Atteinte pulmonaire sévère au cours de la neurofibromatose de type 1

Type 1 neurofibromatosis (NF1) is a hereditary disease inherited as an autosomal dominant. Respiratory involvement is rare. We report the case of a woman suffering from NF1 with mutation of the corresponding gene and with respiratory involvement combining diffuse parenchymatous lesions, severe precapillary pulmonary hypertension and an enlarging, spiculated pulmonary nodule, very suspicious of malignancy, though histological examination was not possible on account of the patient\u27s poor respiratory function. There was progressive deterioration of the patient\u27s respiratory condition, leading to death, despite the introduction of oral therapy combining a phosphodiesterase 5 inhibitor and an endothelin receptor antagonist. Our case illustrates the development of multiple severe respiratory pathologies in the setting of NF1. The specific contribution of the NF1 gene mutation in the pathophysiology of these different pulmonary manifestations needs to be examined in detail

The Photodetector Array Camera and Spectrometer (PACS) on the Herschel Space Observatory

The Photodetector Array Camera and Spectrometer (PACS) is one of the three science instruments on ESA's far infrared and submillimetre observatory. It employs two Ge:Ga photoconductor arrays (stressed and unstressed) with 16x25 pixels, each, and two filled silicon bolometer arrays with 16x32 and 32x64 pixels, respectively, to perform integral-field spectroscopy and imaging photometry in the 60-210\mu\ m wavelength regime. In photometry mode, it simultaneously images two bands, 60-85\mu\ m or 85-125\mu\m and 125-210\mu\ m, over a field of view of ~1.75'x3.5', with close to Nyquist beam sampling in each band. In spectroscopy mode, it images a field of 47"x47", resolved into 5x5 pixels, with an instantaneous spectral coverage of ~1500km/s and a spectral resolution of ~175km/s. We summarise the design of the instrument, describe observing modes, calibration, and data analysis methods, and present our current assessment of the in-orbit performance of the instrument based on the Performance Verification tests. PACS is fully operational, and the achieved performance is close to or better than the pre-launch predictions

VIS: the visible imager for Euclid

Euclid-VIS is the large format visible imager for the ESA Euclid space mission in their Cosmic Vision program, scheduled for launch in 2020. Together with the near infrared imaging within the NISP instrument, it forms the basis of the weak lensing measurements of Euclid. VIS will image in a single r+i+z band from 550-900 nm over a field of view of ~0.5 deg2. By combining 4 exposures with a total of 2260 sec, VIS will reach to V=24.5 (10σ) for sources with extent ~0.3 arcsec. The image sampling is 0.1 arcsec. VIS will provide deep imaging with a tightly controlled and stable point spread function (PSF) over a wide survey area of 15000 deg2 to measure the cosmic shear from nearly 1.5 billion galaxies to high levels of accuracy, from which the cosmological parameters will be measured. In addition, VIS will also provide a legacy dataset with an unprecedented combination of spatial resolution, depth and area covering most of the extra-Galactic sky. Here we will present the results of the study carried out by the Euclid Consortium during the period up to the Preliminary Design Review. © (2014) COPYRIGHT Society of Photo-Optical Instrumentation Engineers (SPIE). Downloading of the abstract is permitted for personal use only

Pilot optical alignment

PILOT (Polarized Instrument for Long wavelength Observations of the Tenuous interstellar medium) is a balloonborne astronomy experiment designed to study the polarization of dust emission in the diffuse interstellar medium in our Galaxy. The PILOT instrument allows observations at wavelengths 240 μm and 550 μm with an angular resolution of about two arcminutes. The observations performed during the two first flights performed from Timmins, Ontario Canada, and from Alice-springs, Australia, respectively in September 2015 and in April 2017 have demonstrated the good performances of the instrument. Pilot optics is composed of an off axis Gregorian type telescope combined with a refractive re-imager system. All optical elements, except the primary mirror, which is at ambient temperature, are inside a cryostat and cooled down to 3K. The whole optical system is aligned on ground at room temperature using dedicated means and procedures in order to keep the tight requirements on the focus position and ensure the instrument optical performances during the various phases of a flight. We’ll present the optical performances and the firsts results obtained during the two first flight campaigns. The talk describes the system analysis, the alignment methods, and finally the inflight performances

