Background The number of inherited diseases and the spectrum of clinical manifestations of primary immunodeficiency disorders (PIDs) are ever-expanding. Molecular diagnosis using genomic approaches should be performed for all PID patients since it provides a resource to improve the management and to estimate the prognosis of patients with these rare immune disorders. Method The current update of Iranian PID registry (IPIDR) contains the clinical phenotype of newly registered patients during last 5 years (2013-2018) and the result of molecular diagnosis in patients enrolled for targeted and nextgeneration sequencing. Results Considering the newly diagnosed patients (n = 1395), the total number of registered PID patients reached 3056 (1852 male and 1204 female) from 31 medical centers. The predominantly antibody deficiency was the most common subcategory of PID (29.5%). The putative causative genetic defect was identified in 1014 patients (33.1%) and an autosomal recessive pattern was found in 79.3% of these patients. Among the genetically different categories of PID patients, the diagnostic rate was highest in defects in immune dysregulation and lowest in predominantly antibody deficiencies and mutations in the MEFV gene were the most frequent genetic disorder in our cohort.

Registration of Iranian PID patients increased the awareness of medical community of Iran and developed diagnostic and therapeutic techniques across more parts of the country. Further efforts must be taken by increasing the coverage of IPIDR via electronically registration and gradual referral system in order to provide better estimation of PID in Iran and reduce the number of undiagnosed cases.

Background:Problem-based learning (PBL) is one of the most commonly used educational methods in medical schools of different countries. By working through this method, students think critically, generate ideas, and acquire the knowledge and skills required to become a doctor.Objectives:This study aimed to compare problem-based learning with lecture-based learning in the education of medical students.Materials and Methods:This crossover interventional study was conducted on 40 medical students in pediatric ward of Zanjan University of Medical Sciences. All of the students were enrolled in the study and divided into two groups by simple randomization. Then two topics in pediatric courses were chosen. One of the topics was presented as LBL for the first group and as PBL for the second group. The other topic was presented as PBL for the first group and as LBL for the second group.Results:The median score of the exam was higher in the intervention group compare to the control group for both topics. However, the difference was not statistically significant. Students preferred problem-based learning over lecture-based learning because of motivation boost, a higher quality of education, knowledge retention, class attractiveness, and practical use.Conclusions:Students’ knowledge was similar in both methods.

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Eosinophils are the principal effector cells involved in the pathogenesis of allergic inflammation. We conducted a study to investigate the validity of the nasal smear examination for detecting eosinophilia in patients with allergic rhinitis. Our study group was made up of 50 patients with allergic rhinitis and 50 age-and sex-matched controls without allergic rhinitis. Smears were obtained from nasal secretions in both groups and then fixed, stained, and studied under light microscopy. Statistical analysis revealed that the odds ratio for eosinophilia positivity in nasal smears in the rhinitis group was 25.61 with a 95% confidence interval of 8 to 78. The sensitivity, specificity, positive predictive value, and negative predictive value of this test were 74, 90, 88, and 77%, respectively. We conclude that the nasal eosinophilia test is highly specific and moderately sensitive in diagnosing allergic rhinitis, and that it therefore can be used as an easy, noninvasive, and inexpensive procedure for screening patients and for conducting epidemiologic studies of this disorder.