Volume 9, Issue 3 (10-2021)
Jorjani Biomed J 2021, 9(3): 61-68 |
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Afshin F, Valizadeh M, Moradpoor R, Damandan M, Amani F. Detection of Hb Bart's and Hb H Diseases Caused by -α3.7 Prevalent Deletion Using Capillary Electrophoresis in Ardabil Province. Jorjani Biomed J 2021; 9 (3) :61-68
URL: http://goums.ac.ir/jorjanijournal/article-1-827-en.html
URL: http://goums.ac.ir/jorjanijournal/article-1-827-en.html
1- Pediatric Hematology and Oncology Department, Ardabil University of Medical Science, Ardabil, Iran
2- Unit of Genomics Research, Digestive Diseases Research Center, Ardabil University of Medical Sciences, Ardabil, Iran , mehdi_valizadeh65@yahoo.com
3- Center for Cell Pathology Research, Department of Life Science, Khazar University, Baku, Azerbaijan
4- Department of Community Medicine, Ardabil University of Medical Science, Ardabil, Iran
2- Unit of Genomics Research, Digestive Diseases Research Center, Ardabil University of Medical Sciences, Ardabil, Iran , mehdi_valizadeh65@yahoo.com
3- Center for Cell Pathology Research, Department of Life Science, Khazar University, Baku, Azerbaijan
4- Department of Community Medicine, Ardabil University of Medical Science, Ardabil, Iran
Abstract: (4826 Views)
Background and Objective: Alpha-thalassemia (α-thal) appears to be the most common monogenic disorder worldwide. The diagnosis of α-thalassemia depends on the detection of Hemoglobin Bart (Hb Bart's) in newborns, which indicates one or more defective or absent α-globin genes. In addition, in patients with Hemoglobin H (Hb H), the Hb H range usually varies between 7-10 g / dL. Therefore, tracking Hb Bart's and Hb H can be useful in diagnosing thalassemia α. This study was performed to evaluate Hb Bart's and Hb H in infants with α thalassemia in Ardabil province, northwestern Iran.
Material and Methods: In this cross-sectional descriptive study, 33 infants with alpha thalassemia mutation, including infants born in Ardabil province, Iran in the years 2020 to 2019. Hemoglobin analysis was performed by capillary electrophoresis system.
Results: Hb H and Hb Bart's were detected in only two cases (6%) and three cases (9%). In this study, only 5 patients (15.15) were observable by detection of Hb Bart's and Hb H levels by electrophoresis. In cases of Hb Bart disease, -α3.7 was the most common genotype. Therefore, most infants with alpha thalassemia were lost when electrophoresis alone was used.
Conclusion: This study showed that molecular analysis of Hb Bart's newborns is necessary to confirm α-thalassemia. Capillary electrophoresis is a way to prevent the diagnosis of rare Hb H and Bart's disease.
Material and Methods: In this cross-sectional descriptive study, 33 infants with alpha thalassemia mutation, including infants born in Ardabil province, Iran in the years 2020 to 2019. Hemoglobin analysis was performed by capillary electrophoresis system.
Results: Hb H and Hb Bart's were detected in only two cases (6%) and three cases (9%). In this study, only 5 patients (15.15) were observable by detection of Hb Bart's and Hb H levels by electrophoresis. In cases of Hb Bart disease, -α3.7 was the most common genotype. Therefore, most infants with alpha thalassemia were lost when electrophoresis alone was used.
Conclusion: This study showed that molecular analysis of Hb Bart's newborns is necessary to confirm α-thalassemia. Capillary electrophoresis is a way to prevent the diagnosis of rare Hb H and Bart's disease.
Keywords: alpha-Thalassemia [MeSH], hemoglobin Bart's [MeSH], Hemoglobin H [MeSH], Electrophoresis Capillary [MeSH]
Type of Article: Original article |
Subject:
Molecular Sciences
Received: 2021/04/24 | Accepted: 2021/09/11 | Published: 2021/09/29
Received: 2021/04/24 | Accepted: 2021/09/11 | Published: 2021/09/29
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