描述性分析居住在法国的镰状细胞病患者：PHEDRE 横断面研究。

Descriptive analysis of sickle cell patients living in France: The PHEDRE cross-sectional study.

机构信息

Service de Pharmacologie Clinique, Centre Hospitalier Universitaire de Nantes, Nantes, France.

Service d'Addictologie et de Psychiatrie, Centre Hospitalier Universitaire de Nantes, Nantes, France.

出版信息

PLoS One. 2021 Mar 18;16(3):e0248649. doi: 10.1371/journal.pone.0248649. eCollection 2021.

DOI:10.1371/journal.pone.0248649

PMID:33735176

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7971579/

Abstract

BACKGROUND

Sickle cell disease (SCD) induces chronic haemolytic anaemia and intermittent vaso-occlusion that results in tissue ischaemia causing acute, severe pain episodes that can lead to frequent hospitalizations. These consequences can have repercussions on family, social, school and/or professional life. Here, we present some of the results of the PHEDRE study (Pharmacodépendance Et DREpanocytose-drug dependence and sickle-cell disease), which is the largest study of patients with SCD in France. This paper intends to describe characteristics of the French SCD population. We also aimed to assess the impact of the disease on the lives of patients using objective and subjective variables.

METHODS

The PHEDRE study was a national multicentric observational study. Adults, adolescents and children with a confirmed SCD diagnosis were included in the study by their referring doctor. Then, they were interviewed by phone about their socioeconomic status, about the impact of the disease on their lives and about their analgesic and psychoactive drug use.

RESULTS

The study population consisted of 872 patients (28% were minors). Seventy-two percent of adults were active, and all minors were in school. Many patients presented criteria of severe SCD. Seventy-five percent were homozygous SS, 15% were double heterozygotes SC and 8% were heterozygotes Sβthal, 87% received specific treatment, 58% were hospitalized at least once for vaso-occlusive crisis in the past 12 months, and the number of analgesic drugs taken averaged 3.8. Seventy-five percent of patients reported academic or professional consequences related to their SCD, and 52% reported social consequences.

CONCLUSIONS

The impact of SCD on patients' lives can be significant, nevertheless their social integration seems to be maintained. We highlighted respect of recommendations regarding analgesic treatments and only a few patients used tobacco, alcohol or cannabis.

TRIAL REGISTRATION

Clinical Trials, NCT02580565; https://clinicaltrials.gov/ Registered 16 October 2015.

摘要

背景

镰状细胞病（SCD）可导致慢性溶血性贫血和间歇性血管阻塞，从而导致组织缺血，引发严重的急性疼痛发作，导致频繁住院。这些后果可能对家庭、社会、学校和/或职业生活产生影响。在这里，我们展示了 PHEDRE 研究（Pharmacodépendance Et DREpanocytose-药物依赖和镰状细胞病）的部分结果，这是法国最大的 SCD 患者研究。本文旨在描述法国 SCD 人群的特征。我们还旨在使用客观和主观变量评估该疾病对患者生活的影响。

方法

PHEDRE 研究是一项全国性多中心观察性研究。通过他们的主治医生，确认患有 SCD 的成年人、青少年和儿童被纳入研究。然后，通过电话对他们的社会经济状况、疾病对他们生活的影响以及他们使用的镇痛和精神活性药物进行了访谈。

结果

研究人群包括 872 名患者（28%为未成年人）。72%的成年人活跃，所有未成年人都在上学。许多患者符合严重 SCD 的标准。75%为纯合子 SS，15%为双重杂合子 SC，8%为杂合子 Sβthal，87%接受了特定治疗，58%在过去 12 个月中至少因血管阻塞性危象住院一次，服用的镇痛药物平均为 3.8 种。75%的患者报告称其 SCD 对其学业或职业有影响，52%的患者报告称有社会影响。