Concomitant inheritance of α-thalassemia in β°-thalassemia/hb e disease
ชื่อเรื่อง
Concomitant inheritance of α-thalassemia in β°-thalassemia/hb e disease
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ผู้สร้าง/เจ้าของผลงาน
Winichagoon, Pranee
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Fucharoen, Suthat
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Weatherall, David
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Wasi, Prawase
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หัวเรื่อง
Hematology
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คำอธิบายรายละเอียดของเอกสาร
This study was supported by a US. Public Health Service research grant AM
09805 from the National Institute of Arthritis, Diabetes, Digestive and Kidney
Diseases.
09805 from the National Institute of Arthritis, Diabetes, Digestive and Kidney
Diseases.
บทคัดย่อ
Concomitant inheritance of α-thalassemia in patients with β°-thalassemia/hemoglobin (Hb) E disease was detected by restriction endonuclease DNA mapping. Among 42 patients with β°-thalassemia/Hb E disease, seven were found to have an α-thalassemia-2 haplotype. Of these, five belonged to the rightward or 3.7-kb type of α-thalassemia-2 and the remaining two the leftward or 4.2-kb type. All the seven patients with α-thalassemia-2 haplotype had hemoglobin levels of 7.4 g/dl or above; those without detectable α-thalassemia had hemoglobin levels both higher and lower than 7.4 g/dI. The latter attended the clinic regularly, the former did occasionally. These findings suggest that concomitant inheritance of α-thalassemia can alleviate the severity of β°-thalassemia/Hb E disease. Failure to find α-thalassemia-1 haplotype in these patients suggests that concomitant inheritance of α-thalassemia-1 with β°-thalassemia/Hb E might lead to so mild a condition that the individuals do not present clinically. The fact that many patients without a detectable α-thalassemia haplotype also had hemoglobin levels of 7.4 g/dl or higher suggests that there are additional factors responsible for the mildness of β°-thalassemia/Hb E disease.
ผู้ร่วมงาน
Department of Medicine, Faculty of Medicine, Siriraj Hospital
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The Thalassemia Center, Faculty of Graduate Studies, Mahidol University, Bangkok, Thailand
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MRC Molecular Hematology Unit, Nuffield Department of Clinical Medicine, John Radcliffe Hospital, Oxford, England
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ปีที่ผลิตเอกสาร
วันที่บันทึกข้อมูล
2023-05-02
ประเภท
Article
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รูปแบบ
ขนาดหรือจำนวนของเอกสาร
6 pages
ต้นฉบับ/แหล่งที่มาของเอกสาร
American Journal of Hematology ; volume 20, issue 3, page 217-222
ภาษา
รหัส หรือตัวบ่งชี้เอกสาร
ขอบเขต
เจ้าของลิขสิทธิ์
John Wiley & Sons, Inc.
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