RESUMO
In the United Arab Emirates, BCG (Bacillus Calmette-Guérin) is administered to all newborns. We present here a young infant with an inborn error of immunity (IEI) who developed fatal adverse events to this live-attenuated vaccine. This male infant received BCG (Serum Institute of India Pvt., Ltd., India) on Day 11 of life. On Day 25, he developed fever, followed by cervical lymphadenitis and bilateral otitis media with fluid drainage. On Day 118, he was admitted with severe hemophagocytic lymphohistiocytosis (HLH), and passed away on Day 145. The diagnostic exome sequencing test identified a hemizygous nonsense variant, NM_000397.3(CYBB):c.676C>T, p.Arg226* (rs137854592). Pathogenic variants of CYBB [cytochrome b(-245), beta subunit; Mendelian Inheritance in Man [MIM] accession code, 300481] are known to cause "immunodeficiency 34, mycobacteriosis, X-linked" (IMD34, MIM#300645) and "chronic granulomatous disease, X-linked" (CGDX, MIM#306400). The natural history of his illness is consistent with "X-linked recessive Mendelian susceptibility to mycobacterial disease (MSMD)." This entity is responsible for his BCG disease and is a likely trigger of his HLH. This disastrous event underlines the importance of developing worldwide policies that target BCG disease prevention, especially in communities with high prevalence of IEI. Moreover, screening for genetic causes of MSMD in the community could pave the way, at least partially, for scale-up of tuberculosis (TB) prevention.
RESUMO
PURPOSE: First, to investigate the added diagnostic value of chest computed tomography (CT) for evaluating COVID-19 in symptomatic children by comparing chest CT findings with chest radiographic findings, and second, to identify the imaging signs and patterns on CT associated with COVID-19 pneumonia in children. MATERIALS AND METHODS: From March 2020 to December 2020, 56 consecutive children (33 males and 23 girls; mean age ± SD, 14.8 ± 5.0 years; range, 9 months-18 years) with mild to moderate symptom and laboratory confirmed COVID-19 (based on Centers for Disease Control criteria) underwent both chest radiography and chest CT on the same day within the first 2 days of initial presentation to the hospital. Two experienced radiologists independently evaluated chest radiographs and chest CT studies for thoracic abnormalities. The findings from chest radiography and chest CT were compared to evaluate the added diagnostic value of chest CT for affecting patient management. Interobserver agreement was measured with Cohen's κ statistics. RESULTS: Eleven (19.6%) of 56 patients had abnormal chest radiographic findings, including ground-glass opacity (GGO) in 5/11 (45.4%) and combined GGO and consolidation in 6/11 (54.5%). On chest CT, 26 (46.4%) of 56 patients had abnormal CT findings, including combined GGO and consolidation in 19/26 (73.1%), GGO in 6/26 (23.1%), and consolidation in 1/26 (3.8%). Chest CT detected all thoracic abnormalities seen on chest radiography in 11/26 (42.3%) cases. In 15/26 (57.7%), chest CT detected lung abnormalities that were not observed on chest radiography, which included GGO and consolidation in 9/15 (60%), GGO in 5/15 (33.3%), and consolidation in 1/15 (6.6%) cases. These additional CT findings did not affect patient management. In addition, chest CT detected radiological signs and patterns, including the halo sign, reversed halo sign, crazy paving pattern, and tree-in-bud pattern. There was almost perfect interobserver agreement between the two reviewers for detecting findings on both chest radiographs (κ, 0.89, p = .001) and chest CT (κ, 0.96, p = .001) studies. CONCLUSION: Chest CT detected lung abnormalities, including GGO and/or consolidation, that were not observed on chest radiography in more than half of symptomatic pediatric patients with COVID-19 pneumonia. However, these additional CT findings did not affect patient management. Therefore, CT is not clinically indicated for the initial evaluation of mild to moderately symptomatic pediatric patients with COVID-19 pneumonia.