Abstract

IntroductionDetermination of hemoglobins (Hbs) F, A2, and E is crucial for diagnosis of thalassemia. This study determined the levels of Hbs F, A2, and E in children aged 6‐23 months and investigated the effect of age, sex, and types of thalassemia on the expression of these Hbs.MethodsA total of 698 blood samples of Laotian children including 272 non‐Hb E, 271 Hb E heterozygotes, and 155 Hb E homozygotes were collected. Hb profiles were determined using the capillary zone electrophoresis. Coinheritance of α‐thalassemia and the homozygosity for Hb E mutation were checked by PCR‐based assay.ResultsChildren heterozygous and homozygous for Hb E had significantly higher Hb F and A2 levels than non‐Hb E children (median Hb F = 1.1% for non‐Hb E group, 2.7% for Hb E heterozygotes, and 9.4% for Hb E homozygotes; median Hb A2 = 2.6% for non‐Hb E group, 3.8% for Hb E heterozygotes, and 5.2% for Hb E homozygotes). The median Hb E levels were 21.9% for Hb E heterozygotes and 85.3% for Hb E homozygotes. Comparing within group, there was a statistically significant difference between children with and without an α‐gene defect for Hb A2 and E, but not Hb F. Based on a multiple regression analysis, age and sex were significantly associated with the expression of Hb F and A2 but not Hb E.ConclusionsOur findings can guide the development of a diagnostic approach to thalassemia in children aged 6‐23 months.

Highlights

  • Determination of hemoglobins (Hbs) F, A2, and E is crucial for diagnosis of thalassemia

  • TA B L E 2 Results of multiple regression analysis demonstrating the effect of age, sex, and thalassemia gene on the expression of Hemoglobin F (Hb F) (Adjusted R2 = .5394)

  • This study reports for the first time the levels of Hb F, A2, and E in Laotian children aged 6-23 months with and without Hb E

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Summary

| INTRODUCTION

Hb F normally declines gradually and reaches normal levels of less than 1% of total hemoglobin at the age of around 10 months.[1]. Its expression increases gradually and has been shown to reach a normal adult level at age 5-6 months.[2]. Inherited disorders of hemoglobin could result in varying levels of Hb F and A2 depending mainly on the types of the affected gene. In addition to thalassemia types, it is questionable for children under 24 months whether age and sex have a significant effect on the expression of these hemoglobins. We used data from the “Lao Zinc study” a community-based intervention trial among young Laotian children 6-23 months of age at enrollment to evaluate the effect of age, sex, and different types of thalassemias on the expression of Hb F, A2, and E in children with and without Hb E

| MATERIALS AND METHODS
| Laboratory methods for detection of thalassemia
Findings
| DISCUSSION
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