Clinical and hematologic manifestations in patients with diamond blackfan anemia in korea
DC Field | Value | Language |
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dc.contributor.author | Kim, S.-K. | - |
dc.contributor.author | Ahn, H.-S. | - |
dc.contributor.author | Kang, H.-J. | - |
dc.contributor.author | Kim, H. | - |
dc.contributor.author | Park, K.-D. | - |
dc.contributor.author | Shin, H.-Y. | - |
dc.contributor.author | Back, H.-J. | - |
dc.contributor.author | Kook, H. | - |
dc.contributor.author | Cho, B. | - |
dc.contributor.author | Chung, N.-G. | - |
dc.contributor.author | Jeoung, D.-C. | - |
dc.contributor.author | Choi, E.-J. | - |
dc.contributor.author | Hwang, P.-H. | - |
dc.contributor.author | Ko, K.-N. | - |
dc.contributor.author | Lim, H.-J. | - |
dc.contributor.author | Park, S.-K. | - |
dc.contributor.author | Seo, J.-J. | - |
dc.contributor.author | Koo, H.-H. | - |
dc.contributor.author | Sung, K.-W. | - |
dc.contributor.author | Lee, K.-C. | - |
dc.contributor.author | Lim, Y.-T. | - |
dc.contributor.author | Lyu, C.-J. | - |
dc.contributor.author | Park, J.-E. | - |
dc.contributor.author | Ryu, K.-H. | - |
dc.contributor.author | Yoo, E.S.. | - |
dc.date.accessioned | 2021-09-07T04:14:19Z | - |
dc.date.available | 2021-09-07T04:14:19Z | - |
dc.date.created | 2021-06-17 | - |
dc.date.issued | 2012 | - |
dc.identifier.issn | 1738-7949 | - |
dc.identifier.uri | https://scholar.korea.ac.kr/handle/2021.sw.korea/110673 | - |
dc.description.abstract | Background Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rarecondition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years. Methodsm The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study. Results The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptosis, coarctation of aorta, ventricular septal defect, strabismus, etc. The mean hemoglobin concentration was 5.1±1.9 g/dL, mean corpuscular volume was 93.4±11.6 fL, and mean number of reticulocytes was 19,700/mm 3. The mean cellularity of bone marrow was 75%, with myeloid:erythroid ratio of 20.4:1. After remission, 48.9% of patients did not need further steroids. Five patients with DBA who received hematopoietic transplantation have survived. Cancer developed in 2 cases (3.6%). Conclusion The incidence of DBA is similar to data already published, but our study had a male predilection. Although all patients responded to initial treatment with steroids, about half needed further steroids after remission. It is necessary to collect further data, including information regarding management pathways, from nationwide DBA registries, along with data on molecular analyses. © 2012 Korean Society of Hematology. | - |
dc.language | English | - |
dc.language.iso | en | - |
dc.subject | deferasirox | - |
dc.subject | deferoxamine | - |
dc.subject | methotrexate | - |
dc.subject | steroid | - |
dc.subject | thymocyte antibody | - |
dc.subject | aorta coarctation | - |
dc.subject | article | - |
dc.subject | Blackfan Diamond anemia | - |
dc.subject | bone marrow | - |
dc.subject | child | - |
dc.subject | clinical article | - |
dc.subject | diabetes mellitus | - |
dc.subject | female | - |
dc.subject | graft versus host reaction | - |
dc.subject | growth retardation | - |
dc.subject | heart ventricle septum defect | - |
dc.subject | hematologic disease | - |
dc.subject | hematopoietic stem cell transplantation | - |
dc.subject | hemoglobin determination | - |
dc.subject | hirsutism | - |
dc.subject | human | - |
dc.subject | impaired glucose tolerance | - |
dc.subject | incidence | - |
