Artykuły w czasopismach: „PCCD”

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Kuner, Valerie, Nicole van Veelen, Stephanie Studer, Bryan Van de Wall, Jürgen Fornaro, Michael Stickel, Matthias Knobe, Reto Babst, Frank J. P. Beeres i Björn-Christian Link. "Application of Pelvic Circumferential Compression Devices in Pelvic Ring Fractures—Are Guidelines Followed in Daily Practice?" Journal of Clinical Medicine 10, nr 6 (21.03.2021): 1297. http://dx.doi.org/10.3390/jcm10061297.

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Early administration of a pelvic circumferential compression device (PCCD) is recommended for suspected pelvic trauma. This study was conducted to evaluate the prevalence of PCCD in patients with pelvic fractures assigned to the resuscitation room (RR) of a Level I trauma center. Furthermore, correct application of the PCCD as well as associated injuries with potential clinical sequelae were assessed. All patients with pelvic fractures assigned to the RR of a level one trauma center between 2016 and 2017 were evaluated retrospectively. Presence and position of the PCCD on the initial trauma scan were assessed and rated. Associated injuries with potential adverse effects on clinical outcome were analysed. Seventy-seven patients were included, of which 26 (34%) had a PCCD in place. Eighteen (23%) patients had an unstable fracture pattern of whom ten (56%) had received a PCCD. The PCCD was correctly placed in four (15%) cases, acceptable in 12 (46%) and incorrectly in ten (39%). Of all patients with pelvic fractures (n = 77, 100%) treated in the RR, only one third (n = 26, 34%) had a PCCD. In addition, 39% of PCCDs were positioned incorrectly. Of the patients with unstable pelvic fractures (n = 18, 100%), more than half either did not receive any PCCD (n = 8, 44%) or had one which was inadequately positioned (n = 2, 11 %). These results underline that preclinical and clinical education programs on PCCD indication and application should be critically reassessed.

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Asatryan, Babken, i Argelia Medeiros-Domingo. "Molecular and genetic insights into progressive cardiac conduction disease". EP Europace 21, nr 8 (26.04.2019): 1145–58. http://dx.doi.org/10.1093/europace/euz109.

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Abstract Progressive cardiac conduction disease (PCCD) is often a primarily genetic disorder, with clinical and genetic overlaps with other inherited cardiac and metabolic diseases. A number of genes have been implicated in PCCD pathogenesis with or without structural heart disease or systemic manifestations. Precise genetic diagnosis contributes to risk stratification, better selection of specific therapy and allows familiar cascade screening. Cardiologists should be aware of the different phenotypes emerging from different gene-mutations and the potential risk of sudden cardiac death. Genetic forms of PCCD often overlap or coexist with other inherited heart diseases or manifest in the context of multisystem syndromes. Despite the significant advances in the knowledge of the genetic architecture of PCCD and overlapping diseases, in a measurable fraction of PCCD cases, including in familial clustering of disease, investigations of known cardiac disease-associated genes fail to reveal the underlying substrate, suggesting that new causal genes are yet to be discovered. Here, we provide insight into genetics and molecular mechanisms of PCCD and related diseases. We also highlight the phenotypic overlaps of PCCD with other inherited cardiac and metabolic diseases, present unmet challenges in clinical practice, and summarize the available therapeutic options for affected patients.

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Li, Tianwei, Jicheng Hu, Chenyang Xu i Jun Jin. "PCBs, PCNs, and PCDD/Fs in Soil around an Industrial Park in Northwest China: Levels, Source Apportionment, and Human Health Risk". International Journal of Environmental Research and Public Health 20, nr 4 (16.02.2023): 3478. http://dx.doi.org/10.3390/ijerph20043478.

