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一名患有I型先天性异常红细胞生成性贫血的儿科患者使用地拉罗司治疗后出现急性肝衰竭

Acute Liver Failure in a Pediatric Patient with Congenital Dysery-Thropoietic Anemia Type I Treated with Deferasirox.

作者信息

Ling Galina, Pinsk Vered, Golan-Tripto Inbal, Ling Eduard

机构信息

Pediatric Gastroenterology and Nutrition Unit.

Pediatrics Department B, Saban Pediatric Medical Center for Israel, Soroka University Medical Center , Beer Sheva, Israel.

出版信息

Hematol Rep. 2015 Sep 23;7(3):5987. doi: 10.4081/hr.2015.5987.

Abstract

Congenital dyserythropoietic anemias (CDA) represent a heterogeneous group of disorders characterized by morphological abnormalities of erythroid precursor cells and various degrees of hemolysis. Iron overload is a result of continuous hemolysis and recurrent transfusions. It is treated with iron chelators, including deferasirox. We present here a case of acute liver failure in a 12 years old girl with CDA type I treated with deferasirox and discuss the approach to treatment.

摘要

先天性红细胞生成异常性贫血(CDA)是一组异质性疾病,其特征为红系前体细胞的形态异常和不同程度的溶血。铁过载是持续溶血和反复输血的结果。使用包括地拉罗司在内的铁螯合剂进行治疗。我们在此报告一例12岁I型CDA女童在接受地拉罗司治疗后发生急性肝衰竭的病例,并讨论其治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9f00/4591501/1c2cd4543b4f/hr-2015-3-5987-g001.jpg

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