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铁粒幼细胞性贫血:诊断标准的不精确性变化,铁粒幼细胞性贫血扩展分类的建议

Sideroblastic anemias: variations on imprecision in diagnostic criteria, proposal for an extended classification of sideroblastic anemias.

作者信息

Koc S, Harris J W

机构信息

Department of Medicine, Case Western Reserve University, School of Medicine at MetroHealth Medical Center, Cleveland, Ohio 44101, USA.

出版信息

Am J Hematol. 1998 Jan;57(1):1-6. doi: 10.1002/(sici)1096-8652(199801)57:1<1::aid-ajh1>3.0.co;2-0.

Abstract

Sideroblastic anemias are caused by a diversity of hereditary, congenital, or acquired disorders. Criteria used in describing sideroblastic anemias vary widely among standard medical textbooks and even so have been imprecisely applied in the literature. Recent discoveries concerning the basic pathophysiologic mechanisms involving the molecular biology of nuclear and mitochondrial DNA, erythroid ALA synthase (ALAS-2), and iron transport have made the classification of sideroblastic anemias very complex. We recommend a more precise evaluation and documentation of the components that characterize the sideroblastic abnormality and propose an extended classification of the sideroblastic anemias.

摘要

铁粒幼细胞性贫血由多种遗传性、先天性或后天性疾病引起。在标准医学教科书中,用于描述铁粒幼细胞性贫血的标准差异很大,甚至在文献中也应用得不准确。最近有关涉及核DNA和线粒体DNA分子生物学、红系δ-氨基-γ-酮戊酸合成酶(ALAS-2)以及铁转运的基本病理生理机制的发现,使得铁粒幼细胞性贫血的分类变得非常复杂。我们建议对表征铁粒幼细胞异常的成分进行更精确的评估和记录,并提出铁粒幼细胞性贫血的扩展分类。

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