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The concentrations of polychlorinated biphenyls (PCBs), polychlorinated naphthalenes (PCNs), and polychlorinated dibenzo-p-dioxins and dibenzofurans (PCDD/Fs) were determined in soil samples collected around an industrial park in Northwest China, to investigate the potential impacts of park emissions on the surrounding environment. The total concentration ranges of PCBs, PCNs, and PCDD/Fs in the soil samples were in 13.2–1240, 141–832, and 3.60–156 pg/g, respectively. The spatial distribution and congener patterns of PCBs, PCNs, and PCCD/Fs indicated that there might be multiple contamination sources in the study area, so source apportionments of PCBs, PCNs, and PCCD/Fs were performed by a positive matrix factorization model based on the concentrations of all target congeners together. The results revealed that these highly chlorinated congeners (CB-209, CN-75, and OCDF) might be derived from phthalocyanine pigments, the legacy of Halowax 1051 and 2,4-D products, which together contributed nearly half of the total concentration of target compounds (44.5%). In addition to highly chlorinated congeners, the local industrial thermal processes were mainly responsible for the contamination of PCBs, PCNs, and PCDD/Fs in the surrounding soil. The total carcinogenic risk of PCBs, PCNs, and PCDD/Fs in a few soil samples (0.22 × 10−6, 0.32 × 10−6, and 0.40 × 10−6) approached the threshold of potential carcinogenic risk (1.0 × 10−6). Since these pollutants can continuously accumulate in the soil, the contamination of PCBs, PCNs, and PCDD/Fs in surrounding soil deserves continuous attention.

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Garza, Alejandro J., Ireneusz W. Bulik, Thomas M. Henderson i Gustavo E. Scuseria. "Range separated hybrids of pair coupled cluster doubles and density functionals". Physical Chemistry Chemical Physics 17, nr 34 (2015): 22412–22. http://dx.doi.org/10.1039/c5cp02773j.

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Silina, Ekaterina, Victor Stupin, Natalia Manturova, Vitaly Vasin, Konstantin Koreyba, Petr Litvitskiy, Alexander Saltykov i Zalim Balkizov. "Acute Skin Wounds Treated with Mesenchymal Stem Cells and Biopolymer Compositions Alone and in Combination: Evaluation of Agent Efficacy and Analysis of Healing Mechanisms". Pharmaceutics 13, nr 10 (22.09.2021): 1534. http://dx.doi.org/10.3390/pharmaceutics13101534.

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We studied the efficacy of using mesenchymal stem cells (MSC) and a polymeric compound (based on chitosan and cellulose with integrated cerium dioxide nanoparticles (PCCD)) in wound healing, and to compare the effects with various invasive and external drugs used for the same purpose. Two wounds were made on the backs of each of 112 Wistar rats, removing the skin. Eight groups were studied: Control_0—intact wounds; Control_ss—0.9% NaCl injections; MSC injections; Control_msc—intact wounds on the opposite side of the body from the MSC group; external application of the PCCD; external application of a combination of the drugs PCCD + MSC; DCh –ointment Dioxomethyltetrahydropyrimidine + Chloramphenicol; and DHCB—injections of a deproteinized hemoderivative of calf blood. After 14 days, we evaluated the state and size of the wounds, studied the level of microcirculation, performed a histological study, and identified and counted the different types of cells. The most effective remedy was combination PCCD + MSC. The treatments in the PCCD and MSC groups were more effective than in the DHCB and DCh groups. Invasive drugs and DCh slowed the regeneration process. DHCB did not affect the rate of healing for acute wounds without ischemia during the first week. The proven efficacy of developed polymeric compounds demonstrates the feasibility of further studies in clinical practice.

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Gu, Dong Liang, Shao Guang Liu, Li Ping Gu, Cheng Wu Chen, Jin Ming Wu i Yu Song Xu. "The Progress of V₂O₅/TiO₂-Based Catalyst in Hg⁰ Removal and PCCD/Fs Decomposition". Advanced Materials Research 873 (grudzień 2013): 534–39. http://dx.doi.org/10.4028/www.scientific.net/amr.873.534.

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The common form of vanadium-based catalyst and characteristics of plate-type catalyst are described in this paper. The catalytic mechanism of vanadium-based catalysts about Hg0-oxidation and De-PCCD/Fs is intensivly analyzed. The influencing factors of V2O5-WO3(MoO3)/TiO2in mercury removal and PCCD/Fs decomposition are expounded.

