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与体重不足原发性免疫缺陷患者相关的并发症:在美国免疫缺陷网注册研究中的流行率及关联。

Complications Associated with Underweight Primary Immunodeficiency Patients: Prevalence and Associations Within the USIDNET Registry.

机构信息

Division of Allergy and Immunology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Department of Pediatrics, Perelman School of Medicine at University of Pennsylvania, Philadelphia, PA, USA.

出版信息

J Clin Immunol. 2018 Apr;38(3):283-293. doi: 10.1007/s10875-018-0492-0. Epub 2018 Apr 4.

Abstract

PURPOSE

The point prevalence of underweight status and obesity in primary immunodeficiency disease (PID) is unknown, despite the described associations between PID and weight loss and failure to thrive. The goal of this study is to estimate the prevalence of underweight status and obesity in PID patients and to investigate the associations between abnormal body weight and complications of PID.

METHODS

Using the US Immunodeficiency Network (USIDNET), we performed a retrospective analysis of 653 pediatric (age 2 to 20 years) and 514 adult (age > 20) patient records with information on patient body mass index (BMI). Prevalence of underweight and obese status in PID patients was compared to data from the National Health and Nutrition Examination Survey (NHANES).

RESULTS

After separating BMI data by year of entry to the database, we demonstrated that both adult and pediatric patients with PID had significantly higher prevalence of underweight patients in multiple years of analysis. Further examination of underweight patients by PID diagnosis revealed that underweight status in adults with CVID was associated with granulomatous disease as well as earlier age of CVID diagnosis. In the pediatric CVID cohort, underweight status was significantly associated with lymphopenia. Examination of obesity in pediatric and adult PID patients compared to NHANES database revealed only a single year when obesity in PID patients was significantly less prevalent. In other 2-year time intervals from 2005 to 2014, the prevalence of obesity was unchanged in children and adults.

CONCLUSIONS

These results quantify the prevalence of underweight status in PID in a North American population and demonstrate that whether as a result of weight loss or poor weight gain, underweight status is more prevalent in the PID population than in the general US population. The prevalence of obesity in PID patients was similar to that seen in the general population. This highlights the need for continued education on the association of low weight and PID.

CLINICAL TRIAL REGISTRATION

NCT01953016.

摘要

目的

尽管已知原发性免疫缺陷病 (PID) 与体重减轻和生长不良有关,但 PID 患者体重不足和肥胖的现患率仍不清楚。本研究的目的是估计 PID 患者体重不足和肥胖的现患率,并探讨异常体重与 PID 并发症之间的关系。

方法

利用美国免疫缺陷网络 (USIDNET),我们对 653 名儿科 (2 至 20 岁) 和 514 名成年 (年龄>20 岁) 患者的病历进行了回顾性分析,这些病历中包含患者的体重指数 (BMI) 信息。将 PID 患者的体重不足和肥胖现患率与国家健康和营养检查调查 (NHANES) 的数据进行比较。

结果

按入数据库的年份对 BMI 数据进行分离后,我们发现 PID 患者在多年的分析中,体重不足患者的比例明显更高。通过 PID 诊断进一步检查体重不足患者,发现成人 CVID 患者的体重不足状态与肉芽肿病以及 CVID 更早的诊断年龄有关。在儿科 CVID 队列中,体重不足状态与淋巴细胞减少显著相关。与 NHANES 数据库相比,对儿科和成年 PID 患者的肥胖进行检查显示,只有一年 PID 患者的肥胖患病率明显较低。在 2005 年至 2014 年的另外两个 2 年时间间隔内,儿童和成人 PID 患者的肥胖患病率没有变化。

结论

这些结果量化了北美人群中 PID 体重不足的现患率,并表明无论体重减轻还是增重不良,PID 人群中的体重不足状态都比一般美国人群更为普遍。PID 患者的肥胖患病率与一般人群相似。这突出了需要继续教育 PID 与低体重之间的关联。

临床试验注册

NCT01953016。

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