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Attentiveness of pediatricians to primary immunodeficiency disorders

DOI: 10.1186/1756-0500-5-393

Keywords: Survey, Primary immunodeficiency, Knowledge, Diagnosis, Management

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Abstract:

A self-administered questionnaire was used to determine the competency of pediatricians in their knowledge of PID disorders. This study questionnaire included questions on PID signs and symptoms, syndromes associated with immunodeficiency, screening tests, interpreting laboratory tests and case management. The participants were 263 pediatricians of diverse education working in the 27 governmental hospitals in all regions of UAE.The overall performance of the pediatricians did not differ based on their age, gender, origin of certification, rank, or years of experience. Of the 50 questions, 20% of pediatricians answered correctly <60% of the questions, 76% answered correctly 60 to 79% of the questions, and 4% answered correctly ≥80% of the questions. Seventeen of the 19 PID signs and symptoms were identified by 55 to 97%. Four of 5 syndromes associated with immunodeficiency were identified by 50 to 90%. Appropriate screening tests were chosen by 64 to 96%. Attention to the laboratory reference range values as function of patient age was notably limited.There was a noteworthy deficiency in PID work-up. Therefore, implementing effective educational strategies is needed to improve the competency of pediatricians to diagnose and manage PID disorders.Primary immunodeficiency (PID) is a cluster of disorders that share a common theme of excessive susceptibility to infection and other associated clinical problems [1,2]. These serious episodes of infection markedly impair the patients’ ability to lead a normal life [3,4]. While individual diseases are rare, PID as an entity is not uncommon [1]. To date, there are more than 160 genetically distinct PID diseases [5], affecting more than 10 million children and adults worldwide [6]. As appropriately stated, PID is common enough that a primary care physician is likely to encounter several patients in their practice [7]. Wood et al. indicated that diagnostic delays remain common, mostly due to limited awareness of the heterogeneity

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