Objective. To evaluate health-related quality of life (HRQOL) in patients with systemic sclerosis (SSc) using the Short Form 36 (SF-36) and to correlate SF-36 scores with clinical and biologic markers. Methods. The SF-36 was administered to 24 controls and 24 SSc patients. SSc patients also were evaluated for subset (limited SSc [lSSc] and diffuse SSc [dSSc]), age, disease duration, angiotensin-converting enzyme (ACE) levels, autoantibodies, and skin and internal organ involvement. Results. The physical summary score (PSS) was lower in SSc patients than in controls (P < 0.05), whereas the mental summary score (MSS) was higher in dSSc than in lSSc patients (P < 0.05). Five of 8 single SF-36 domain scores were lower in SSc patients than in controls (P < 0.05). Vitality was higher in dSSc than in controls (P < 0.001). In SSc, elder age correlated with lower PSS; low ACE levels and high skin score correlated with higher general mental health and role limitations due to physical problems, respectively (P < 0.05). Patients with heart involvement had higher scores in general health perceptions (P < 0.05). Conclusion. The SF-36 shows that HRQOL is impaired in patients with SSc. Higher scores in MSS and vitality in patients with dSSc and correlations of high SF-36 scores with specific organ involvement suggest that SSc patients with severe disease are more able to cope with HRQOL modification.

No abstract

The patients with a lethal outcome were presented under the eight pharmacologic-therapeutic classes of drugs and the classification proposed by NS Irey. This is based on long histopathologic experience and helps to identify preventable risks.

Airborne particulate matter samples were collected in an urban and a rural-suburban monitoring stations of the city of Rome, Italy, and the particles were analyzed through the Raman microspectroscopy. A careful examination of the spectral bands, performed with a five-(Voigt) curve deconvolution model previously described by the literature and here adapted to the purpose, lead to the characterization of the graphitic and carbonaceous material plus the identification of the mineral particles associated with it. Statistical analysis of the full-width at half-maximum (FWHM) values of the bands, as well as of their intensity ratio, revealed the presence of two classes of soot particles that can be ascribed to a different degree of crystallinity. The population of soot collected at the urban site, where the vehicular emission component prevails, exhibits mostly crystalline characteristics (with a D1 FWHM of 150-155 cm −1 ), whereas the population collected at the rural-suburban site, particularly the coarse fraction, shows a prevailing amorphous nature (with a D1 FWHM of ∼175 cm −1 ). A similar aspect emerges for the pure black carbon particles, mainly crystalline, and the black carbon particles associated with minerals, generally disordered. These results add useful information and characterization of the soot, a relevant component of the ambient air, and its different features with respect to the urban or rural-suburban areas.

The central and peripheral nervous systems are variably affected in the rheumatic diseases. Automated standardized infrared pupillometry allows the safe, noninvasive assessment of the pupillary innervation. Pupillometry has already been used in studying the autonomic nervous system (ANS) in various rheumatic diseases. In systemic lupus erythematosus, the irideal parasympathetic branch of ANS was more affected then the sympathetic branch. In Sjögren's syndrome, signs of pupillary parasympathetic denervation have been reported. In rheumatoid arthritis, pupil parasympathetic dysfunction has been shown to correlate with ocular dryness. In systemic sclerosis (SSc), both sympathetic and parasympathetic irideal impairment have been demonstrated. Beside providing autonomic innervation, sensory nerves fibers are able to control iris diameter. Exogenous ocular instillation of substance P (SP), a sensory neuropeptide, can determine an omathropine-resistant, non-cholinergic myosis, acting on specific receptors present on the iris sphincter muscle. We first studied pupillary SP-ergic responsiveness in SSc, evaluating substance P (SP)-stimulated pupillary diameters by pupillometry. A higher basal and SP-stimulated myosis was found in lSSc versus both dSSc and controls, whereas no differences existed between dSSc and controls. From the literature, the pupillary parasympathetic nervous system seems to be more affected than the sympathetic branch of ANS in the rheumatic diseases characterized by an inflammatory status. However, we found in SSc both sympathetic and parasympathetic pupil control to be equally impaired. From our experience, we conclude that pupillary nervous control is differently affected in the two subsets of SSc, and that the SP-ergic system seems to be impaired only in lSSc.

Pyrochlore-group minerals that exhibit high Si contents are fairly common in geochemically evolved parageneses. However, the role of Si in the structure of these minerals is unclear. Different explanations have been invoked to clarify the way in which Si is incorporated in natural pyrochlores. These include the presence of impurities, the presence of Si in an amorphous or dispersed state, and its presence as an essential part of the structure.This paper reports an integrated XREF, SEM, EMPA, and TEM study on pyrochlore samples with high SiO 2 content (up to 11.51 wt%) from Narssârssuk, Julianehaab district (Greenland). TEM observations reveal that Si-poor areas have strong and sharp diffraction peaks, whereas the Si-rich areas showed weaker spots with the diffuse diffraction halo typical of a metamict material. No evidence of crystalline phases other than pyrochlore was observed. Two single crystals having the unit-cell parameter a = 10.4200(7) and 10.3738(7) Å, respectively, were analyzed by X-ray diffraction and the structure was refi ned to R obs = 2.62 and 4.35%. On the basis of both the refi ned site scattering and the octahedral bond distance and the results of the TEM investigation, only a fraction (~30-50%) of the Si detected by EMPA is incorporated in the structure. A comparison with structural data of Si-free pyrochlores reported in the literature supports this assumption and allows a linear multiple regression to model the effect of the substitution of (Nb,Ta) by Ti and Si. The remaining 50-70% of the total silicon detected is incorporated in the radiation-damaged portions of pyrochlore.

No abstract

The involvement of Gi proteins in the modulation of pain perception has been widely established, and mutations in G-proteins have already been identified as the aetiopathological cause of human diseases. The aim of the present study was to determine whether a deficiency or a hypofunctionality of the Gi proteins occurred in primary headache. The functionality and the level of expression of Gi proteins were investigated in lymphocytes from migraine without aura, migraine with aura and cluster headache sufferers. A reduced capability to inhibit forskolin-stimulated adenylyl cyclase activity in headache patients was observed. Migraine patients also showed basal adenosine cAMP levels about four times higher than controls. The reduced activity of Gi proteins seems not to be related to a reduction of protein levels since no significant reduction of the Gialpha subunits was observed. These results indicate Gi protein hypofunctionality as an aetiopathogenic mechanism in migraine and cluster headache.