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去铁胺间断及调整剂量对重型β地中海贫血伴高丙氨酸转氨酶水平患儿的疗效

Efficacy Of Interrupted And Modified Deferasirox Dose Among Paediatric Patients With Β- Thalassemia Major And High Alanine Aminotransferase Level.

作者信息

Al Hafidh Nashwan M

机构信息

Department of Paediatrics, College of Medicine, Ninevah University, Mosul, Iraq.

出版信息

J Ayub Med Coll Abbottabad. 2019 Apr-Jun;31(2):182-184.

Abstract

BACKGROUND

Abnormal liver function tests lead to interruptions of Deferasirox therapy. The aim of this study is to assess the efficacy of deferasirox dose 30 mg /kg /day in maintaining cardiac protective level of serum ferritin of <2500 ng/ml among patients who received interrupted and modified doses.

METHODS

A retrospective cohort study was conducted in Ibn Al Atheer paediatric hospital in Mosul city, Iraq, utilizing the monthly reading of serum ferritin level during the period started in February 2013 to march 2014 using documented patients' records. Group A, patients included thirty-five patients with β- thalassemia major whose Deferasirox dose of 30 mg/kg/day was interrupted and modified due to ≥ 5-fold raise in alanine aminotransferase during any month of the study period. Compared group B patients included 40 children who received constant median deferasirox dose 30 mg/kg/day throughout one year of study period. Serum ferritin and alanine aminotransferase levels were routinely analysed every month among those patients.

RESULTS

Interrupted and modified Deferasirox dose of 30 mg/kg/day significantly (p=0.000) increase the frequency of having mean serum ferritin >2500 ng/ml, and was associated with 55 times relative risk of having mean serum ferritin >2500 ng/ml compared to group B with steady median deferasirox dose.

CONCLUSIONS

Interrupted and modified deferasirox dose of 30 mg/kg/day has a significant adverse effect on cardiac protective level of serum ferritin.

摘要

背景

肝功能检查异常会导致地拉罗司治疗中断。本研究的目的是评估在接受中断和调整剂量的患者中,每日30mg/kg剂量的地拉罗司在维持血清铁蛋白心脏保护水平<2500ng/ml方面的疗效。

方法

在伊拉克摩苏尔市的伊本·阿西尔儿童医院进行了一项回顾性队列研究,利用2013年2月至2014年3月期间记录的患者病历,每月读取血清铁蛋白水平。A组包括35例重型β地中海贫血患者,在研究期间的任何一个月,由于丙氨酸转氨酶升高≥5倍,其30mg/kg/天的地拉罗司剂量被中断并调整。B组为对照组,包括40名儿童,在整个一年的研究期间接受持续的中位剂量30mg/kg/天的地拉罗司治疗。这些患者每月定期分析血清铁蛋白和丙氨酸转氨酶水平。

结果

与B组稳定的中位剂量地拉罗司相比,中断和调整后的30mg/kg/天的地拉罗司剂量显著(p=0.000)增加了平均血清铁蛋白>2500ng/ml的频率,且平均血清铁蛋白>2500ng/ml的相对风险是B组的55倍。

结论

中断和调整后的30mg/kg/天的地拉罗司剂量对血清铁蛋白的心脏保护水平有显著不良影响。

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