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Impact of Aspiration Pneumonia on the Clinical Course of Progressive Supranuclear Palsy: A Retrospective Cohort Study

<div><p>Introduction</p><p>Although aspiration pneumonia is the most common complication of progressive supranuclear palsy (PSP), the clinical impact of aspiration pneumonia on disease course and survival has not been fully estimated. Thus, we retrospectively analyzed the prognostic factors and clinical consequences of pneumonia in PSP.</p><p>Methods</p><p>The clinical course of patients with aspiration pneumonia was surveyed. The association between baseline clinical features (2 years from disease onset) and latency to the initial development of pneumonia was investigated using survival time and Cox regression analyses.</p><p>Results</p><p>Ninety patients with a clinical diagnosis of PSP were observed for 5.1±3.8 years (mean±SD), and 22 had aspiration pneumonia. Subsequently, 20 patients (91%) had to discontinue oral feeding entirely and 13 (59%) died, whereas, of 68 patients without pneumonia, only three patients (4%) died. Time to initial development of pneumonia was strongly correlated with survival time (Spearman R = 0.92, <i>P</i><0.001), with a mean latency of 2.3 years to death. Among baseline clinical features, early fall episodes and cognitive decline were significant predictors of pneumonia (<i>P</i> = 0.001 and <i>P</i><0.001, respectively, log rank test). Cox regression analysis demonstrated that early fall episodes (adjusted hazard ratio: 3.9, 95% confidence interval: 1.2–12.5, <i>P</i> = 0.03) and cognitive decline (adjusted hazard ratio: 5.2, 95% confidence interval: 1.4–19.3, <i>P</i> = 0.02) independently predicted pneumonia. By contrast, dysphagia was not associated with pneumonia (<i>P</i> = 0.2, log rank test).</p><p>Conclusion</p><p>Initial development of pneumonia indicates an unfavorable clinical course and predicts survival time (mean survival time 2.3 years). Patients with early falls and cognitive decline were at high risk of early development of pneumonia.</p></div

Comparison of patients with and without the experience of aspiration pneumonia.

<p>RS/PSP-P, Richardson syndrome / PSP-Parkinsonism</p><p>^aMann–Whitney test</p><p>^bFisher’s exact test</p><p>^cLatency from disease onset</p><p>Comparison of patients with and without the experience of aspiration pneumonia.</p

Cox proportional hazards regression models for the predictive factors of early development of aspiration pneumonia.

<p>Model 1, HR adjusted by age and sex; Model 2, HR adjusted by age, sex, and dysphagia (yes/no) during the first 2 years of the disease.</p><p>Cox proportional hazards regression models for the predictive factors of early development of aspiration pneumonia.</p

Survival analyses stratified by patients’ clinical features and phenotypes during initial 2 years of disease.

<p>Latency from the start of the study to the initial development of pneumonia, stratified by with or without (A) fall episodes (log rank <i>P</i> = 0.001), (B) cognitive decline (log rank <i>P</i><0.001), (C) dysphagia (log rank <i>P</i> = 0.08), and (D) clinical phenotypes (RS and PSP-P; log rank <i>P</i> = 0.05).</p

Clinical courses of 22 patients with experience of pneumonia.

<p>Pneumonia developed in 22 patients and 13 patients died in the observation period. ^aPathologically confirmed cases of progressive supranuclear palsy.</p

Additional file 1 of Simultaneous measurement of the size and methylation of chromosome 4qA-D4Z4 repeats in facioscapulohumeral muscular dystrophy by long-read sequencing

Additional file 1: Fig. S1. Characteristic sequences detected by nCATS. Sequences of representative (A) 4qA- and (B) 10q-derived reads obtained from the indicated samples. The XapI/non-XapI and BlnI/non-BlnI sites in the most distal D4Z4 RU are shown. In Samples 8, 14, and 15, the XapI, XapI and non-BlnI, and non-XapI sites, respectively, in the second most distal D4Z4 RU are shown, due to the difficulty in identifying restriction sites. Table S1. Lengths of reads derived from the 4qA locus in each patient. Table S2. Lengths of reads derived from the 10q locus in each patient. Table S3. Methylation rates across all D4Z4 RUs at the 4qA and 10q loci