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국내 유전용혈빈혈 : 1997년 ~ 2006년간의 후향적 조사

Other Titles
 Hereditary Hemolytic Anemia in Korea: a Retrospective Study from 1997 to 2006 
 조희순  ;  하정옥  ;  황평한  ;  현명수  ;  최은진  ;  진종률  ;  조덕연  ;  조군제  ;  정혜림  ;  정주섭  ;  정소영  ;  정대철  ;  임호준  ;  임재영  ;  임영탁  ;  이종석  ;  이정애  ;  이영호  ;  이순용  ;  이기현  ;  이광철  ;  이건수  ;  윤성수  ;  유철주  ;  유은선  ;  오도연  ;  안효섭  ;  신희영  ;  신호진  ;  손상균  ;  서창인  ;  서종진  ;  방수미  ;  박준은  ;  박선양  ;  박상규  ;  김흥식  ;  김태형  ;  김철수  ;  김지윤  ;  김영대  ;  김승택  ;  김순기  ;  김병국  ;  국훈  ;  구홍회  ;  곽재용  ;  강형진  ;  강임주 
 Korean Journal of Hematology, Vol.42(3) : 197-205, 2007 
Journal Title
 Korean Journal of Hematology 
Issue Date
Background The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. Methods By the use of questionnaires, information on the clinical and laboratory findings of HHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. Results The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of β-thalassemia, a case (0.2%) of α-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8±0.7U/1012 RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. Conclusion The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
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1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Lyu, Chuhl Joo(유철주) ORCID logo https://orcid.org/0000-0001-7124-7818
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