普通变异型免疫缺陷病住院患者的营养状况及支持现状调查

An investigation on the nutritional status and support of in-patients with common variable immunodeficiency

探讨普通变异型免疫缺陷病(CVID)住院患者的临床特点、营养代谢状况及支持现状,为进一步改善其长期营养管理提供研究依据和参考。本研究为回顾性横断面研究,查询北京协和医院电子病历系统,连续筛选2016年1月至2021年6月符合CVID诊断标准的住院患者共33例,男、女分别为16例、17例,年龄范围2~52岁。回顾性分析其临床资料及营养状况、营养干预情况。连续性资料符合正态分布的均值比较采用两组独立样本的t检验,非正态分布的计量资料采用非参数检验进行比较。结果显示,纳入患者中位起病年龄为22(10.0,36.5)岁,中位病程9.0(2.0,16.0)年。病例均有累及不同系统的反复感染(33/33),部分合并自身免疫性疾病(8/33)、淋巴增殖性疾病与恶性肿瘤(9/33)。85.19%(23/27)的成人患者营养风险筛查(nutritional risk screening 2002,NRS 2002)评分≥3分,33.33%(2/6)的青少年儿童患者年龄别体重指数Z值<-2。66.67%(22/33)的患者在病程中有体重下降,87.88%(29/33)、69.70%(23/33)与81.82%(27/33)的患者分别存在贫血、低白蛋白血症与前白蛋白降低。22例进行了微量营养素评价,提示77.27%(17/22)、22.73%(5/22)与31.82%(7/22)的患者分别存在血清铁降低、叶酸缺乏与维生素B12不足。另有6例检测25-OH-VD3,均提示存在维生素D缺乏。存在营养风险的患者中,56.00%(14/25)在住院期间接受了营养支持:口服营养补充(14例),管饲肠内营养(4例),肠外营养支持(5例)。综上,CVID患者存在营养不良的状况突出,目前合理营养支持的管理仍存在不足。

The study aimed to reveal for the first time the clinical characteristics,nutritional and metabolic status and support of hospitalized patients with common variant immunodeficiency disease(CVID),and provide reference to improve the long-term nutritional management for such patients.This is a retrospective cross-sectional study.Through searching the electronic medical record system of Peking Union Medical College Hospital,the study included 33 consecutive in-patients with CVID diagnosed in Jan 2016 to Jun 2021,with the male to female ratio of 16∶17.All their medical data,nutritional assessment and intervention retrospectively summarized and analyzed.Data with normal distribution were described using(x¯±s),and analyzed with independent sample t-test.Data with non-normal distribution were compared with non-parametric test.The results showed that the median onset-age of the included patients was 22(10.0,36.5)years old,and the median duration was 9.0(2.0,16.0)years.All patients had recurrent infections involving various systems(33/33),with development of autoimmune diseases(8/33)and lymphoproliferative disease or malignancy(9/33)in some cases among them.The nutritional risk screening 2002(NRS 2002)scores revealed that 85.19%of adults had an NRS 2002≥3 points,and 33.33%of children had a BMI-for-age z score<-2.Weight loss occurred in 66.67%of patients(22/33),while 87.88%(29/33),69.70%(23/33)and 81.82%(27/33)of patients respectively had anemia,hypoalbuminemia and decreased prealbumin.Among 22 patients with micronutrients status evaluated,77.27%(17/22),22.73%(5/22)and 31.82%(7/22)of patients respectively had lowered serum iron,folate deficiency and vitamin B12 insufficiency.Six patients underwent 25-OH-VD3 measurement,and were all testified to have vitamin D deficiency.Among all patients with nutritional risk,56.00%of them underwent nutritional support:oral nutritional supplements(14 cases),enteral feeding(4 cases)and parenteral nutrition(5 cases).In conclusion,the condition of malnutrition was prevalent in patients with CVID,but was under-recognized and undertreated to some degree.