Primary immunodeficiency investigation in patients during and after hospitalization in a pediatric Intensive Care Unit

Objective: To analyze whether the patients with severe infections, admitted in the Pediatric Intensive Care Unit of the Hospital de Clínicas of the Universidade Federal de Uberlândia, underwent the active screening for primary immunodeficiencies (PID).Methods: Retrospective study that assessed the data records of patients with any severe infections admitted in the Pediatric Intensive Care Unit, covering a period from January 2011 to January 2012, in order to confirm if they performed an initial investigation for PID with blood count and immunoglobulin dosage.Results: In the studied period, 53 children were hospitalized with severe infections in the Pediatric Intensive Care Unit, and only in seven (13.2%) the initial investigation of PID was performed. Among these patients, 3/7 (42.8%) showed quantitative alterations in immunoglobulin G (IgG) levels, 1/7 (14.3%) had the diagnosis of cyclic neutropenia, and 1/7 (14.3%) presented thrombocytopenia and a final diagnosis of Wiskott-Aldrich syndrome. Therefore, the PID diagnosis was confirmed in 5/7 (71.4%) of the patients.Conclusions: The investigation of PID in patients with severe infections has not been routinely performed in the Pediatric Intensive Care Unit. Our findings suggest the necessity of performing PID investigation in this group of patients.

研究目的：分析乌贝兰迪亚联邦大学临床医院儿科重症监护病房（Pediatric Intensive Care Unit）收治的重症感染患者是否接受了原发性免疫缺陷病（primary immunodeficiencies, PID）的主动筛查。
研究方法：本研究为回顾性研究，纳入2011年1月至2012年1月期间于该医院儿科重症监护病房收治的所有重症感染患者的病历数据，以确认其是否通过血常规与免疫球蛋白定量检测完成原发性免疫缺陷病的初始排查。
研究结果：研究周期内，共有53名因重症感染入住儿科重症监护病房的儿童，其中仅7名（13.2%）接受了原发性免疫缺陷病的初始排查。在上述7名患者中，3名（42.8%）存在免疫球蛋白G（immunoglobulin G, IgG）水平定量异常，1名（14.3%）被诊断为周期性中性粒细胞减少症，另有1名（14.3%）出现血小板减少症，最终确诊为维斯科特-奥尔德里奇综合征（Wiskott-Aldrich syndrome）。综上，7名患者中有5名（71.4%）最终确诊原发性免疫缺陷病。
研究结论：儿科重症监护病房并未将原发性免疫缺陷病排查作为重症感染患者的常规诊疗操作。本研究结果提示，该类患者群体有必要开展原发性免疫缺陷病的相关排查。