Assessment of plasma chitotriosidase activity, CCL18/PARC concentration and NP-C suspicion index in the diagnosis of Niemann-Pick disease type C: A prospective observational study

Background: Niemann-Pick disease type C (NP-C) is a rare, autosomal recessive neurodegenerative disease caused by mutations in either the NPC1 or NPC2 genes. The diagnosis of NP-C remains challenging due to the non-specific, heterogeneous nature of signs/symptoms. This study assessed the utility of plasma chitotriosidase (ChT) and Chemokine (C-C motif) ligand 18 (CCL18)/pulmonary and activation-regulated chemokine (PARC) in conjunction with the NP-C suspicion index (NP-C SI) for guiding confirmatory laboratory testing in patients with suspected NP-C. Methods: In a prospective observational cohort study, incorporating a retrospective determination of NP-C SI scores, two different diagnostic approaches were applied in two separate groups of unrelated patients from 51 Spanish medical centers (n = 118 in both groups). From Jan 2010 to Apr 2012 (Period 1), patients with =2 clinical signs/symptoms of NP-C were considered ''suspected NP-C'' cases, and NPC1/NPC2 sequencing, plasma chitotriosidase (ChT), CCL18/PARC and sphingomyelinase levels were assessed. Based on findings in Period 1, plasma ChT and CCL18/PARC, and NP-C SI prediction scores were determined in a second group of patients between May 2012 and Apr 2014 (Period 2), and NPC1 and NPC2 were sequenced only in those with elevated ChT and/or elevated CCL18/PARC and/or NP-C SI =70. Filipin staining and 7-ketocholesterol (7-KC) measurements were performed in all patients with NP-C gene mutations, where possible. Results: In total across Periods 1 and 2, 10/236 (4%) patients had a confirmed diagnosis o NP-C based on gene sequencing (5/118 4.2%] in each Period): all of these patients had two causal NPC1 mutations. Single mutant NPC1 alleles were detected in 8/236 (3%) patients, overall. Positive filipin staining results comprised three classical and five variant biochemical phenotypes. No NPC2 mutations were detected. All patients with NPC1 mutations had high ChT activity, high CCL18/PARC concentrations and/or NP-C SI scores =70. Plasma 7-KC was higher than control cut-off values in all patients with two NPC1 mutations, and in the majority of patients with single mutations. Family studies identified three further NP-C patients. Conclusion: This approach may be very useful for laboratories that do not have mass spectrometry facilities and therefore, they cannot use other NP-C biomarkers for diagnosis

International nosocomial infection control consortium (INICC) report, data summary of 36 countries, for 2004-2009

The results of a surveillance study conducted by the International Nosocomial Infection Control Consortium (INICC) from January 2004 through December 2009 in 422 intensive care units (ICUs) of 36 countries in Latin America, Asia, Africa, and Europe are reported. During the 6-year study period, using Centers for Disease Control and Prevention (CDC) National Healthcare Safety Network (NHSN; formerly the National Nosocomial Infection Surveillance system [NNIS]) definitions for device-associated health care-associated infections, we gathered prospective data from 313,008 patients hospitalized in the consortium's ICUs for an aggregate of 2,194,897 ICU bed-days. Despite the fact that the use of devices in the developing countries' ICUs was remarkably similar to that reported in US ICUs in the CDC's NHSN, rates of device-associated nosocomial infection were significantly higher in the ICUs of the INICC hospitals; the pooled rate of central line-associated bloodstream infection in the INICC ICUs of 6.8 per 1,000 central line-days was more than 3-fold higher than the 2.0 per 1,000 central line-days reported in comparable US ICUs. The overall rate of ventilator-associated pneumonia also was far higher (15.8 vs 3.3 per 1,000 ventilator-days), as was the rate of catheter-associated urinary tract infection (6.3 vs. 3.3 per 1,000 catheter-days). Notably, the frequencies of resistance of Pseudomonas aeruginosa isolates to imipenem (47.2% vs 23.0%), Klebsiella pneumoniae isolates to ceftazidime (76.3% vs 27.1%), Escherichia coli isolates to ceftazidime (66.7% vs 8.1%), Staphylococcus aureus isolates to methicillin (84.4% vs 56.8%), were also higher in the consortium's ICUs, and the crude unadjusted excess mortalities of device-related infections ranged from 7.3% (for catheter-associated urinary tract infection) to 15.2% (for ventilator-associated pneumonia). Copyright © 2012 by the Association for Professionals in Infection Control and Epidemiology, Inc. Published by Elsevier Inc. All rights reserved

