To investigate the characteristics and risk factors of a novel parenchymal lung disease (LD), increasingly detected in systemic juvenile idiopathic arthritis (sJIA).
In a multicentre retrospective ...study, 61 cases were investigated using physician-reported clinical information and centralised analyses of radiological, pathological and genetic data.
LD was associated with distinctive features, including acute erythematous clubbing and a high frequency of anaphylactic reactions to the interleukin (IL)-6 inhibitor, tocilizumab. Serum ferritin elevation and/or significant lymphopaenia preceded LD detection. The most prevalent chest CT pattern was septal thickening, involving the periphery of multiple lobes ± ground-glass opacities. The predominant pathology (23 of 36) was pulmonary alveolar proteinosis and/or endogenous lipoid pneumonia (PAP/ELP), with atypical features including regional involvement and concomitant vascular changes. Apparent severe delayed drug hypersensitivity occurred in some cases. The 5-year survival was 42%. Whole exome sequencing (20 of 61) did not identify a novel monogenic defect or likely causal PAP-related or macrophage activation syndrome (MAS)-related mutations. Trisomy 21 and young sJIA onset increased LD risk. Exposure to IL-1 and IL-6 inhibitors (46 of 61) was associated with multiple LD features. By several indicators, severity of sJIA was comparable in drug-exposed subjects and published sJIA cohorts. MAS at sJIA onset was increased in the drug-exposed, but was not associated with LD features.
A rare, life-threatening lung disease in sJIA is defined by a constellation of unusual clinical characteristics. The pathology, a PAP/ELP variant, suggests macrophage dysfunction. Inhibitor exposure may promote LD, independent of sJIA severity, in a small subset of treated patients. Treatment/prevention strategies are needed.
The purpose of this research project was to investigate the effect of repeated composition opportunities on the tonal structure of fifth-grade subjects' compositions. The songs were analyzed for the ...subjects' use of pitch range, degree of tonality, establishment of tonal patterns, and tonal pattern development. A sample of 37 fifth-grade subjects participated in this study. The subjects were interviewed individually and asked to compose a new song on an electric keyboard once each week, for five consecutive weeks. Subjects were given a maximum of ten minutes to compose and rehearse their song. Subjects played their songs twice, and both performances were tape-recorded by the research. Compositions resulting from the first, third, and fifth interviews were analyzed for components of tonal structure and replicability by the research and three independent judges. Results indicated that there were no significant changes in the tonal structure of fifth-grade children's compositions during repeated opportunities for composition. There were significant differences (p $<$.05) in the subjects' ability to replicate their compositions between the first and the fifth interview. The effect of repeated opportunities for composition was that the subjects were better able to engage in the creation of a composition.
PDF
