Abstract

ABSTRACTObjective:To evaluate medical knowledge of primary immunodeficiency in the city of Sao Paulo (SP).Methods:A 14-item questionnaire about primary immunodeficiency was applied to physicians who worked at general hospitals. One of the questions presented 25 clinical situations that could be associated or not with primary immunodeficiency, and the percentage of appropriate answers generated a knowledge indicator.Results:Seven hundred and forty-six participated in the study, among them 215 pediatricians (28.8%), 244 surgeons (32.7%), and 287 clinicians (38.5%). About 70% of the physicians responded that they had learned about primary immunodeficiency in graduate school or in residency training. Treatment of patients that use antibiotics frequently was reported by 75% dos physicians, but only 34.1% had already investigated a patient and 77.8% said they did not know the ten warning signs for primary immunodeficiency. The knowledge indicator obtained showed a mean of 45.72% (±17.87). Only 26.6% if the pediatricians and 6.6% of clinicians and surgeons showed a knowledge indicator of at least 67% (equivalent to an appropriate answer in two thirds of the clinical situations).Conclusion:There is a deficit in medical knowledge of primary immunodeficiency in the city of Sao Paulo, even among pediatricians, despite having greater contact with the theme over the last few years. The improvement of information on primary immunodeficiency in the medical community is an important step towards the diagnosis and treatment process of these diseases.

Highlights

  • Primary immunodeficiency (PIM) represents more than 200 genetic diseases, in which alterations in the immune system (IS) cause greater predisposition towards infections, autoimmune phenomena, allergies, and neoplasms[1]

  • Seven hundred and sixty-one physicians were approached, of which 746 (98%) responded to the questionnaire distributed among pediatricians, clinicians, and surgeons

  • More than 50% of the participants had some form of link with teaching institutions, had concluded graduate school after the year 2000, and worked both in public and private services (Table 1)

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Summary

Introduction

Primary immunodeficiency (PIM) represents more than 200 genetic diseases, in which alterations in the immune system (IS) cause greater predisposition towards infections, autoimmune phenomena, allergies, and neoplasms[1]. The estimated frequency varies from 1:2,000 to 1:10,000 as a group[2,3]. It is known, that most of these diseases have not yet been described[4,5]. The large diversity of genetic defects and clinical manifestations makes recognition and diagnosis of patients with PIM a challenge[8]. Serious immune defects are more frequently recognized, but there are cases in which PIM is diagnosed only when the patient is submitted to one or more hospitalizations and may already present with permanent sequelae[9,10]

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