Both the hyperproduction of oxygen free radicals (OFR) and the weakening of natural scavenging mechanisms have been implicated as contributors to multiple organ failure in septic shock. This study examined whether the antioxidants glutathione (GSH) and N-acetyl-L-cysteine (NAC) play a protective role against damage by OFR in early septic shock. We randomly entered 30 patients with septic shock into one of three groups within 24 h of diagnosis. All of the patients received septic shock therapy, including parenteral nutrition, antibiotics, and volume-expanding and inotropic agents. One group (Group B) also received 70 mg/kg/d of intravenous GSH, and a second group (Group C), 70 mg/kg/d of intravenous GSH and 75 mg/kg/d of intravenous NAC. The protection against OFR damage was evaluated by measuring expired ethane, plasma malondialdehyde, erythrocyte deformability, complement activation, and clinical scores at admission and on Days 3 and 5 of treatment. A significant decrease in peroxidative indexes was observed at Day 5 in Group B as compared with both the control group and basal values. The decrease in peroxidative indexes was even more marked in Group C. Clinical scores in this group were also significantly improved. In conclusion, the administration of high doses of NAC added to GSH significantly decreased the peroxidative stress of patients with septic shock.

Trisomy of chromosome 21 (TS21) is the most common autosomal aneuploidy compatible with postnatal survival with a prevalence of 1 in 700 newborns. Its phenotype is highly complex with constant features, such as mental retardation, dysmorphic traits and hypotonia, and variable features including heart defects, susceptibility to Alzheimer’s disease (AD), type 2 diabetes, obesity and immune disorders. Overexpression of genes on chromosome-21 (Hsa21) is responsible for the pathogenesis of Down syndrome (DS) phenotypic features either in a direct or in an indirect manner since many Hsa21 genes can affect the expression of other genes mapping to different chromosomes. Many of these genes are involved in mitochondrial function and energy conversion, and play a central role in the mitochondrial dysfunction and chronic oxidative stress, consistently observed in DS subjects.Recent studies highlight the deep interconnections between mitochondrial dysfunction and DS phenotype. In this short review we first provide a basic overview of mitochondrial phenotype in DS cells and tissues. We then discuss how specific Hsa21 genes may be involved in determining the disruption of mitochondrial DS phenotype and biogenesis. Finally we briefly focus on drugs that affect mitochondrial function and mitochondrial network suggesting possible therapeutic approaches to improve and/or prevent some aspects of the DS phenotype.

product, yielding phenylene-modified hybrid mesoporous ethenylenesilica (Ph-HME). The Ph-HME was then sulfonated by heating at 80 C for 3 days in concentrated H 2 SO 4 . The solid was recovered and washed in water at 80 C for 6 h. The washing treatment was repeated twice, and the final product was dried at room temperature to afford Ph±SO 3 H HME.Acid-Catalyzed Reaction: The esterification of acetic acid and pinacol±pinacolone rearrangement were employed as test reactions to examine the acid properties of the prepared samples. Esterification of acetic acid was examined in the liquid phase as follows: 0.20 g of the catalyst was evacuated at 423 K for 1 h and then added to a mixture of acetic acid (0.10 mol) and ethanol (1.0 mol). The reaction was carried out at 343 K in an Ar atmosphere, and the liquid phase was analyzed using gas chromatography with capillary columns. Pinacol± pinacolone rearrangement was performed in the liquid phase as follows: 0.20 g of the catalyst was evacuated at 423 K for 1 h and then added to pinacol (42.3 mol) without the addition of solvent. The reaction was carried out at 403 K in an Ar atmosphere, and the liquid phase was analyzed using gas chromatography with capillary columns.Measurement: Nitrogen adsorption±desorption isotherms were measured at 77 K using a Micrometrics Coulter SA-3100 system. Prior to measurement, the samples were pretreated at 423 K for 3 h under vacuum to a final pressure of 1 10 ±5 torr (1 torr = 133 Pa). The Brunauer±Emmett±Teller (BET) surface area was estimated over a relative pressure (P/P 0 ) range of 0.05±0.30. The pore size distribution was obtained from an analysis of the adsorption branch of the isotherms using the Barrett±Joyner±Halenda (BJH) method. Solid-state 13 C and 29 Si CP-MAS NMR measurements were acquired on Bruker ASX200 and ASX400 spectrometers, respectively. The Larmor frequencies for 13 C and 29 Si were 50.3 and 79.5 MHz, respectively. The experimental parameters were as follows.13 C CP-MAS NMR: 4.0 kHz spin rate, 5 s recycle delay, 2 ms contact time, and 4.0 ls p/2 pulse width.29 Si CP-MAS NMR experiment: 4.0 kHz spin rate, 5 s recycle delay, 5 ms contact time, 5.9 ls p/2 pulse width. The chemical shifts of 13 C and 29 Si spectra were expressed with respect to neat tetramethylsilane.

Focal task-specific dystonia of the musicians' hand (FTSDmh) is an occupational movement disorder that affects instrumental musicians and often derails careers. There has been speculation on the role of intense practice or the specific technical demands of various instruments as triggers for the development of FTSDmh. In this study, we review the clinical features of all published cases (899 patients) and 61 previously unpublished cases of FTSDmh. Our primary goals were to search for patterns in the clinical phenotype, and to discern if specific instrumental technical demands might be related to the development of dystonia. Symptoms of FTSDmh began at a mean age 35.7 years (SD = 10.6), with an overwhelming male predominance (M:F = 4.1:1). The right hand was preferentially affected in keyboard and plucked string players (77%), and the left hand in bowed string players (68%). Flexion movements were the most common dystonic movement in each instrument class, and fingers 3, 4, and 5, either in isolation or combination, were most frequently involved. The clinical implications of these findings and their possible relationship to the pathophysiology of focal task-specific dystonia are explored.