Browsing by Author "Ramees, L."

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The Evolutionary and clinical implications of the uneven distribution of the frequency of the inherited haemoglobin variants over short geographical distances
(Wiley-Blackwell, 2017) Premawardhena, A.; Allen, A.; Piel, F.; Fisher, C.; Perera, L.; Rodrigo, R.; Goonathilaka, G.; Ramees, L.; Peto, T.; Olivieri, N.; Weatherall, D.
Studies of the frequency of heterozygous carriers for common inherited diseases of haemoglobin in over 7500 adolescent children in 25 districts in Sri Lanka have disclosed a highly significant variation over very short geographical distances. A further analysis of these findings, including their relationship to the past frequency and distribution of malaria, climatic variation, altitude, ethnic origin and consanguinity rates, have provided evidence regarding the evolutionary basis for the variable distribution of these conditions over short distances. It is likely that the complex interplay between malaria and the environment, together with related ethnic and social issues, exists in many countries across the tropical belt. Hence, these observations emphasise the importance of micromapping heterozygote distributions in high-frequency countries in order to define their true burden and the facilities required for the prevention and management of the homozygous and compound heterozygous disorders that result from their interaction.
Hepcidin detects iron deficiency in Sri Lankan adolescents with a high burden of hemoglobinopathy: A diagnostic test accuracy study
(Wiley-Blackwell, 2017) Wray, K.; Allen, A.; Evans, E.; Fisher, C.; Premawardhena, A.; Perera, L.; Rodrigo, R.; Goonathilaka, G.; Ramees, L.; Webster, C.; Armitage, A.E.; Prentice, A.M.; Weatherall, D.J.; Drakesmith, H.; Pasricha, S.R.
Anemia affects over 800 million women and children globally. Measurement of hepcidin as an index of iron status shows promise, but its diagnostic performance where hemoglobinopathies are prevalent is unclear. We evaluated the performance of hepcidin as a diagnostic test of iron deficiency in adolescents across Sri Lanka. We selected 2273 samples from a nationally representative cross-sectional study of 7526 secondary schoolchildren across Sri Lanka and analyzed associations between hepcidin and participant characteristics, iron indices, inflammatory markers and hemoglobinopathy states. We evaluated the diagnostic accuracy of hepcidin as a test for iron deficiency with estimation of the AUCROC , sensitivity/specificity at each hepcidin cutoff, and calculation of the Youden Index to find the optimal threshold. Hepcidin was associated with ferritin, sTfR and hemoglobin. The AUCROC for hepcidin as a test of iron deficiency was 0.78; hepcidin outperformed Hb and sTfR. The Youden index-predicted cutoff to detect iron deficiency (3.2ng/mL) was similar to thresholds previously identified to predict iron utilization and identify deficiency in African populations. Neither age, sex, nor α- or β-thalassemia trait affected diagnostic properties of hepcidin. Hepcidin pre-screening would prevent most iron-replete thalassemia carriers from receiving iron whilst still ensuring most iron deficient children were supplemented. Our data indicate that the physiological relationship between hepcidin and iron status transcends specific populations. Measurement of hepcidin in individuals or populations could establish the need for iron interventions. This article is protected by copyright. All rights reserved.
Marriage patterns in Sri Lanka and the role of consanguinity in thalassaemia: A cross sectional descriptive analysis
(Sri Lanka Medical Association, 2019) Goonatilleke, M.D.D.C.; de Silva, S.T.; Rodrigo, R.; Perera, S.; Goonathilaka, G.W.M.R.G.B.; Ramees, L.; Mettananda, S.; Weatherall, D.L.; Premawardhena, A.P.
INTRODUCTION & OBJECTIVES: Consanguineous marriages play an important role in the propagation of thalassaemia. There is a paucity of data on consanguineous marriages in modern Sri Lankan society and its effect on thalassaemia births. We studied sociological aspects of Sri Lankan marriages with emphasis on consanguinity, and the prevalence of consanguinity in the parents of patients with thalassaemia. METHODS: Three marriage registrars from each district were asked to record details about the partners of all marriages they registered for 6 months, from July 2009. Parents of patients with thalassemia were interviewed separately, to identify any consanguinity. RESULTS: A total of 5249 marriages were recorded from 22 districts. Average age at marriage was 27.2 9 years for males and 24.0 8 years for females. 3737/5249 (71.2%) of all marriages were Love Marriages, except in the Moor community where 89.2% were Arranged Marriages. Female literacy and education tier levels were higher than in males. Overall national consanguinity rate was 6.4% (337/5249). It was highest among Tamils (20.4%), but only 3.3% among Sinhalese and 3.1% among Moors. In the parents of 405 patients with thalassaemia, overall consanguinity rate was 11.1% (45/405): it was highest among Tamils (33.3%) and lowest among Sinhalese (9.4%). CONCLUSION: Consanguinity among patients with Thalassaemia was almost double the national average. Though not the dominant cause, more emphasis should be given to consanguinity when conducting thalassaemia prevention campaigns. Since they are older and better educated at marriage, female partners should be better targeted in such health education campaigns.