BACKGROUND Regular blood transfusion therapy still remains the cornerstone in the management of β-thalassemia. Although recommendations are clear for patients with β-thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E β-thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with β-thalassemia major and hemoglobin E β-thalassemia. METHODS/PROCEDURE This cross-sectional study was performed among all regularly transfused patents with β-thalassemia aged 2 to 18 years attending three large thalassemia centers in Sri Lanka. Data were collected using an interviewer-administered questionnaire, perusal of clinical records, and physical examination of patients by trained doctors. RESULTS A total of 328 patients (male 47%) were recruited; 83% had β-thalassemia major, whereas 16% had hemoglobin E β-thalassemia. Sixty-one percent of patients had low pretransfusion hemoglobin levels (  200 mL/kg/year) by a majority (56%). Median pretransfusion hemoglobin was significantly lower in patients with hemoglobin E β-thalassemia compared with β-thalassemia major (P < 0.001); however, there was no difference in requirement for high transfusion volumes over 200 mL/kg/year in both groups (P = 0.14). Hepatomegaly and splenomegaly were more common in hemoglobin E β-thalassemia and were associated with lower pretransfusion hemoglobin. Transfusion requirements were higher among patients with hepatitis C and in those who are underweight. CONCLUSIONS Over 60% of regularly transfused patients with β-thalassemia have low pretransfusion hemoglobin levels despite receiving large transfusion volumes. Patients with hemoglobin E β-thalassemia are undertransfused and specific recommendations should be developed to guide transfusions in these patients.

Blood transfusion therapy for β-thalassemia major and hemoglobin E β-thalassemia: Adequacy, trends, and determinants in Sri Lanka

Stroke in Sickle cell disease is a devastating complication As Sickle cell disease is uncommon in Sri Lanka many clinicians may be unfamiliar with management of the disease and its complications A 10-year-old boy presented with a transient ischaemic attack He has had a silent large parietal infarct previously He had been managed with transfusions and had undergone a splenectomy However, he had not received hydroxyurea or undergone trans-cranial Doppler assessment

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Stroke in sickle beta thalassemia - a case report highlighting pitfalls in management in a low prevalence country

No abstract available
Sri Lanka Journal of Child Health, 2023: 52(2): 204-208

/pdf/management-of-iron-deficiency-anaemia-in-children-1hdmsfz6.pdf

Management of iron deficiency anaemia in children

Oral Presentation Abstract (OP 042), 130th Anniversary International Medical Congress, Sri Lanka Medical Association, 13th-16th July 2017 Colombo, Sri Lanka

N.N. de Silva

Papers

An islandwide hospital based epidemiological survey of haemoglobinopathies and an assessment of standards of care in 23 centres