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普通可变免疫缺陷疾病成年患者的健康相关生活质量及治疗影响

Health-Related Quality of Life in Adult Patients with Common Variable Immunodeficiency Disorders and Impact of Treatment.

作者信息

Rider Nicholas L, Kutac Carleigh, Hajjar Joud, Scalchunes Chris, Seeborg Filiz O, Boyle Marcia, Orange Jordan S

机构信息

Texas Children's Hospital and Baylor College of Medicine, 1102 Bates St, Houston, TX, 77030, USA.

Section of Immunology, Allergy and Rheumatology, Texas Children's Hospital, 1102 Bates St, Suite 330, Houston, TX, 77030, USA.

出版信息

J Clin Immunol. 2017 Jul;37(5):461-475. doi: 10.1007/s10875-017-0404-8. Epub 2017 May 23.

Abstract

PURPOSE

Common variable immunodeficiency disorder (CVID) is a primary immunodeficiency disease (PIDD) often associated with severe and chronic infections. Patients commonly receive immunoglobulin (Ig) treatment to reduce the cycle of recurrent infection and improve physical functioning. However, how Ig treatment in CVID affects quality of life (QOL) has not been thoroughly evaluated. The purpose of a recent Immune Deficiency Foundation (IDF) mail survey was to assess the factors that are associated with QOL in patients with CVID receiving Ig treatment.

METHODS

A 75-question survey developed by the IDF and a 12-item Short Form Health Survey (SF-12) to assess QOL were mailed to adults with CVID. Mean SF-12 scores were compared between patients with CVID and the general US adult population normative sample.

RESULTS

Overall, 945 patients with CVID completed the surveys. More than half of the patients (54.9%) received intravenous Ig and 44.9% received subcutaneous Ig treatment. Patients with CVID had significantly lower SF-12 scores compared with the general US population regardless of sex or age (p < 0.05). Route of IgG replacement did not dramatically improve QOL. SF-12 scores were highest in patients with CVID who have well-controlled PIDD, lacked physical impairments, were not bothered by treatment, and received Ig infusions at home.

CONCLUSION

These data provide insight into what factors are most associated with physical and mental health, which can serve to improve QOL in patients in this population. Improvements in QOL can result from early detection of disease, limiting digestive system disease, attention to fatigue, and implementation of an individual treatment plan for the patient.

摘要

目的

普通变异型免疫缺陷病(CVID)是一种原发性免疫缺陷病(PIDD),常与严重的慢性感染相关。患者通常接受免疫球蛋白(Ig)治疗,以减少反复感染的周期并改善身体机能。然而,CVID患者接受Ig治疗对生活质量(QOL)的影响尚未得到充分评估。免疫缺陷基金会(IDF)最近进行的一项邮件调查旨在评估接受Ig治疗的CVID患者生活质量的相关因素。

方法

IDF编制的一份包含75个问题的调查问卷和一份用于评估生活质量的12项简短健康调查问卷(SF-12)被邮寄给患有CVID的成年人。比较了CVID患者与美国成年人群体标准样本的SF-12平均得分。

结果

总体而言,945名CVID患者完成了调查。超过一半的患者(54.9%)接受静脉注射Ig,44.9%接受皮下注射Ig治疗。无论性别或年龄,CVID患者的SF-12得分均显著低于美国普通人群(p < 0.05)。Ig替代途径并未显著改善生活质量。在PIDD得到良好控制、没有身体损伤、不受治疗困扰且在家接受Ig输注的CVID患者中,SF-12得分最高。

结论

这些数据深入揭示了哪些因素与身心健康最为相关,这有助于改善该人群患者的生活质量。生活质量的提高可源于疾病的早期发现、限制消化系统疾病、关注疲劳以及为患者实施个性化治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d39b/5489588/041eb1130778/10875_2017_404_Fig1_HTML.jpg

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