RESUMO
Primary immunodeficiencies are a diverse group of rare genetic disorders, among which phagocytic dysfunction impairs neutrophil function in a wide range of inherited disorders. Due to the heterogeneity of the disorders a multidisciplinary approach is often required for early diagnosis and initiation of appropriate treatments. The aim of this study was to evaluate the imaging findings in children admitted with phagocytic primary immunodeficiencies. Thirty-five children who fulfilled the inclusion criteria for phagocytic dysfunction were enrolled in this study. The patients were under close observation and monitoring from January 2011 until data locking in December 2017. The diagnosis of phagocytic immunodeficiency was confirmed by the patient's clinical course, presentation features, and laboratory data. Among the 35 patients studied, the most frequent condition was chronic granulomatous disease (CGD) (23 patients), followed by different types of neutropenia (8 patients) and Job's syndrome (4 patients). Mediastinal and hilar lymphadenopathies and consolidation were the most frequent presentations. There was a significant relationship between mediastinal/hilar lymphadenopathies and fungal infections. A meaningful relationship was also found between pulmonary nodules without halo signs in patients with concomitant tuberculosis and fungal infections. A significant correlation was found between CGD, pulmonary fibrotic changes, and mediastinal lymphadenopathies. The most frequent radiological manifestations in children included mediastinal and hilar consolidations. Physicians' awareness of the radiological and clinical manifestations of these inherited diseases may be helpful in the early diagnosis and timely initiation of specific prophylaxis measures to prevent infections and also to initiate hematopoietic stem cell transplantation as the curative management modality.
RESUMO
BACKGROUND: Despite the worldwide spread of Severe Acute Respiratory Syndrome Corona Virus 2 (SARS-CoV-2), information about the epidemiological and clinical patterns of this infection is still largely unknown in children. In addition, the prevalence of this disease is still very high in some parts of the world, including Iran. Thus, this study aims to evaluate the epidemiological features, laboratory and imaging findings, and the type of treatments in children with novel coronavirus 2019 (COVID-19). METHOD: This study is conducted from March 2020-March 2021 by using the medical records of hospitalized confirmed COVID-19 children younger than 18 years in five cities of Iran: Tehran, Ahwaz, Isfahan, Bandar-Abbas, and Khorramabad. In addition to demographic and epidemiological data, we also studied clinical signs and treatments. RESULTS: In total 278 confirmed COVID-19 children, the average age was 5.3 years, and 59.4%were boys. A total of 37.8% had an underlying disease, in which the most common was a malignancy. The most common symptoms were fever and cough. In this group of pediatrics, some abnormal laboratory findings have been seen. GGO (Ground-Glass Opacity) had been diagnosed in 58.6% of children. 3.6% needed oxygen therapy with ventilators, and 83.09% had received antibiotic treatments with the majority of ceftriaxone. Also, 10% had got steroids. In this study, the mortality rate was 4.3%. CONCLUSION: In this study, most of the children who died had an underlying disease, so timely care and action is important in them. Most children admitted to our study received antibiotics and were prescribed antivirals and steroids for a smaller number. Also, a small number of children received oxygen therapy, most of whom were in the age group of 1 to 5 years.