Factors related with low QoL in current study were higher age, increased transfusion frequency and injectable mode of chelation. In order to improve the QoL in thalassemic children appropriate programmes and interventions should be started targeting above domains.
Folate deficiency is common in epileptic children on long term antiepileptic drugs (AEDs), contributes to poor seizure control and should be considered in the etiologic differentials of drug resistant epilepsy. Folate supplementation improves seizure control in these children.
Hb J α mutation is a rare haemoglobin variant. No case of Hb J α mutation with hemophilia A has been reported. Here we are reporting a rare variant of Hb J α mutation found accidentally in our patient in which electrophoresis done to find out the cause of severe anemia with hepato-splenomegaly and association of this hemoglobin variant with Hemophilia A.
CONTEXT:T2* magnetic resonance imaging (MRI) is being increasingly used for the assessment of organ iron content in thalassemics, but cost is a major prohibitive factor for repeated measurements. If serum ferritin correlates well with the T2* MRI liver and heart, it will be economical and more simple tool to assess organ iron deposition.AIMS:The aim of this study was to find out the relationship between serum ferritin level and T2* MRI-derived liver and heart iron content in transfusion-dependent thalassemic childrenSETTINGS:Thalassemia day-care center of a teaching hospitalDESIGN:This was a cross-sectional studySUBJECTS AND METHODS:Seventy-three transfusion-dependent beta thalassemic children belonging to 2–18 years of age were subjected to T2* MRI of heart and liver to assess their iron content. Values obtained here were related to serum ferritin.STATISTICAL ANALYSIS USED:Keeping the correlation between serum ferritin and T2* MRI as primary outcome, spearman's correlation coefficient was calculated.RESULTS:We found poor (negative) correlation between serum ferritin level and T2* MRI liver (r = -0.448, P = 0.000) but no correlation between serum ferritin and T2*MRI heart (r = -0.221, P = 0.060).Conclusions:Serum ferritin cannot reliably predict the liver and heart iron content in Indian children with β thalassemia.
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