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Xu, Zhen, Yong Liu i Bang-Ce Ye. "PccD Regulates Branched-Chain Amino Acid Degradation and Exerts a Negative Effect on Erythromycin Production inSaccharopolyspora erythraea". Applied and Environmental Microbiology 84, nr 8 (9.02.2018): e00049-18. http://dx.doi.org/10.1128/aem.00049-18.

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ABSTRACTBranched-chain amino acid (BCAA) degradation is a major source of propionyl coenzyme A (propionyl-CoA), a key precursor of erythromycin biosynthesis inSaccharopolyspora erythraea. In this study, we found that thebkdoperon, responsible for BCAA degradation, was regulated directly by PccD, a transcriptional regulator of propionyl-CoA carboxylase genes. The transcriptional level of thebkdoperon was upregulated 5-fold in apccDgene deletion strain (ΔpccDstrain) and decreased 3-fold in apccDoverexpression strain (WT/pIB-pccD), demonstrating that PccD was a negative transcriptional regulator of the operon. The deletion ofpccDsignificantly improved the ΔpccDstrain's growth rate, whereaspccDoverexpression repressed WT/pIB-pccDgrowth rate, in basic Evans medium with 30 mM valine as the sole carbon and nitrogen source. The deletion ofgdhA1and the BcdhE1 gene (genes in thebkdoperon) resulted in lower growth rates of ΔgdhA1and ΔBcdhE1 strains, respectively, on 30 mM valine, further suggesting that thebkdoperon is involved in BCAA degradation. Bothbkdoverexpression (WT/pIB-bkd) andpccDinactivation (ΔpccDstrain) improve erythromycin production (38% and 64%, respectively), whereas the erythromycin production of strain WT/pIB-pccDwas decreased by 48%. Lastly, we explored the applications of engineeringpccDandbkdin an industrial high-erythromycin-producing strain.pccDdeletion in industrial strainS. erythraeaE3 (E3pccD) improved erythromycin production by 20%, and the overexpression ofbkdin E3ΔpccD(E3ΔpccD/pIB-bkd) increased erythromycin production by 39% compared withS. erythraeaE3 in an industrial fermentation medium. Addition of 30 mM valine to industrial fermentation medium further improved the erythromycin production by 23%, a 72% increase from the initial strainS. erythraeaE3.IMPORTANCEWe describe abkdoperon involved in BCAA degradation inS. erythraea. The genes of the operon are repressed by a TetR regulator, PccD. The results demonstrated that PccD controlled the supply of precursors for biosynthesis of erythromycin via regulating the BCAA degradation and propionyl-CoA assimilation and exerted a negative effect on erythromycin production. The findings reveal a regulatory mechanism in feeder pathways and provide new strategies for designing metabolic engineering to increase erythromycin yield.

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Boguslawski, Katharina. "Open-shell extensions to closed-shell pCCD". Chemical Communications 57, nr 92 (2021): 12277–80. http://dx.doi.org/10.1039/d1cc04539c.

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Zhang, Zhu-Shan, Joseph Tranquillo, Valentina Neplioueva, Nenad Bursac i Augustus O. Grant. "Sodium channel kinetic changes that produce Brugada syndrome or progressive cardiac conduction system disease". American Journal of Physiology-Heart and Circulatory Physiology 292, nr 1 (styczeń 2007): H399—H407. http://dx.doi.org/10.1152/ajpheart.01025.2005.

