The patients, aged between 5 and 12 years, exhibited the phenotypic variability associated with TMEM173-activating mutations,2-4 with lung disease and systemic inflammation being the major features ...in patient 1 (P1) and patient 3 (P3), while in patient 2 (P2) skin involvement was most prominent (Fig 1; see Supplemental Text and Table E1 in this article's Online Repository at www.jacionline.org). Modest and incomplete downregulation of ISG was recently described in splenic B cells of mice treated with tofacitinib, a JAK1/3 inhibitor, with differential signaling effects suggesting currently poorly understood facets of IFN regulation.9 In this regard, our kinetic ex vivo experiments provide insights into the rapid dynamic changes in IFN signaling secondary to JAK1 blockade.
Objective To investigate the risk factors of empyema after acute viral infection and to clarify the hypothesized association(s) between empyema and some viruses and/or the use of nonsteroidal ...anti-inflammatory drugs (NSAIDs). Study design A case-control study was conducted in 15 centers. Cases and controls were enrolled for a source population of children 3-15 years of age with acute viral infections between 2006 and 2009. Results Among 215 empyemas, 83 cases (children with empyema and acute viral infection within the 15 preceding days) were included, and 83 controls (children with acute viral infection) were matched to cases. Considering the intake of any drug within 72 hours after acute viral infection onset and at least 6 consecutive days of antibiotic use and at least 1 day of NSAIDs exposure, the multivariable analysis retained an increased risk of empyema associated with NSAIDs exposure (aOR 2.79, 95% CI 1.4-5.58, P = .004), and a decreased risk associated with antibiotic use (aOR 0.32, 95% CI 0.11-0.97, P = .04). The risk of empyema associated with NSAIDs exposure was greater for children not prescribed an antibiotic and antibiotic intake diminished that risk for children given NSAIDs. Conclusions NSAIDs use during acute viral infection is associated with an increased risk of empyema in children, and antibiotics are associated with a decreased risk. The presence of antibiotic-NSAIDs interaction with this risk is suggested. These findings suggest that NSAIDs should not be recommended as a first-line antipyretic treatment during acute viral infections in children.
Objective To evaluate respiratory morbidities and lung function tests in the cohort of patients with scimitar syndrome evaluated at our center since 1976. Study design Eighty-one children were ...investigated. Twenty-six patients died, all with the infantile form. The median duration of follow-up of surviving children was 7.2 years. Results A high rate of respiratory morbidities was measured, with 38% and 43% of children reporting pulmonary infections or wheezing episodes during the last 12 months of follow-up, respectively. One-third of children have been rehospitalized for a respiratory cause. Lung function tests were obtained in 20 children. The median value of total lung capacity was 73.0% of the predicted value (IQR, 65.3-86.8), and the median value of the ratio of the forced expiratory volume in one second to the forced vital capacity was −1.26 Z score (−2.25; −0.31). Significantly lower total lung capacity values were obtained in children with the infantile form ( P < .005) or with a history of thoracic surgery ( P = .002). The ratio of the forced expiratory volume in one second to the forced vital capacity Z score values were significantly lower in boys ( P < .05) and in children with a history of wheezing ( P = .01). Wheezing episodes were not associated with significant salbutamol-induced reversibility. Conclusion Respiratory complications frequently are observed in children with scimitar syndrome. Pulmonary hypoplasia appears as an independent marker of long-term severity in these patients.
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