Hereditary hemolytic anemia in Korea from 2007 to 2011: A study by the Korean hereditary hemolytic anemia working party of the Korean Society of Hematology

Title
Hereditary hemolytic anemia in Korea from 2007 to 2011: A study by the Korean hereditary hemolytic anemia working party of the Korean Society of Hematology
Author(s)
하정옥박은실[박은실]Hye Lim Jung[Hye Lim Jung]Hee -Jin Kim[Hee -Jin Kim]Sung Sup Park[Sung Sup Park]Sang Hoon Song[Sang Hoon Song]Hee Young Shin[Hee Young Shin]Kyung Nam Koh[Kyung Nam Koh]Chuhi Joo Lyu[Chuhi Joo Lyu]Young Tak Lim[Young Tak Lim]배순환
Keywords
folic acid; adolescent; alpha thalassemia; article; beta thalassemia; child; erythrocyte; family history; female; globin gene; hemoglobinopathy; hereditary hemolytic anemia; human; inborn error of metabolism; incidence; infant; Korea; laboratory test; major clinical study; male; medical society; preschool child; prevalence; questionnaire; school child; splenectomy; symptomatology; transfusion
Issue Date
201309
Citation
Blood Research, v.48, no.3, pp.211 - 216
Abstract
Background: The number of patients diagnosed with hereditary hemolytic anemia (HHA) has increased since the advent of novel diagnostic techniques that accurately identify this disorder. Here, we report data from a survey on the prevalence and characteristics of patients diagnosed with HHA in Korea from 2007 to 2011. Methods: Information on patients diagnosed with HHA in Korea and their clinical and laboratory results were collected using a survey questionnaire. Globin gene and red blood cell (RBC) enzyme analyses were performed. In addition, we analyzed data collected by pediatricians. Results: In total, 195 cases of HHA were identified. Etiologies identified for HHA were RBC membranopathies, hemoglobinopathies, and RBC enzymopathies, which accounted for 127 (64%), 39 (19.9%), and 26 (13.3%) cases, respectively. Of the 39 patients with hemoglobinopathies, 26 were confirmed by globin gene analysis, including 20 patients with ��-thalassemia minor, 5 patients with a-thalassemia minor, and 1 patient with unstable hemoglobin disease. Conclusion: The number of patients diagnosed with hemoglobinopathies and RBC enzymopathies has increased considerably since the previous survey on HHA in Korea, dated from 1997 to 2006. This is likely the result of improved diagnostic techniques. Nevertheless, there is still a need for more sensitive diagnostic tests utilizing flow cytometry and for better standardization of test results to improve the accuracy of diagnosis of RBC membranopathies in Korea. Additionally, more accurate assays for the identification of RBC enzymopathies are warranted. ? 2013 Korean Society of Hematology.
URI
http://hdl.handle.net/YU.REPOSITORY/28950http://dx.doi.org/10.5045/br.2013.48.3.211
ISSN
2287-979X
Appears in Collections:
의과대학 > 소아청소년과학교실 > Articles
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