Simulated Moving Bed Chromatography In The Production Of Enantiomerically Pure Or Enriched Compounds In Large Scale [cromatografia Em Leito Móvel Simulado Na Produção De Substâncias Enantiomericamente Puras Ou Enriquecidas Em Larga Escala]

There is great interest nowadays in the use of preparative liquid chromatography as an effective tool for the production of enantiomerically pure, or enriched, compounds for the pharmaceutical industry. To make the chromatographic process economically attractive, attention is now focused on the choice of the chromatographic operating mode to minimize eluent consumption and to maximize productivity. Among the alternatives to the traditional batch chromatography, attention is now shifting towards simulated moving bed (SMB) technologies and a review covering the latest developments in this area seems timely. Several aspects of this important analytical technique are presented and details concerning the SMB technology for process optimization are outlined.29510271037Wang, X., Ching, C.-B., (2004) J. Chromatogr., A, 1035, p. 167Aboul-Enein, H.Y., (2001) J. Chromatogr., A, 906, p. 185Maier, N., Franco, P., Linder, W., (2001) J. 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Investigation of electronic structure, morphological features, optical, colorimetric, and supercapacitor electrode properties of CoWO4 crystals

Cobalt tungstate (CoWO4) crystals were synthesized by the co-precipitation (CP) and polymeric precursor (PP) methods with posterior heat treatment at 800 °C for 4 h. The electronic structure, morphological features, optical, colorimetric, and supercapacitive properties were investigated in detail. X-ray diffraction, Rietveld refinement data, micro-Raman spectra, and Fourier transform-infrared spectra proved the crystallization of both CoWO4 materials with a wolframite-type monoclinic structure. Rietveld refinement data were employed as input data to simulate all clusters found in this crystalline structure as well as electron density maps. These results indicated the existence of distortions in both octahedral [CoO6] and [WO6] clusters, yielding an inhomogeneous charge distribution in the monoclinic lattice. Field emission scanning electron microscopy and transmission electron microscopy techniques show the presence of asymmetrical CoWO4 crystals. The ultraviolet–visible diffuse reflectance spectroscopy revealed optical band energy values of 2.84 and 2.89 eV for CoWO4 crystals prepared by the CP and PP methods, respectively. Colorimetric results indicated that the CoWO4 crystals have a desirable feature for the development of blue inorganic pigments. The experimental specific capacitance measurements of CoWO4 crystals as an electrode (CP and PP) were 192.5 Fg−1 and 249.1 Fg−1 at 40 mV s−1 and 5 mV s−1 in an electrode with 0.4 mg and 0.8 mg of electroactive materials in 1 M Na2SO4 solution, respectively

