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

Abstract

ObjectiveThis 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.ResultsOut 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 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.Electronic supplementary materialThe online version of this article (10.1186/s13104-018-3634-9) contains supplementary material, which is available to authorized users.

Major Depression in Children with β-Thalassemia Major is Strongly Associated with the Number of Blood Transfusions, Iron Overload and Increased Levels of Interleukin-1β

“…Our study showed that blood transfusions were significantly associated with increased IO in β-TM, including higher serum iron, TS%, and ferritin as compared with controls. Our results are in line with previous results reporting a significant elevation in serum iron in β-TM patients as compared with healthy controls [25,54]. The predominant mechanisms driving the iron loading process includes transfusion therapy and enhanced intestinal absorption as a consequence of the impairments in erythropoiesis [51].…”

“…The predominant mechanisms driving the iron loading process includes transfusion therapy and enhanced intestinal absorption as a consequence of the impairments in erythropoiesis [51]. Increased ferritin levels are associated with iron-related damage to tissues including liver and heart [54], which is not only related to increased oxidative stress but also to the direct effects of IO on the tissues.…”

Health related quality of life among children with transfusion dependent β-thalassaemia major and haemoglobin E β-thalassaemia in Sri Lanka: a case control study

“…However, a vast majority (over two-thirds of patients) are managed in three large tertiary referral centres, namely Kurunegala, Anuradhapura and Ragama thalassaemia centres [17]. All three centres have unrestricted access to blood products through a state-run National Blood Transfusion service, provide iron chelation medication to all patients free of charge and follow the TIF guidelines, hence, can be considered as units providing optimal care for patients with TDBT [18].…”

Cytokine Dependent Hematopoietic Cell Linker (CLNK) is Highly Elevated in Blood Transfusion Dependent Beta-Thalassemia Major Patients

“…These results are also in accordance with those of previous studies. 3,27 The predominant mechanism driving the iron loading process includes transfusion therapy and enhanced intestinal absorption secondary to ineffective erythropoiesis. 2 Serum ferritin measurement and noninvasive imaging techniques are available to diagnose iron overload, quantify its extent in different organs, and monitor clinical response to therapy.…”

“…2 Higher levels of serum ferritin (especially over 2500 µg/l) are associated with iron-related organ failure particularly in the liver and heart. 27 This organ damage is not only related to oxidative stress end products, 18 but also to the direct effect of iron overload on the tissues. In the current study, serum CLNK was significantly associated with number of transfusions, ferritin and iron.…”