Abstract
Early differentiation of adenosine deaminase deficient severe combined immunodeficiency (ADA-SCID) from other forms of SCID may initiate appropriate treatment interventions with the aim of metabolic detoxification and improved outcome. Our hypothesis was that previously described radiological features (inferior scapular angle squaring and spurring and costochondral cupping) can differentiate ADA-SCID from other forms of SCID. Chest radiographs at clinical presentation between 2000 and 2017 of children with ADA-SCID were retrospectively included, provided that the radiological features were assessable. Random chest radiographs of children with other forms of SCID were included for comparison. Three paediatric radiologists (2 senior, 1 junior) assessed the radiographs for the specific radiological features and stated their diagnosis (ADA-SCID or non-ADA-SCID). An optimal threshold for test performance was defined using a ROC curve. Thirty-six patients with ADA-SCID and twenty-five patients with non-ADA-SCID were included (median age 3.8 months). The optimal threshold for test performance was at approximately < 7 months old: sensitivity 91.7%, specificity 80.7%, interreader agreement was k = 0.709, AUC 0.862. The positive likelihood ratio for scapular squaring, scapular spur, and costochondral cupping was 4.0, 54.6 and 7.8, respectively. The test was valid when performed by both senior and junior paediatric radiologists. Radiological features such as scapular spurring, scapular squaring and costochondral cupping can reliably differentiate between ADA-SCID and other forms of SCID. This is true for children aged approximately < 7 months, and this is reliable when assessed by both senior and junior paediatric radiologists.
Highlights
It is unclear whether high-dose influenza vaccine (HD) is more effective at reducing mortality among seniors
Beginning with the 2010/11 season, the Veterans Health Administration (VHA) began to introduce the HD across its medical centres, largely relying on individual facilities to decide the volume to order and on physicians to decide the recipients of the specific type of vaccine
We included 569,552 person-seasons of observation where 207,574 (36%) were among HD recipients and 361,978 (64%) were among standard-dose influenza vaccines (SD) recipients during the three seasons; 99% were male and 80% were of nonHispanic white origin (Table 1)
Summary
It is unclear whether high-dose influenza vaccine (HD) is more effective at reducing mortality among seniors. Pooling findings from all three seasons, the adjusted rVE estimate of HD vs SD during the high influenza periods was 42% (95% confidence interval (CI): 24–59) against influenza/pneumonia-associated mortality and 27% (95% CI: 23–32) against cardiorespiratory mortality. Excluding individuals with high 1-year predicted mortality at baseline reduced the residual confounding and yielded rVE of 36% (95% CI: 10–62) and 25% (95% CI: 12–38) against influenza/pneumonia-associated and cardiorespiratory mortality, respectively. These were confirmed by results from two-stage residual inclusion estimations. While the majority of VHA senior patients still receive SD, the proportion of HD
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