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Some mutations of the sodium channel gene NaV1.5 are multifunctional, causing combinations of LQTS, Brugada syndrome and progressive cardiac conduction system disease (PCCD). The combination of Brugada syndrome and PCCD is uncommon, although they both result from a reduction in the sodium current. We hypothesize that slow conduction is sufficient to cause S-T segment elevation and undertook a combined experimental and theoretical study to determine whether conduction slowing alone can produce the Brugada phenotype. Deletion of lysine 1479 in one of two positively charged clusters in the III/IV inter-domain linker causes both syndromes. We have examined the functional effects of this mutation using heterologous expression of the wild-type and mutant sodium channel in HEK-293-EBNA cells. We show that ΔK1479 shifts the potential of half-activation, V1/2m, to more positive potentials ( V1/2m = −36.8 ± 0.8 and −24.5 ± 1.3 mV for the wild-type and ΔK1479 mutant respectively, n = 11, 10). The depolarizing shift increases the extent of depolarization required for activation. The potential of half-inactivation, V1/2h, is also shifted to more positive potentials ( V1/2h = −85 ± 1.1 and −79.4 ± 1.2 mV for wild-type and ΔK1479 mutant respectively), increasing the fraction of channels available for activation. These shifts are quantitatively the same as a mutation that produces PCCD only, G514C. We incorporated experimentally derived parameters into a model of the cardiac action potential and its propagation in a one dimensional cable (simulating endo-, mid-myocardial and epicardial regions). The simulations show that action potential and ECG changes consistent with Brugada syndrome may result from conduction slowing alone; marked repolarization heterogeneity is not required. The findings also suggest how Brugada syndrome and PCCD which both result from loss of sodium channel function are sometimes present alone and at other times in combination.

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Palladino, Alberto, Andrea Antonio Papa, Roberta Petillo, Marianna Scutifero, Salvatore Morra, Luigia Passamano, Vincenzo Nigro i Luisa Politano. "The Role of TRPM4 Gene Mutations in Causing Familial Progressive Cardiac Conduction Disease: A Further Contribution". Genes 13, nr 2 (28.01.2022): 258. http://dx.doi.org/10.3390/genes13020258.

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Progressive cardiac conduction disease (PCCD) is a relatively common condition in young and elderly populations, related to rare mutations in several genes, including SCN5A, SCN1B, LMNA and GJA5, TRPM4. Familial cases have also been reported. We describe a family with a large number of individuals necessitating pacemaker implantation, likely due to varying degrees of PCCD. The proband is a 47-year-old-patient, whose younger brother died at 25 years of unexplained sudden cardiac death. Three paternal uncles needed a pacemaker (PM) implantation between 40 and 65 years for unspecified causes. At the age of 42, he was implanted with a PM for two episodes of syncope and the presence of complete atrioventricular block (AVB). NGS analysis revealed the missense variation c. 2351G>A, p.Gly844Asp in the exon 17 of the TRPM4 gene. This gene encodes the TRPM4 channel, a calcium-activated nonselective cation channel of the transient receptor potential melastatin (TRPM) ion channel family. Variations in TRPM4 have been shown to cause an increase in cell surface current density, which results in a gain of gene function. Our report broadens and supports the causative role of TRPM4 gene mutations in PCCD. Genetic screening and identification of the causal mutation are critical for risk stratification and family counselling.

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Knops, Simon P., Marcel P. J. M. van Riel, Richard H. M. Goossens, Esther M. M. van Lieshout, Peter Patka i Inger B. Schipper. "Measurements of the Exerted Pressure by Pelvic Circumferential Compression Devices". Open Orthopaedics Journal 4, nr 1 (17.02.2010): 101–6. http://dx.doi.org/10.2174/1874325001004010101.

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Background: Data on the efficacy and safety of non-invasive Pelvic Circumferential Compression Devices (PCCDs) is limited. Tissue damage may occur if a continuous pressure on the skin exceeding 9.3 kPa is sustained for more than two or three hours. The aim of this study was to gain insight into the pressure build-up at the interface, by measuring the PCCD-induced pressure when applying pulling forces to three different PCCDs (Pelvic Binder®, SAM-Sling® and T-POD®) in a simplified model. Methods: The resulting exerted pressures were measured at four ‘anatomical’ locations (right, left, posterior and anterior) in a model using a pressure measurement system consisting of pressure cuffs. Results: The exerted pressure varied substantially between the locations as well as between the PCCDs. Maximum pressures ranged from 18.9-23.3 kPa and from 19.2-27.5 kPa at the right location and left location, respectively. Pressures at the posterior location stayed below 18 kPa. At the anterior location pressures varied markedly between the different PCCDs. Conclusion: The circmferential compression by the different PCCDs showed high pressures measured at the four locations using a simplified model. Difference in design and functional characteristics of the PCCDs resulted in different pressure build-up at the four locations. When following the manufacturer’s instructions, the exerted pressure of all three PCCDs tested exceeded the tissue damaging level (9.3 kPa). In case of prolonged use in a clinical situation this might put patients at risk for developing tissue damage.

