镰状细胞贫血患者的临床流行病学特征及死亡率：一项1980年至2018年的回顾性队列研究

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018.

作者信息

Pompeo Carolina Mariano, Ferreira Júnior Marcos Antonio, Cardoso Andreia Insabralde de Queiroz, Souza Mercy da Costa, Frota Oleci Pereira, Mota Felipe Machado, Ivo Maria Lúcia

机构信息

Integrated Institute of Health, Federal University of Mato Grosso do Sul, Campo Grande, Mato Grosso do Sul, Brazil.

Graduate Program in Health and Development in the Midwest Region, Federal University of Mato Grosso do Sul, Campo Grande, Mato Grosso do Sul, Brazil.

出版信息

Int J Gen Med. 2022 Feb 2;15:1057-1074. doi: 10.2147/IJGM.S342971. eCollection 2022.

DOI:10.2147/IJGM.S342971

PMID:35140508

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8818769/

Abstract

PURPOSE

To analyze the clinical-epidemiological characteristics and mortality in patients with sickle-cell anemia (SCA).

PATIENTS AND METHODS

A cohort study with retrospective data, conducted in two reference hospitals for SCA treatment from January 1980 to December 2018, recorded in two reference services. With a 5% significance level, the Chi-Square and Student's t-tests were employed in the inferential statistical analysis.

RESULTS

A total of 128 patients with SCA were studied. Diagnosis up to the fifth day of life was made in 10 patients. There were 19 deaths, of which 12 (63.2%) were female, and the average age at death was 27.05 (± 14.78) years. The leading causes of death were septic shock and cardiogenic shock. The use of invasive medical devices was considered a risk factor for death (RR=2.63; 95% CI=1.16-5.96; p=0.018), and monitoring time up to 20 years presented a 31% reduction in the risk of dying (RR=0.31; 95% CI=0.12-0.82; p=0.011) when compared to the monitoring of more than 20 years.

CONCLUSION

These findings are to be considered in the treatment of patients with SCA, mainly regarding early diagnosis and access to the treatment immediately afterward, since they are fundamental in improving survival and reducing severe complications.

摘要

目的

分析镰状细胞贫血（SCA）患者的临床流行病学特征及死亡率。

患者与方法

一项回顾性队列研究，于1980年1月至2018年12月在两家SCA治疗参考医院进行，数据记录于两个参考科室。在推断性统计分析中采用卡方检验和学生t检验，显著性水平为5%。

结果

共研究了128例SCA患者。10例患者在出生后第五天内确诊。有19例死亡，其中12例（63.2%）为女性，平均死亡年龄为27.05（±14.78）岁。主要死亡原因是感染性休克和心源性休克。使用侵入性医疗设备被认为是死亡的危险因素（RR = 2.63；95%CI = 1.16 - 5.96；p = 0.018），与监测时间超过20年相比，监测时间长达20年的死亡风险降低了31%（RR = 0.31；95%CI = 0.12 - 0.82；p = 0.011）。