dc.subject | infant | - |
dc.subject | iron chelation | - |
dc.subject | Korea | - |
dc.subject | large cell lymphoma | - |
dc.subject | lung metastasis | - |
dc.subject | male | - |
dc.subject | mean corpuscular volume | - |
dc.subject | medical record | - |
dc.subject | moon face | - |
dc.subject | osteosarcoma | - |
dc.subject | preschool child | - |
dc.subject | ptosis | - |
dc.subject | pulmonary hypertension | - |
dc.subject | reticulocyte | - |
dc.subject | retrospective study | - |
dc.subject | strabismus | - |
dc.subject | thumb malformation | - |
dc.subject | university hospital | - |
dc.title | Clinical and hematologic manifestations in patients with diamond blackfan anemia in korea | - |
dc.type | Article | - |
dc.contributor.affiliatedAuthor | Lee, K.-C. | - |
dc.identifier.doi | 10.5045/kjh.2012.47.2.131 | - |
dc.identifier.scopusid | 2-s2.0-84866922773 | - |
dc.identifier.bibliographicCitation | Korean Journal of Hematology, v.47, no.2, pp.131 - 135 | - |
dc.relation.isPartOf | Korean Journal of Hematology | - |
dc.citation.title | Korean Journal of Hematology | - |
dc.citation.volume | 47 | - |
dc.citation.number | 2 | - |
dc.citation.startPage | 131 | - |
dc.citation.endPage | 135 | - |
dc.type.rims | ART | - |
dc.type.docType | Article | - |
dc.identifier.kciid | ART001672005 | - |
dc.description.journalClass | 1 | - |
dc.description.journalRegisteredClass | scopus | - |
dc.description.journalRegisteredClass | kci | - |
dc.subject.keywordPlus | deferasirox | - |
dc.subject.keywordPlus | deferoxamine | - |
dc.subject.keywordPlus | methotrexate | - |
dc.subject.keywordPlus | steroid | - |
dc.subject.keywordPlus | thymocyte antibody | - |
dc.subject.keywordPlus | aorta coarctation | - |
dc.subject.keywordPlus | article | - |
dc.subject.keywordPlus | Blackfan Diamond anemia | - |
dc.subject.keywordPlus | bone marrow | - |
dc.subject.keywordPlus | child | - |
dc.subject.keywordPlus | clinical article | - |
dc.subject.keywordPlus | diabetes mellitus | - |
dc.subject.keywordPlus | female | - |
dc.subject.keywordPlus | graft versus host reaction | - |
dc.subject.keywordPlus | growth retardation | - |
dc.subject.keywordPlus | heart ventricle septum defect | - |
dc.subject.keywordPlus | hematologic disease | - |
dc.subject.keywordPlus | hematopoietic stem cell transplantation | - |
dc.subject.keywordPlus | hemoglobin determination | - |
dc.subject.keywordPlus | hirsutism | - |
dc.subject.keywordPlus | human | - |
dc.subject.keywordPlus | impaired glucose tolerance | - |
dc.subject.keywordPlus | incidence | - |
dc.subject.keywordPlus | infant | - |
dc.subject.keywordPlus | iron chelation | - |
dc.subject.keywordPlus | Korea | - |
dc.subject.keywordPlus | large cell lymphoma | - |
dc.subject.keywordPlus | lung metastasis | - |
dc.subject.keywordPlus | male | - |
dc.subject.keywordPlus | mean corpuscular volume | - |
dc.subject.keywordPlus | medical record | - |
dc.subject.keywordPlus | moon face | - |
dc.subject.keywordPlus | osteosarcoma | - |
dc.subject.keywordPlus | preschool child | - |
dc.subject.keywordPlus | ptosis | - |
dc.subject.keywordPlus | pulmonary hypertension | - |
dc.subject.keywordPlus | reticulocyte | - |
dc.subject.keywordPlus | retrospective study | - |
dc.subject.keywordPlus | strabismus | - |
dc.subject.keywordPlus | thumb malformation | - |
dc.subject.keywordPlus | university hospital | - |
dc.subject.keywordAuthor | Anemia | - |
dc.subject.keywordAuthor | Congenital defects | - |
dc.subject.keywordAuthor | Diamond blackfan anemia | - |
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