ABSTRACT
Primary immunodeficiency diseases (PIDs) consist of a group of genetic disorders that predispose the patients to immune-mediated complications. The aim of this study was to assess the knowledge of Iranian general practitioners and pediatricians about PIDs.A questionnaire consisting 52 closed questions on clinical symptoms, laboratory data, associated syndromes and management of PIDs patients was made valid and reliable by a pair pilot study. Then the questionnaire was filled by pediatricians, general practitioners and pediatric residents from different regions of Iran. Totally, 333 physicians (50 general practitioners, 52 pediatric residents, 182 pediatric specialists, and 49 pediatric sub specialists) participated in this study. The mean total score was 55.9±14.3 (i.e. about 29 correct answers out of 52 questions). One hundred and five participants (31.9%) answered correctly more than two third of all questions. In order to qualitatively compare the groups a ranking system was used. Total scores was significantly different between physicians groups (p<0.01). Pediatric subspecialties gained the highest rank, which was significantly over the other participants (p<0.05). This study showed that there is a considerable lack of awareness on PIDs in physicians. This may be one of the major reasons in late diagnosis and the delay in adequate treatment deteriorating patients' morbidity and mortality. Retraining classes and reconsidered educating schedules are needed as an efficient strategies and improving physicians' knowledge about PIDs.
Subject(s)
Attitude of Health Personnel , Awareness , Health Knowledge, Attitudes, Practice , Immunologic Deficiency Syndromes/diagnosis , Physicians/psychology , Adult , Aged , Aged, 80 and over , Clinical Competence , Delayed Diagnosis , Female , General Practice , General Practitioners/psychology , Humans , Immunologic Deficiency Syndromes/epidemiology , Iran/epidemiology , Male , Middle Aged , Pediatrics , Predictive Value of Tests , Surveys and QuestionnairesABSTRACT
OBJECTIVE: Treatment with intravenous immunoglobulin (IVIG) is considered a safe therapy for patients with primary antibody deficiencies (PADs), whilst adverse effects have been frequently reported. Meantime behavioral disorders reactions have not been reported yet. In this study, we describe for the first time a group of patients with PADs, who were under IVIG therapy and experienced some behavioral disorders. METHODS: Five patients, including two hyper IgM syndromes, one X-linked agammaglobulinemia, one common variable immunodeficiency, and one hypo IgM disease, were surveyed. Analysis of Conner's Parents Rating Scales-Revised Short (CPRS-R:S) and child behavior checklist (CBCL) was performed for the patients, suspected to hyperactivity. RESULTS: Analysis of CPRS-R:S showed an evidence of mild hyperactivity before IVIG administration in four patients, whereas another patient had evidence of severe hyperactivity. After IVIG administration, hyperactivity scores of three patients were changed from mild hyperactive behavior to markedly hyperactive behavior or attention deficit hyperactivity disorder range of hyperactivity. In the CBCL scores, there were abnormal externalization scores for three patients; while two remaining patients had abnormal internalization scores. CONCLUSIONS: Although predisposition to behavioral disorders can be due to a genetic background, further investigations are necessary to test the hypotheses about responsibility of either IVIG or underling disease in progression of behavioral abnormalities.
