First On-Sky Fringes with an Up-Conversion Interferometer Tested on a Telescope Array.

The Astronomical Light Optical Hybrid Analysis project investigates the combined use of a telescope array interferometer and nonlinear optics to propose a new generation of instruments dedicated to high-resolution imaging for infrared astronomy. The nonlinear process of optical frequency conversion transfers the astronomical light to a shorter wavelength domain. Here, we report on the first fringes obtained on the sky with the prototype operated at 1.55 µm in the astronomical H band and implemented on the Center for High Angular Resolution Astronomy telescope array. This seminal result allows us to foresee a future extension to the challenging midinfrared spectral domain.

CYP24A1 Mutations in a Cohort of Hypercalcemic Patients: Evidence for a Recessive Trait.

CONTEXT: Loss-of-function mutations of CYP24A1 (which encodes the 25-OH-D3-24-hydroxylase) have recently been reported to cause hypercalcemia. OBJECTIVES: The aims of this study were: 1) to evaluate the frequency of CYP24A1 mutations in patients with medical history of hypercalcemia; 2) to show the clinical utility of a simultaneous assay of serum 25-hydroxyvitamin D3 (25-OH-D3) and 24,25-dihydroxyvitamin D3 (24,25-[OH]2D3) by liquid chromatography tandem mass spectrometry (LC-MS/MS); and 3) to investigate biochemical parameters in heterozygous gene carriers with CYP24A1 mutations. PATIENTS AND METHODS: We screened for CYP24A1 mutations in 72 patients with serum calcium levels > 2.6 mmol/L and PTH levels < 20 pg/mL and recruited 24 relatives after genetic counseling for subsequent investigations. Vitamin D metabolite concentrations were assessed in a subset of patients by LC-MS/MS and results expressed as a ratio (R) of 25-OH-D3:24,25-(OH)2D3. RESULTS: Twenty-five patients with hypercalcemia (35%) harbored CYP24A1 variations. Twenty (28%) had biallelic variations, mostly found in subjects with nephrocalcinosis or renal stones (19/20). Five patients, all neonates, were heterozygous, without renal disease. We describe 15 new variations leading to loss-of-function according to pathogenicity prediction programs, and we functionally characterized 5 of them in vitro. A dramatic increase of R, usually >80, was found in patients harboring biallelic mutations providing evidence in vivo for the loss of CYP24A1 activity. In contrast, R value remains <25 in patients without CYP24A1 mutations. Subjects carrying one mutant allele, hypercalcemic individuals, as well as gene-carrier relatives, had a detectable 24,25-(OH)2D3 level and R < 25, indicating normal 24-hydroxylase activity. CONCLUSION: CYP24A1 biallelic mutations are frequently found in patients presenting with hypercalcemia, low PTH, and renal disease. We confirm the accuracy and effectiveness of a novel blood test estimating the ratio between relevant vitamin D metabolites as a useful screening tool for CYP24A1 mutations. Haploinsufficiency is not associated with CYP24A1 deficiency.

Laboratory demonstration of spatial-coherence analysis of a blackbody through an up-conversion interferometer.

In the field of high resolution imaging in astronomy, we experimentally demonstrate the spatial-coherence analysis of a blackbody using an up-conversion interferometer in the photon counting regime. The infrared radiation of the blackbody is converted to a visible one in both arms of the interferometer thanks to the sum-frequency generation processes achieved in Ti-diffused periodically poled lithium niobate waveguides. The coherence analysis is performed through a dedicated imaging stage which mimics a classical telescope array analyzing an astrophysical source. The validity of these measurements is confirmed by the comparison with spatial-coherence analysis through a reference interferometer working at infrared wavelengths.

Non-invasive and quantitative assessment of sudomotor function for peripheral diabetic neuropathy evaluation.

AIMS: Perturbation of pain sensation is considered one of the major initiating risk factors for diabetic foot ulcer. Sweat dysfunction leading to abnormal skin conditions, including dryness and fissures, can increase foot ulcer risk. The aim of this study was to evaluate Sudoscan™, a new, quick, non-invasive and quantitative method of measuring sudomotor dysfunction as a co-indicator of the severity of diabetic polyneuropathy (DPN). METHODS: A total of 142 diabetic patients (age 62±18 years, diabetes duration 13±14 years, HbA(1c) 8.9±2.5%) were measured for vibration perception threshold (VPT), using a biothesiometer, and for sudomotor dysfunction, using electrochemical sweat conductance (ESC) based on the electrochemical reaction between sweat chloride and electrodes in contact with the hands and feet. Retinopathy status was also assessed, as well as reproducibility between two ESC measurements and the effect of glycaemia levels. RESULTS: ESC measurements in the feet of patients showed a descending trend from 66±17 µS to 43±39 µS, corresponding to an ascending trend in VPT threshold from <15 V to >25 V (P=0.001). Correlation between VPT and ESC was -0.45 (P<0.0001). Foot ESC was lower in patients with fissures, while VPT was comparable. Both VPT and foot ESC correlated with retinopathy status. Bland-Altman plots indicated good reproducibility between two measurements, and between low and high glycaemia levels. CONCLUSION: Sudoscan™ is a reproducible technique with results that are not influenced by blood glucose levels. Sweating status may be a quantitative indicator of the severity of polyneuropathy that may be useful for the early prevention of foot skin lesions.

The place of adsorption and biochromatography in extracorporeal liver support systems.

Artificial and bioartificial liver devices aim at replacing some or all liver functions in the cases of end stage or fulminant disorders. Among all of its function, liver plays a key role in detoxification of substances that are hydrosoluble or bound to albumin. In this paper, the authors first reviewed the requirements for temporary liver support, then the adsorption-based systems that can be found on the market and finally propose new applications of biochromatography using perfusion-based bioartificial systems.

Sodium alginate sponges with or without sodium hyaluronate: in vitro engineering of cartilage.

Studies are underway to design biosystems containing embedded chondrocytes to fill osteochondral defects and to produce a tissue close to native cartilage. In the present report, a new alginate three-dimensional support for chondrocyte culture is described. A sodium alginate solution, with or without hyaluronic acid (HA), was freeze-dried to obtain large-porosity sponges. This formulation was compared with a hydrogel of the same composition. In the sponge formulation, macroscopic and microscopic studies demonstrated the formation of a macroporous network (average pore size, 174 microm) associated with a microporous one (average pore size, 250 nm). Histological and biochemical studies showed that, when loaded with HA, the sponge provides an adapted environment for proteoglycan and collagen synthesis by chondrocytes. Cytoskeleton organization was studied by three-dimensional fluorescence microscopy (CellScan EPR). Chondrocytes exhibit a marked spherical shape with a nonoriented and sparse actin microfilament network. Type II collagen was detected in both types of sponges (with or without HA) using immunohistochemistry. In conclusion, the sponge formulation affords new perspectives with respect to the in vitro production of "artificial" cartilage. Furthermore, the presence of hyaluronate within the alginate sponge mimics a functional environment, suitable for the production by embedded chondrocytes of an extracellular matrix.