The Control Unit of the KM3NeT Data Acquisition System

The KM3NeT Collaboration runs a multi-site neutrino observatory in the Mediterranean Sea. Water Cherenkov particle detectors, deep in the sea and far off the coasts of France and Italy, are already taking data while incremental construction progresses. Data Acquisition Control software is operating off-shore detectors as well as testing and qualification stations for their components. The software, named Control Unit, is highly modular. It can undergo upgrades and reconfiguration with the acquisition running. Interplay with the central database of the Collaboration is obtained in a way that allows for data taking even if Internet links fail. In order to simplify the management of computing resources in the long term, and to cope with possible hardware failures of one or more computers, the KM3NeT Control Unit software features a custom dynamic resource provisioning and failover technology, which is especially important for ensuring continuity in case of rare transient events in multi-messenger astronomy. The software architecture relies on ubiquitous tools and broadly adopted technologies and has been successfully tested on several operating systems

Pilot optical alignment

PILOT (Polarized Instrument for Long wavelength Observations of the Tenuous interstellar medium) is a balloonborne astronomy experiment designed to study the polarization of dust emission in the diffuse interstellar medium in our Galaxy. The PILOT instrument allows observations at wavelengths 240 μm (1.2THz) with an angular resolution about two arc-minutes. The observations performed during the first flight in September 2015 at Timmins, Ontario Canada, have demonstrated the optical performances of the instrument

In-Flight Performance of the HERSCHEL Sorption Coolers – One Year of Operation

Presented at the 16th International Cryocooler Conference, held May 17-20, 2008 in Atlanta, Georgia.HERSCHEL, the fourth ‘cornerstone’ mission in the ESA science program, was launched on 14 May 2009 from Kourou, French Guyana. With a 3.5 m Cassegrain telescope it is the largest space telescope ever launched. HERSCHEL is performing photometry and spectroscopy in the electromagnetic spectrum for wavelengths 55-672 μm. This bridges the gap between earlier infrared space missions and ground-based facilities. HERSCHEL’s payload consists of three instruments built by international scientific consortia: HIFI (Heterodyne Instrument for the Far Infrared), PACS (Photo-conductor Array Camera & Spectrometer) and SPIRE (Spectral and Photometric Imaging REceiver). Two of these instruments, SPIRE and PACS, use bolometric detectors cooled to 300 mK. The HERSCHEL cryogenic subsystem relies on passive cooling down to liquid nitrogen temperatures for both the cryostat & telescope. It features a 2367 liter superfluid helium tank that vents to space and provides the instruments with cooling at four interface temperatures between 1.6 K and 15 K. Two dedicated sorption coolers are then used to lower the PACS and SPIRE bolometer temperatures to below 300 mK. These units are single shot devices and need to be recycled on a regular basis. Their typical hold time is over 48 hours for less than 2 hours recycling time. To date these systems have been in operation for approximately a year. As of April 2010, over 150 re-cycles have been successfully performed and the performance of these coolers is stable and fully in line with predictions

Recent achievements on the development of the HERSCHEL/PACS bolometer arrays

International audienceA new type of bolometer arrays sensitive in the far Infrared and Submillimeter range has been developed and manufactured by CEA/LETI/SLIR since 1997. These arrays will be integrated in the PACS instrument (Photodetector Array Camera and Spectrometer) of ESA's Herschel Space Observatory (launch date 2007). The main innovations of CEA bolometers are their collective manufacturing technique (production of 3-side buttable 16×16 arrays) and their high mapping efficiency (large format detector and instantaneous Nyquist sampling). The measured NEP is 2.10W/Hz and the thermometric passband about 4 5 Hz. In this article we describe CEA bolometers and present the results obtained during the last test campaign