Microstructural And Electrical Properties Of Pbtio 3 Screen-printed Thick Films

In this article we will study the structural and electrical properties of PbTiO 3 (PTO) screen-printed thick films. These properties can change with synthetic routes employed for synthesis of PTO. The ceramic powder has been synthesized by mechanical alloying and solid state route. This material crystallizes in the tetragonal system, with space group P4mm and unit cell dimensions a = b = 3.904, c = 4.152 Å, and Z = 1. From our results, it was observed that PTO obtained by mechanical alloying provides smaller crystallite size. The Rietveld refinement showed that the samples synthesized by mechanical alloying and solid state route crystallize in the tetragonal system but with different cell parameters. The Raman shifts of these films agree with modes from literature. However, some displacements happen, because the morphology and structural characteristics of the thick films are different, compared to single crystal and thin film. The photomicrographs of the PTO thick films showed that the choice of synthetic routes obtained samples with different microstructures. © Springer Science+Business Media, LLC 2007.1910973980Jha, P., Ganguli, A.K., (2003) Proc. Indian Acad. Sci. (Chem. Sci.), 115, p. 431Kolar, D., Suvorov, D., (1995) Eur. J. Solid State Inorg. Chem., 32, p. 751Akbas, M.A., Davies, P.K., (1998) J. Am. Ceram. Soc., 81, p. 670Akbas, M.A., Davies, P.K., (1997) J. Am. Ceram. Soc., 80, p. 1727Cava, R.J., (2001) J. Mater. Chem., 11, p. 54Cava, R.J., Krajewski, J.J., Roth, R.S., (1999) Mater. Res. Bull., 34, p. 355Thirumal, M., Jawahar, I.N., Surendran, K.P., Mohanan, P., Ganguli, A.K., (2002) Mater. Res. Bull., 37, p. 185Lee, H.J., Hong, K.S., Kim, S.J., Kim, I.T., (1997) Mater. Res. Bull., 32, p. 847Wu, Y.J., Chen, X.M., (2001) J. Mater. Res., 16, p. 1734Subramanian, M.A., Li, D., Duan, N., Reisner, B.A., Sleight, A.W., (2000) J. Solid State Chem., 151, p. 323Homes, C.C., Vogt, T., Shapiro, S.M., Wakimoto, S., Ramirez, A.P., (2001) Science, 293, p. 673Jha, P., Arora, P., Ganguli, A.K., (2003) Mater. Lett., 57, p. 2443Kretly, L.C., Almeida, A.F.L., Fechine, P.B.A., de Oliveira, R.S., Sombra, A.S.B., (2004) J. Mater. Sci.: Mater. Electron., 15, p. 657Almeida, A.F.L., Fechine, P.B.A., Góes, J.C., Valente, M.A., Miranda, M.A.R., Sombra, A.S.B., (2004) Mater. Sci. Eng. B, 111, pp. 113-123Valim, D., Souza Filho, A.G., Freire, P.T.C., Fagan, S.B., Ayala, A.P., Mendes Filho, J., Almeida, A.F.L., Sombra, A.S.B., (2004) Phys. Rev. B, 70, p. 132103Moulson, A.J., Herbert, J.M., (1997) Electroceramics (Materials-Properties-Applications), , (Chapman & Hall, London)Goodman, G., Buchanan, R.C., Reynolds, T.G., (1991) Ceramic Materials for Electronics, , in ed. by R.C. Buchanan (Marcel Dekker Inc., New york)dos Santos, L.P.S., Caracterização óptica e estrutural de PbTiO 3 nanoestruturado obtido por moagem de alta energia (2002), Dissertação de Mestrado, Universidade de São PauloMazon, T., Obtenção de PZN com fase e microestrutura controladas (1997), 142 f., Universidade Estadual PaulistaUdompom, A., Ananta, S., (2004) Curr. Appl. Phys., 4, p. 186Megaw, H.D., (1945) Proc. Phys. Soc., 58, p. 133Udomporn, A., Anata, S., (2004) Mater. Lett., 58, p. 1154Forrester, J.S., Zobec, J.S., Phelan, D., Kisi, E.H., (2004) J. Solid State Chem., 117, p. 3553Szwagierczak, D., Kulawik, J., (2004) J. Eur. Ceram. Soc., 24, p. 1979Rietveld, H.M., (1969) J. Appl. Crystallogr., 10, p. 65Larson, A.C., Von Dreele, R.B., (2004) General Structure Analysis System (GSAS), pp. 86-748. , Los Alamos National Laboratory Report LAURTompson, P., Cox, D.E., Hastings, J.B., (1987) J. Appl. Crystallogr., 20, p. 79Young, R.A., Desai, P., (1989) Arch. Nauki Mater., 71Paiva-Santos, C.O., Cavalheiro, A.A., Zaghete, M.A., Cilense, M., Varela, J.A., Silva Giotto, M.T., Mascarenhas, Y.P., (2001) Adv. X Ray Anal., 44, p. 38Scherrer, P., (1918) Nachr. Ges. Wiss. Gottingen, Math.-Phys. Kl., 2, p. 96Azároff, L.V., (1968) Elements of X-ray Crystallography, , (McGraw-Hill, New York)Stokes, A.R., Wilson, A.J.C., (1944) Proc. Phys. Soc. London, 56, p. 174Foster, C.M., Li, Z., Grimsditch, M., Chan, S.K., (1993) D.J. Lam, Phys. Rev. B, 48, p. 10160Taguchi, I., Pignolet, A., Wang, L., Proctor, M., Levynand, F., Schimd, P.E., (1993) J. Appl. Phys., 73, p. 394Mendiola, J., Calzada, M.L., Ramos, P., Martin, M.J., Agulló-Rueda, F., (1998) Thin Solid Films, 315, p. 19