Clinical Manifestation of β-Thalassemia/Hemoglobin E Disease
ชื่อเรื่อง
Clinical Manifestation of β-Thalassemia/Hemoglobin E Disease
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ผู้สร้าง/เจ้าของผลงาน
Fucharoen, Suthat
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Ketvichit, Phairawh
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Pootrakul, Pensri
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Siritanaratkul, Noppadol
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Piankijagum, Anong
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Wasi, Prawase
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หัวเรื่อง
Thalassemia
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Hemoglobin
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คำอธิบายรายละเอียดของเอกสาร
This article was supported by grants from Prajadhipok-Rambhai Barni Foundation and National Center for Genetic Engineering and Biotechnology.
บทคัดย่อ
Purpose
To review the clinical manifestation and changes in hematologic parameters of patients with β-thalassemia/hemoglobin (Hb) E.
Materials and Methods
Retrospective analysis of the clinical manifestation of 378 patients with β-thalassemia/Hb E attending the hematology clinic at Siriraj Hospital between 1957 and 1982.
Results
A wide spectrum of clinical phenotypes has been observed. Most patients show clinical symptoms by 10 years of age. The majority of patients survive with or without occasional transfusion. Splenectomy was performed in 26.5% of patients. Patients come to the hospital because of anemia, fever, abdominal mass, and jaundice. Gastrointestinal tract disturbances are the most common presenting symptoms (34.6%), especially abdominal pain (10%) and cholecystitis (5.1%). Respiratory tract infections were found in 21.8% of patients and cardiovascular complications, including congestive heart failure, occurred in 11.9%. Other less common symptoms and complications included bone pain, chronic leg ulcers, paraplegia and hypertension-associated convulsions, and cerebral hemorrhage after multiple blood transfusion. Patients usually die between 20 to 40 years of age (67%), mainly from congestive heart failure and septicemia. Septicemia was often caused by Gram-negative bacteria.
Conclusion
These clinical features observed in patients with β-thalassemia/Hb E are probably the results of chronic anemia and iron overload. The study of the life history and clinical courses of patients with β-thalassemia/Hb E should provide important information for the better management of these patients.
To review the clinical manifestation and changes in hematologic parameters of patients with β-thalassemia/hemoglobin (Hb) E.
Materials and Methods
Retrospective analysis of the clinical manifestation of 378 patients with β-thalassemia/Hb E attending the hematology clinic at Siriraj Hospital between 1957 and 1982.
Results
A wide spectrum of clinical phenotypes has been observed. Most patients show clinical symptoms by 10 years of age. The majority of patients survive with or without occasional transfusion. Splenectomy was performed in 26.5% of patients. Patients come to the hospital because of anemia, fever, abdominal mass, and jaundice. Gastrointestinal tract disturbances are the most common presenting symptoms (34.6%), especially abdominal pain (10%) and cholecystitis (5.1%). Respiratory tract infections were found in 21.8% of patients and cardiovascular complications, including congestive heart failure, occurred in 11.9%. Other less common symptoms and complications included bone pain, chronic leg ulcers, paraplegia and hypertension-associated convulsions, and cerebral hemorrhage after multiple blood transfusion. Patients usually die between 20 to 40 years of age (67%), mainly from congestive heart failure and septicemia. Septicemia was often caused by Gram-negative bacteria.
Conclusion
These clinical features observed in patients with β-thalassemia/Hb E are probably the results of chronic anemia and iron overload. The study of the life history and clinical courses of patients with β-thalassemia/Hb E should provide important information for the better management of these patients.
ผู้ร่วมงาน
Thalassemia Research Center, Mahidol University
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Institute of Science and Technology for Research and Development, Mahidol University
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Division of Hematology, Faculty of Medicine Siriraj Hospital, Mahidol University
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Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University
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ปีที่ผลิตเอกสาร
วันที่บันทึกข้อมูล
2023-05-03
ประเภท
Article
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รูปแบบ
ขนาดหรือจำนวนของเอกสาร
6 pages
ต้นฉบับ/แหล่งที่มาของเอกสาร
Journal of Pediatric Hematology/Oncology ; volume 22, issue 6, page 552-557
ภาษา
รหัส หรือตัวบ่งชี้เอกสาร
ขอบเขต
เจ้าของลิขสิทธิ์
Wolters Kluwer Health, Inc.
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