Inborn errors of immunity (IEIs), formerly primary immune deficiencies (PIDs), are a group of rare yet profoundly impactful conditions that compromise immune function, leading to recurrent infections, autoimmunity, and malignancies. While early diagnosis is critical to improving outcomes, awareness of the Jeffrey Modell Foundation’s “10 Warning Signs of PID” remains insufficient among healthcare professionals. This study explores the knowledge and preparedness of pediatric residents at SUNY Downstate Health Sciences University to identify and manage IEIs.
To evaluate the baseline knowledge of IEIs among pediatric residents, highlight areas of strength and gaps, and assess the impact of training exposure on diagnostic confidence and accuracy.
An anonymous questionnaire assessing recognition of IEI warning signs, diagnostic protocols, and management strategies was distributed among pediatric residents. Out of the total 81 residents in the program, 43 completed the survey. Responses were analyzed by postgraduate year (PGY) and prior rotations in Allergy and Immunology (AI).
Progressive knowledge growth: The overall percentage of correct answers improved with training level: PGY-1 residents answered 67.5% of questions correctly, PGY-2 residents 71%, and PGY-3 residents 79%. Specialized training matters: Residents with prior AI rotations had an accuracy rate of 79.5% compared with 69% among those without, emphasizing the value of targeted exposure. Key strengths: Most participants were able to recognize patterns of severe IEI presentations, such as persistent oral thrush (93%) and recurrent sepsis (97%). Critical gaps: Participants rate of recognizing cases with subtle signs such as frequent ear infections (27%) and recurrent sinusitis (54%) was lower, potentially delaying diagnoses.
Misunderstandings about vaccine safety (70%) and universal immunoglobulin therapy (18%) further highlight the need for comprehensive education.
While residents show proficiency in identifying severe IEI manifestations, gaps remain in recognizing subtle presentations and understanding management protocols. These findings emphasize the need for targeted educational interventions, including case-based learning and diagnostic frameworks, to improve early detection, appropriate management, and patient outcomes.