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影响李-弗劳梅尼综合征患者对高危监测方案依从性的因素。

Factors affecting adherence to a high-risk surveillance protocol among patients with Li-Fraumeni syndrome.

作者信息

Underkofler Kaylee A, Thomas Martha H, Taylor Christina J, Mazur Christa L, Erickson Sarah H, Ring Kari L

机构信息

Emily Couric Clinical Cancer Center, University of Virginia, 1240 Lee St, 22903, Charlottesville, VA, USA.

Department of Radiology and Medical Imaging, University of Virginia, Charlottesville, VA, USA.

出版信息

Hered Cancer Clin Pract. 2023 Aug 11;21(1):15. doi: 10.1186/s13053-023-00259-z.

DOI:10.1186/s13053-023-00259-z
PMID:37568169
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10422839/
Abstract

BACKGROUND

High-risk surveillance for patients with Li-Fraumeni syndrome (LFS) has shown a stage shift and improved overall survival, but is demanding. Our objective was to evaluate surveillance adherence in a population of patients with LFS presenting for high-risk care.

METHODS

A retrospective analysis of surveillance adherence of adult patients with LFS at a single institution was performed. Adherence was defined by the duration from initial University of Virginia (UVA) LFS clinic visit to the time of first missed surveillance test. Two-sample t-tests and ANOVA tests were used to identify factors associated with duration of adherence.

RESULTS

A total of 42 patients were evaluated in the UVA LFS clinic between 2017 and 2021. Of these, 21 patients met inclusion criteria. At the time of review, 6 patients (29%) were up to date with high-risk surveillance recommendations. The mean duration of adherence was 17 months. Female sex was found to be associated with longer duration of adherence (mean 21 mo vs. 3.5 mo for males, p = 0.02). A personal history or active diagnosis of cancer was also associated with increased adherence (p = 0.02). However, neither age (p = 0.89), geography (p = 0.84), or known family history of LFS (p = 0.08) were associated with duration of adherence.

CONCLUSION

Female sex as well as a personal history of cancer were associated with longer duration of adherence to recommended high-risk surveillance among patients with LFS. Identification of barriers to surveillance will be essential moving forward to increase adherence and promote early detection of cancer, thereby reducing the morbidity and mortality of LFS.

摘要

背景

对李-弗劳梅尼综合征(LFS)患者进行的高风险监测已显示出分期转变并改善了总生存期,但要求较高。我们的目标是评估前来接受高风险护理的LFS患者群体的监测依从性。

方法

对一家机构的成年LFS患者的监测依从性进行回顾性分析。依从性定义为从弗吉尼亚大学(UVA)LFS诊所首次就诊到首次错过监测检查的时间间隔。使用双样本t检验和方差分析来确定与依从性持续时间相关的因素。

结果

2017年至2021年间,UVA LFS诊所共评估了42例患者。其中,21例患者符合纳入标准。在审查时,6例患者(29%)符合高风险监测建议。依从性的平均持续时间为17个月。发现女性的依从性持续时间更长(平均21个月,男性为3.5个月,p = 0.02)。个人癌症病史或现患癌症诊断也与依从性增加相关(p = 0.02)。然而,年龄(p = 0.89)、地理位置(p = 0.84)或已知的LFS家族病史(p = 0.08)均与依从性持续时间无关。

结论

女性以及个人癌症病史与LFS患者对推荐的高风险监测的更长依从性持续时间相关。确定监测障碍对于提高依从性和促进癌症早期检测至关重要,从而降低LFS的发病率和死亡率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/589a/10422839/7462cefb9482/13053_2023_259_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/589a/10422839/7462cefb9482/13053_2023_259_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/589a/10422839/7462cefb9482/13053_2023_259_Fig1_HTML.jpg

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