Dataset Information

Body iron status of children and adolescents with transfusion dependent ?-thalassaemia: trends of serum ferritin and associations of optimal body iron control.

ABSTRACT:

Objective

This cross sectional study aims to describe the body iron status, trends of serum ferritin and associations of optimal body iron control in patients aged below 16 years with transfusion dependent ?-thalassaemia attending Paediatric and Adolescent Thalassaemia Centres of the Colombo North Teaching Hospital of Sri Lanka.

Results

Out of 54 children, 51% were males and a majority were aged 11-16 years; 83% had ?-thalassaemia major while 13% had HbE ?-thalassaemia. Mean serum ferritin was 1778(±?1458) µg/l and 29% had optimal serum ferritin (below 1000 µg/l). Trend of mean serum ferritin over time showed gradual decline between 2011 and 2017 and longitudinal trend of individual patients at yearly intervals showed gradual rise until 5 years of age and plateauing thereafter. All except two patients were receiving iron chelator medication of which the most commonly used was oral deferasirox (92%). The most common iron-related complications were short stature (24.1%) and pubertal delay (42.8% of?>?14 years). None of the patients had hypothyroidism, hypoparathyroidism or diabetes. Optimal serum ferritin levels were significantly associated with the diagnosis of thalassaemia at a later age (23.6 vs 9.0 months) and higher family income (OR-4.81;95%CI 1.17-19.67) however was not associated with the age of the patient or duration of transfusion.

SUBMITTER: Suriapperuma T

PROVIDER: S-EPMC6071405 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature

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Publications

Body iron status of children and adolescents with transfusion dependent β-thalassaemia: trends of serum ferritin and associations of optimal body iron control.

Suriapperuma Tharindi T Peiris Ravindu R Mettananda Chamila C Premawardhena Anuja A Mettananda Sachith S

BMC research notes 20180802 1

<h4>Objective</h4>This cross sectional study aims to describe the body iron status, trends of serum ferritin and associations of optimal body iron control in patients aged below 16 years with transfusion dependent β-thalassaemia attending Paediatric and Adolescent Thalassaemia Centres of the Colombo North Teaching Hospital of Sri Lanka.<h4>Results</h4>Out of 54 children, 51% were males and a majority were aged 11-16 years; 83% had β-thalassaemia major while 13% had HbE β-thalassaemia. Mean serum ...[more]

PMID: 30071883

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Project description:BackgroundHydroxyurea (hydroxycarbamide) promotes the production of foetal haemoglobin (HbF) by reactivating gamma-genes. Evidence has shown clinical benefits of hydroxyurea in people with sickle cell anemia; however, only a few studies have assessed this treatment in people with beta (β)-thalassaemia.ObjectivesThe primary objective is to review the efficacy of hydroxyurea in reducing or ameliorating the requirement of blood transfusions in people with transfusion-dependent β-thalassaemia. The second objective is to review the safety of hydroxyurea with regards to severe adverse effects in this population.Search methodsWe searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, compiled from electronic database searches and hand searching of journals and conference abstract books. We also searched electronic databases and trial registries, including ClinicalTrials.gov, the WHO ICTRP and PubMed (09 October 2018).Date of last search of the Group's haemoglobinopathies trials register: 04 March 2019.Selection criteriaRandomised controlled trials of hydroxyurea in people with transfusion-dependent β-thalassaemia, compared with placebo or standard treatment or comparing different doses of hydroxyurea.Data collection and analysisTwo authors independently assessed trials for inclusion in the review, which was verified by a third author.Main resultsNo trials were eligible for inclusion in this review.Authors' conclusionsCurrently, there is no high-quality evidence to support or challenge the continued use of hydroxyurea for managing people with transfusion-dependent β-thalassaemia. Multicentre, randomised controlled trials (compared to placebo or other available treatment, i.e. blood transfusion and iron chelation) are needed in order to assess the efficacy and safety of hydroxyurea for reducing the need for blood transfusion, for maintaining or improving mean haemoglobin levels, as well as for determining its cost-effectiveness.