The use of erythropoietin-stimulating agents versus supportive care in newborns with hereditary spherocytosis: a single centre's experience.
The use of erythropoietin-stimulating agents versus supportive care in newborns with hereditary spherocytosis: a single centre's experience.
复制标题
在患有遗传性球形红细胞增多症的新生儿中使用促红细胞生成素药物与支持治疗：单一中心的经验。
DOI：
10.1111/ejh.12321
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发表时间：
2014
影响因子：
3.1
通讯作者：
Grace,RachaelF
中科院分区：
文献类型：
--
作者：
Morrison,JacquelineF;Neufeld,EllisJ;Grace,RachaelF
遗传性球形红细胞增多症（HS）是一种常见的溶血性贫血，其中75%的病例为常染色体显性。由于大多数患有HS的新生儿都有家族史，血液科医生经常在他们的生生性血红蛋白最低点之前看到这些婴儿，这与健康婴儿相比被夸大了。本研究的目的是评估在单一儿科项目中对HS患儿使用促红细胞生成素（EPO）与输血的频率和成本。在过去十年中，我们中心只有15%的HS患儿接受了EPO治疗，其费用是单次输血的两倍，而且EPO治疗的患儿并不总是避免输血。在我们的中心，很少处方促生成素治疗婴儿HS可能与不完整的数据支持其使用有关。
Hereditary Spherocytosis (HS) is a common haemolytic anaemia in which 75% of cases are autosomal dominant. As most newborns with HS have a family history of disease, haematologists often see these infants before their physiologic haemoglobin nadir, which is exaggerated in comparison with healthy infants. The objective of this study was to evaluate the frequency of implementation and cost of erythropoietin‐stimulating agents (EPO) versus transfusion in infants with HS at a single paediatric programme. In the last decade, only 15% of infants with HS at our centre have been treated with EPO, which costs twice that of a single transfusion and EPO treated infants did not always avoid transfusion. Infrequent prescription of EPO therapy to infants with HS at our centre may be related to the incomplete data supporting its use.