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This repository contains the published and unpublished research of the Faculty of Medicine by the staff members of the faculty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 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 An attempt to study the behavior of plasma Hemoglobin in post transfusion iron chelated state in beta thalassemia major patients at Thalassemia Unit Kurunegala(Faculty of Medicine, University of Kelaniya, Sri Lanka, 2021) Adikari, A.A.S.R.; Nawarathna, N.H.LA.; Fernando, K.M.I.; Abeypala, B.M.P.P.M.; Kottahachchi, D.U.; Gunathilaka, S.Introduction: Beta thalassemia major (pTM) patients require regular blood transfusions due to improper synthesis of normal hemoglobin. Objectives: The aim of this study is to make an attempt to examine the behavior of plasma hemoglobin of pTM patient in post transfusion iron chelated state. Methods: 145 patients from age 2-35 years (Male=l 13; Female=32) who were diagnosed with pTM selected for the study. Monthly haemoglobin values and 6-month Serum Ferritin (SF) values were obtained from their BHTs for two consecutive years. Correlations in between SF and haemoglobin were established using SPSS (version 20). The maximum and minimum pre-transfusion haemoglobin values (MaxPreHb and MinPreHb) were calculated for each month based on gender. Results: Most of the pre-transfusion haemoglobin values of patients lie within the 4-11 g/dl range and a statistically significant difference (p=0.021) is shown between MaxPreHb and MinPreHb. In gender wise analysis, it was shown that statistically significant differences were observed between MaxPreHb and MinPreHb of females (p=0.004) and males (p=0.013). The overall variation among MinPreHb (SD=0.81) is more compared with that of MaxPreHb (SD=0.53). The variation among MinPreHb of females and males (SD=0.71 and 1.75) is more compared with that of MaxPreHb (SD=0.40 and 1.75). However, there was no correlation between SF and haemoglobin. Conclusions: The data reveals, the group of haemoglobin around 5.0 g/dl indicates that those patients had not been compensated well by the blood transfusion. This may be due to excess haemolysis in that group. However, the other group of haemoglobin around 11.0 g/dl indicates the transfusion may have affected well.