RESUMEN
BACKGROUND: Primary immunodeficiency disorders (PID) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections and increased susceptibility to malignancies, lymphoproliferative and autoimmune conditions. National registries of PID disorders provide epidemiological data and increase the awareness of medical personnel as well as health care providers. METHODS: This study presents the demographic data and clinical manifestations of Iranian PID patients who were diagnosed from March 2006 till the March of 2013 and were registered in Iranian PID Registry (IPIDR) after its second report of 2006. RESULTS: A total number of 731 new PID patients (455 male and 276 female) from 14 medical centers were enrolled in the current study. Predominantly antibody deficiencies were the most common subcategory of PID (32.3 %) and were followed by combined immunodeficiencies (22.3 %), congenital defects of phagocyte number, function, or both (17.4 %), well-defined syndromes with immunodeficiency (17.2 %), autoinflammatory disorders (5.2 %), diseases of immune dysregulation (2.6 %), defects in innate immunity (1.6 %), and complement deficiencies (1.4 %). Severe combined immunodeficiency was the most common disorder (21.1 %). Other prevalent disorders were common variable immunodeficiency (14.9 %), hyper IgE syndrome (7.7 %), and selective IgA deficiency (7.5 %). CONCLUSIONS: 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.
Asunto(s)
Síndromes de Inmunodeficiencia/epidemiología , Síndromes de Inmunodeficiencia/patología , Sistema de Registros , Adolescente , Adulto , Niño , Preescolar , Consanguinidad , Femenino , Humanos , Síndromes de Inmunodeficiencia/clasificación , Síndromes de Inmunodeficiencia/diagnóstico , Lactante , Recién Nacido , Irán/epidemiología , Masculino , Persona de Mediana Edad , PrevalenciaRESUMEN
INTRODUCTION: The surgical management of chronic frontal sinus disorders remains a challenge for rhinologists. The aim of this study was to evaluate the result of Draf III in a series of patients who underwent this procedure. MATERIALS AND METHODS: Twenty patients were included in this study. Demographic data, history of prior surgery, asthma, aspirin sensitivity and Lund-Mackay score were recorded. A visual analog scale was used for frontal-related symptoms. Patients were followed for a mean duration of 17.5 months and the patency of the frontal sinus ostium was closely monitored. RESULTS: Fifteen patients with chronic frontal sinusitis, two patients with mucoceles, two with malignancy, and one with osteoma underwent Draf III. The mean symptoms score significantly decreased from 5.9 to 3. No ostial closure was seen in the follow-up period. Among 15 patients with chronic frontal sinusitis, 12 had patent ostia of whom three had significant stenosis. All patients with mucocele and osteoma had patent ostia in the follow-up period but patients with sinonasal malignancy showed significant stenosis. CONCLUSION: Draf III frontal sinusotomy is successful in alleviating patient symptoms and the frontal sinus neo-ostium will remain patent in long-term follow-up of most patients. Revision surgery will be required in some cases, which seems to be related to the nature of the underlying chronic sinus diseases.