Molecular basis and hematologic characterization of δβ-thalassemia and hereditary persistence of fetal hemoglobin in Thailand
نویسندگان
چکیده
From the Graduate School (SP), Department of Clinical Chemistry (SP, SF) and Department of Clinical Microscopy, Faculty of Associated Medical Sciences, Khon Kaen University, Khon Kaen, Thailand (GF, KS), Department of Clinical Pathology, Maharaj Hospital, Nakornsrithammaraj, Thailand (SS), Center for Research and Development of Medical Diagnostic Laboratories, Khon Kaen University, Khon Kaen, Thailand (SP, SF, GF).
منابع مشابه
Homozygous delta-beta Thalassemia in a Child: a Rare Cause of Elevated Fetal Hemoglobin
Abstract Background Delta beta (δβ) thalassemia is an unusual variant of thalassemia with elevated level of fetal hemoglobin (HbF). Homozygous patients of this disorder, unlike β-thalassemia, show mild anemia. Only few cases of δβ-thalassemia have been reported from India in the available indexed English literature. Case presentation A four-year old male child was evaluated for recent-onset...
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Fetal hemoglobin (Hb F) is characteristic of the fetal development period. However, in some genetic conditions, such as hereditary persistence of fetal hemoglobin (HPFH) and delta-beta thalassemia (δβ-thalassemia), Hb F continues to be produced in adulthood. We evaluated the frequency of two mutations of HPFH, HPFH-1 and HPFH-2 African, and two mutations in δβ-thalassemia, Sicilian and Spa...
متن کاملWhat influences Hb fetal production in adulthood?
Human hemoglobin genes are located in α and β globin gene clusters in chromosomes 16 and 11, respectively. Different types of hemoglobin are synthesized according to the stage of development with fetal hemoglobin (α(2)γ(2)) (Hb F) being the main hemoglobin in the fetal period. After birth, there is a reduction (to about 1%) in Hb F levels and adult hemoglobin, Hb A (2α(2)β(2)), increases to mor...
متن کاملHaematological characterisation and molecular basis of asian Indian inversion deletions delta Beta thalassemia: a case report.
The hereditary persistence of fetal hemoglobin (HPFH) and delta beta thalassemia are heterogeneous disorders characterised by increased levels of fetal hemoglobin and high level of this Hb continues in adulthood. The distinction between these two conditions is not always possible with routine hematologic analysis and molecular characterisation of the defect is required. We encountered such a ra...
متن کاملNature of fetal hemoglobin in the Greek type of hereditary persistence of fetal hemoglobin with and without concurrent beta-thalassemia.
The fetal hemoglobin in the affected members of three Greek families with the hereditary persistence of fetal hemoglobin has only gamma-chains of the type with alanine in position 136. Although certain Negro families had been considered to have only this type of gamma-chains in their fetal hemoglobin, further studies required that they be reclassified. Consequently, the Greek cases are the sole...
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تاریخ انتشار 2004