A nationwide survey of hospital-based thalassemia patients and standards of care and a preliminary assessment of the national prevention program in Sri Lanka.
Anuja P Premawardhana,RM Mudiyanse,Shamila T De Silva,Nilam Jiffry,Udaya B. Nelumdeniya,Udaya de Silva,Sanath P Lamabadusuriya,K.R.C. Pushpakumara,Randima Dissanayaka,M. Jansz,I Rifaya,Upul Navarathne,V. Thirukumaran,M. Arambepola,Wijesundara Dayanada Bandara,U Vaidyanatha,Devan Mendis,K.P. Weerasekara,Nalika De Silva,D K Shantha Kumara,Sujeewa D Amarasena,K K Hemantha,M.A.C.M. Refai,Ishari Silva,Nizri Hameed,F Rajiyah,Sachith Mettananda,Angela Allen,David J. Weatherall,Nancy F Oliveri +29 more
TLDR
Central coordination of the treatment and ultimately prevention of thalassemia is urgently needed in Sri Lanka and development of expert centers with designated staff with sufficient resources will improve the quality of care.Abstract:
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.read more
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World distribution, population genetics, and health burden of the hemoglobinopathies
TL;DR: In this paper, the inherited hemoglobin disorders pose an increasing burden on global health resources in the future, due to natural selection combined with a high frequency of consanguineous marriages in many countries, together with an epidemiological transition.
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The parental perspective of thalassaemia in Bangladesh: lack of knowledge, regret, and barriers.
TL;DR: Investigation of parental knowledge gaps and perceptions regarding thalassemia in Bangladesh suggests poor parental knowledge on thalassaemia including prenatal diagnosis and the challenges they are facing while caring for their children.
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A randomised double-blind placebo-controlled clinical trial of oral hydroxyurea for transfusion-dependent β-thalassaemia
Nirmani Yasara,Nethmi Wickramarathne,Chamila Mettananda,Ishari Silva,Nizri Hameed,K Attanayaka,Rexan Rodrigo,Nirmani Wickramasinghe,Lakshman Perera,A. Manamperi,Anuja Premawardhena,Sachith Mettananda +11 more
TL;DR: A randomized, double-blind, placebo-controlled clinical trial to evaluate the efficacy and safety of hydroxyurea in transfusion-dependent β-thalassaemia was conducted in this article .
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Prevalence of Transfusion Transmitted Infections and the Quality of Life in β-thalassemia Major Patients.
TL;DR: The study concludes that transfusion-transmitted disease is very high and that HCV is the leading TTI followed by HBV and HIV, and QoL in patients with TTIs was poor.
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Psychological morbidity among children with transfusion dependent β-thalassaemia and their parents in Sri Lanka.
Sachith Mettananda,Ravindu Peiris,Hashan Pathiraja,Miyuru Chandradasa,Dayananda Bandara,Udaya de Silva,Chamila Mettananda,Anuja Premawardhena +7 more
TL;DR: Mothers of the children with transfusion dependent β-thalassaemia had significantly higher depressive symptoms which were significantly associated with psychological symptoms among children.
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