RESUMO
Background: It is widely accepted that concerns have been recently raised regarding the impact of air pollution on the health of children with cystic fibrosis (CF). Air pollution probably affects the exacerbation of CF and its laboratory findings. On the other hand, the World Health Organization (WHO) has asked all countries to update their data and reports on the distribution and prevalence of CF in different areas. The purpose of the present study was to investigate the distribution and prevalence of CF based on the levels of atmospheric pollutants, such as PM10, PM2.5, SO2, NO2, CO, and O3 in 22 zones of Tehran, and to report the abnormal laboratory findings that might indicate the exacerbation of CF. Materials and Methods: The studied statistical population included children with CF referred to Masih Daneshvari Hospital from 2003 to 2020. Demographic data, location of living area, and laboratory findings were extracted from patient records. The geographic information system (GIS) was applied to indicate the distribution and dispersion of the disease. The information related to air pollutants was collected from all stations in Tehran during the studied period by the Department of Environment of Tehran Province, and the average levels were used for final reporting. Results: The analysis results on 287 CF patients demonstrated that the risk of disease exacerbation significantly increased by the presence of air pollutants. In areas with multiple air pollutants, more laboratory findings were observed to be abnormal, and the lower survival rate for patients with CF was recorded. Investigating the CF distribution pattern based on climatic layers and above mean sea level (AMSL) indicated that distribution of the disease was higher in dry areas with lower AMSL and the higher volume of the atmospheric pollutants, which were primarily centralized in southern and central Tehran. Conclusion: Environmental factors, such as air pollution, can be considered vital parameters, along with high-risk factors, such as pure and integrated race, migration, and mutation, influencing the prevalence and exacerbation of CF symptoms. Considering the higher prevalence of CF in deprived areas of Tehran, households' cultural and economic level appears to be a factor in the lack of diagnostic screening and prevention of CF in these areas. On the other hand, continuous monitoring of the air pollution caused by traffic and giving warnings to CF patients and their parents is particularly important.
RESUMO
Chronic granulomatous disease (CGD) is a rare disorder of phagocytes, predisposes patients to bacterial and fungal infections. The main purpose of this study was to determine the clinical, radiological, pathological features, outcome and response to treatment of children with CGD. Thirteen patients with CGD, who had been referred to National Research Institute of Tuberculosis and Lung Disease (NRITLD), were reviewed during a 6 year period (1999-2005). There were 10 (76%) male and 3(24%) female cases. The median age of the patients was 9 years (1 month-12 years). Family history of CGD was reported by 7 patients. The median diagnostic age was 8 years, with a diagnostic delay of 4.5 years. The most common manifestations of CGD were pulmonary infections and skin involvement, followed by generalized lymphadenopathy. The most common radiological findings were multiple lymphadenopathy in mediastinal region and fibrotic changes in lung fields. Two patients died of pulmonary infections. Based on the results of this research, immunologic evaluations especially evaluation for CGD is highly recommended in children suffering from recurrent pulmonary infections, cutaneous or hepatic abscesses, or infections caused by uncommon pathogens. Early diagnosis and prophylactic treatment both, prevent further development of the lesions, irreversible complications and decreasing mortality and morbidity rates in children suffering from CGD.
