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病例报告:一名患有X连锁铁粒幼细胞贫血的男婴通过脐带血造血干细胞移植成功治愈。

Case report: An infant boy with X-linked sideroblastic anaemia successfully treated by umbilical cord blood haematopoietic stem cell transplantation.

作者信息

Ma Zhongyang, Li Dongjun, Yang Xue, Liang Juan, Zhu Yiping

机构信息

Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, China.

Key Laboratory of Birth Defects and Related Diseases of Women and Children, Ministry of Education, Sichuan University, Chengdu, China.

出版信息

Front Genet. 2022 Nov 15;13:1009988. doi: 10.3389/fgene.2022.1009988. eCollection 2022.

Abstract

X-linked sideroblastic anaemia (XLSA) is an inherited disorder caused by mutations in genes encoding proteins involved in the biosynthesis of haem. The pathogenic gene, as well as the pathogenesis and diagnosis of XLSA, have been fully elucidated in previous studies. However, only a few new advances have been made in managing XLSA in recent years, and blood transfusion remains the primary treatment. We report a case of umbilical cord blood haematopoietic stem cell transplantation in a male infant diagnosed with XLSA who was born with asphyxia due to severe anaemia. Early hepatic vein occlusion occurred after transplantation. However, this complication was rapidly controlled after active treatment, and the child's quality of life improved significantly. Haematopoietic stem cell transplantation is a promising alternative treatment for XLSA.

摘要

X连锁铁粒幼细胞贫血(XLSA)是一种遗传性疾病,由参与血红素生物合成的蛋白质编码基因突变引起。先前的研究已经充分阐明了致病基因以及XLSA的发病机制和诊断方法。然而,近年来在XLSA的治疗方面仅取得了一些新进展,输血仍然是主要治疗方法。我们报告了一例患有XLSA的男婴,因严重贫血出生时窒息,接受了脐带血造血干细胞移植。移植后早期发生了肝静脉闭塞。然而,经过积极治疗,这种并发症得到了迅速控制,患儿的生活质量显著改善。造血干细胞移植是XLSA一种有前景的替代治疗方法。

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