron deficiency and living at high altitude significantly reduced the risk of iron depletion. CONCLUSIONS: Low iron status and anaemia remain common problems in Sri Lankan secondary school students especially females, younger students and the socioeconomically disadvantaged Tamil population. More research is needed to identify factors other than low iron status that contribute to anaemia in adolescents.