THE IMPORTANCE OF EDUCATING PEDIATRICIANS ABOUT PRIMARY IMMUNODEFICIENCY DISORDERS: A TERTIARY HOSPITAL EXPERIENCE.
Academic Article
Overview
abstract
Primary immunodeficiency disorders (PIDs) are several genetic disorders that alter the essential components of the immune system leading to errors in differentiation, function or both of these components.There are more than 200 reported different PID diseases with more than 140 identified gene mutations, affecting almost six million individuals globally, but only 27,000-60,000 have being diagnosed.Early diagnosis of PIDs can markedly reduce morbidity and mortality via proper intervention The aim of the study was to estimate the knowledge and attitude of pediatric residents of PIDs.To the best of our knowledge, this is the first study that targets resident physicians in the field of PIDs. A prospective and cross-sectional study was conducted at Hamad Medical Corporation, the only tertiary care, academic and teaching hospital in the state of Qatar. The study took place between January, 2014 and April 30, 2014. A self-administered questionnaire was distributed to 68 pediatric residents (post-graduate year 1-4). In all, 68 eligible resident physicians were included in the study. Out of the 68 questionnaires distributed, 59 (86.7%) were returned by the end of the study. Among the participants, 18 (30.5%) were post-graduate year-1 (PGY-1), 18 (30.5%) PGY-2, 11 (18.6%) PGY-3, and 12 (20.3%) PGY-4.The mean overall score was 58.5 %. The mean score in the clinical presentation was 67.5%, in associated syndromes and diseases was 59%, in screening laboratory work up 55.3%, and in the section of laboratory investigations that suggest PIDs 52%. There is a significant lack of knowledge of PIDs among pediatric residents. In addition, a large number of pediatric physicians in training do not feel comfortable in diagnosing and managing young children with PIDs. Pediatric residency working hours rule restrict the luxury of having an allergy/immunology rotation during residency. A mutual effort in sharing diagnosis and management of patients with PIDs between pediatric residents and attending immunologists can ameliorate the lack of knowledge and improve the trainee's confidence when facing such cases.
