Abstract
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.
Highlights
Primary immunodeficiencies (PIDs) are rare diseases considered in isolation; they comprise a set of over 200 different changes already described and have an estimated prevalence of 1:2,000 live births[1]
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
Summary
Primary immunodeficiencies (PIDs) are rare diseases considered in isolation; they comprise a set of over 200 different changes already described and have an estimated prevalence of 1:2,000 live births[1]. Patients with PIDs present, in general, recurrent and/or more severe infections than those usually observed for each age group[3]. In previous surveys conducted by the Primary Immunodeficiency Foundation, the infections most associated to subsequent diagnosis of PIDs in patients in the United States were pneumonia, acute otitis media, sinusitis, tracheobronchitis, and acute diarrhea, which are common in childhood[4]. In this sense, the Jeffrey Modell Foundation, along with other American institutions, formulated warning signs to draw attention for the need to investigate a possible immune deficiency in this group of patients[5]. Among the warning signs (Chart 1), there is an episode of severe systemic infection (meningitis, osteoarthritis, and sepsis)(6,7)
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
