63 research outputs found
Results of the standard set forpulmonary sarcoidosis: Feasibility and multicentre outcomes
Our study presents findings on a previously developed standard set of clinical outcome data for pulmonary sarcoidosis patients. We aimed to assess whether changes in outcome varied between the different centres and to evaluate the feasibility of collecting the standard set retrospectively. This retrospective observational comparative benchmark study included six interstitial lung disease expert centres based in the Netherlands, Belgium, the UK and the USA. The standard set of outcome measures included 1) mortality, 2) changes in pulmonary function (forced vital capacity (FVC), forced expiratory volume in 1 s, diffusing capacity of the lung for carbon monoxide), 3) soluble interleukin-2 receptor (sIL-2R) change, 4) weight changes, 5) quality-of-life (QoL) measures, 6) osteoporosis and 7) clinical outcome status (COS). Data collection was considered feasible if the data were collected in ⩾80% of all patients. 509 patients were included in the retrospective cohort. In total six patients died, with a mean survival of 38±23.4 months after the diagnosis. Centres varied in mean baseline FVC, ranging from 110 (95% CI 92–124)% predicted to 99 (95% CI 97–123)% pred. Mean baseline body mass index (BMI) of patients in the different centres varied between 27 (95% CI 23.6–29.4) kg·m−2 and 31.8 (95% CI 28.1–35.6) kg·m−2. 310 (60.9%) patients were still on systemic therapy 2 years after the diagnosis. It was feasible to measure mortality, changes in pulmonary function, weight changes and COS. It is not (yet) feasible to retrospectively collect sIL-2R, osteoporosis and QoL data internationally. This study shows that data collection for the standard set of outcome measures for pulmonary sarcoidosis was feasible for four out of seven outcome measures. Trends in pulmonary function and BMI were similar for different hospitals when comparing different practices
The On-orbit Calibrations for the Fermi Large Area Telescope
The Large Area Telescope (LAT) on--board the Fermi Gamma ray Space Telescope
began its on--orbit operations on June 23, 2008. Calibrations, defined in a
generic sense, correspond to synchronization of trigger signals, optimization
of delays for latching data, determination of detector thresholds, gains and
responses, evaluation of the perimeter of the South Atlantic Anomaly (SAA),
measurements of live time, of absolute time, and internal and spacecraft
boresight alignments. Here we describe on orbit calibration results obtained
using known astrophysical sources, galactic cosmic rays, and charge injection
into the front-end electronics of each detector. Instrument response functions
will be described in a separate publication. This paper demonstrates the
stability of calibrations and describes minor changes observed since launch.
These results have been used to calibrate the LAT datasets to be publicly
released in August 2009.Comment: 60 pages, 34 figures, submitted to Astroparticle Physic
Therapeutic approach of hepatic sarcoidosis.
PURPOSE OF REVIEW: Surveillance of hepatic involvement in sarcoidosis has not been standardized. Therefore, management of hepatic involvement is a clinical challenge. This review analyses published data on the pharmacological treatment of hepatic sarcoidosis. RECENT FINDINGS: Only 5-30% of patients with hepatic sarcoidosis display symptoms. Occasionally, it has a rapid progressive course with serious complications, stressing an appropriate and carefully timed therapeutic approach. Because symptomatic hepatic sarcoidosis is uncommon, therapeutic studies are scarce. Answers to the questions when to initiate which treatment are lacking. Case reports describe beneficial effects of prednisone and the augmentation of cytotoxic and anti-tumor necrotic factor-alpha (TNF-alpha) therapy. However, because of small sample sizes, no meaningful conclusions could be drawn. In symptomatic hepatic sarcoidosis patients, it is recommended to start to treat the sarcoidosis with prednisone, preceded by ursodeoxycholic acid when signs of cholestasis are present. In refractory cases or when prednisone weaning is impossible, cytotoxic drugs or anti-TNF-alpha therapy should be considered. SUMMARY: This review illustrates the importance of an appropriate therapeutic approach of sarcoidosis patients with hepatic involvement. It emphasizes the need for future studies to evaluate treatment options to avoid disease progression and hepatic complications
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