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Lin, Shiang-Yi, i Kevin Kien Hoa Chung. "Risk Perception, Perception of Collective Efficacy and Sleep Quality in Chinese Adults during COVID-19 Pandemic in Hong Kong: A Cross-Sectional Study". International Journal of Environmental Research and Public Health 18, nr 21 (3.11.2021): 11533. http://dx.doi.org/10.3390/ijerph182111533.

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Background: Only a few studies have studied the link between risk perception and sleep in the context of the COVID-19 pandemic. The purpose of our study is to propose and test a theoretical model to understand the relationships between COVID-19 risk appraisals—risk perception and perception of collective coordinated defense (PCCD) in particular—and subjective sleep quality in Chinese adults in Hong Kong during the COVID-19 pandemic. COVID-19-related fear and rumination were examined as potential mediators of the relationships. Methods: Data were collected using a self-report online questionnaire from a convenience sample of 224 Chinese adults during the fourth wave of the COVID-19 pandemic in Hong Kong. Results: Risk perception and PCCD were found to predict poor sleep quality. Mediation analysis showed that both fear and rumination mediated the relationship between risk perception and sleep quality, whereas only fear mediated the relationship between PCCD and sleep quality. The model was an excellent fit to the data and accounted for 44% of the variance in sleep quality in Chinese adults. This study indicated that both perception of high risks of contracting COVID-19 and anticipations of collective disease preventive efforts had adverse effects on subjective sleep quality via increasing COVID-19-related fear. Conclusions: These findings underscore the need for addressing sleep problems induced by psychological consequences of the pandemic. While policy makers often deliver public messaging campaigns that frame disease prevention as a collective goal, developing evidence-based coping strategies to combat COVID-19 adverse impacts on psychological health is equally important.

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Kopylov, U., M. Lipkin, B. Ungar, O. Haj, E. Shachar, A. Lahat, S. Ben-Horin i R. Eliakim. "P160 Pan-enteric mucosal inflammation in CD patients treated with vedolizumab – interim results of a prospective observational study using a panenteric capsule (PiilCam CD)". Journal of Crohn's and Colitis 16, Supplement_1 (1.01.2022): i235. http://dx.doi.org/10.1093/ecco-jcc/jjab232.288.

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Abstract Background Mucosal healing is associated with improved short- and long-term outcomes in Crohn’s disease (CD). However, the likelihood of reaching mucosal healing differs between segments of the mucosal tract. The prevalence of panenteric mucosal healing is scarcely described. Capsule endoscopy is the prime modality for evaluation of the entire small bowel. Recently, a combined pan-enteric capsule (PillCam Crohn’s capsule (PCCD) that enables evaluation of mucosal inflammation in the entire digestive tract in a single procedure, has been developed. The main aim of our study was to evaluate the rate of panenteric mucosal healing in Crohn’s disease patients treated with vedolizumab (VDZ) after 52 weeks of therapy. The current interim report focuses on the rates of mucosal improvement after 14 weeks of VDZ treatment. Methods This was a prospective open- label observational study. Patients starting vedolizumab (VDZ) for Crohn’s disease underwent a patency capsule (PC) and if it was excreted within 30 hours, enrolled in the study. The patients were evaluated with PCCD at week 0, 14 and 52 of VDZ treatment. Mucosal inflammation was quantified using the Lewis score (LS) and the panenteric Pillcam score (PS). In addition, fecal calprotectin (FC) and CRP levels were measured at each visit. Patients without active mucosal inflammation on week 0 PCCD were excluded from further participation Results We enrolled 30 patients; 5/30 failed PC and were dropped out of the study.Twenty five patients underwent PCCD at week 0 and 19/25 (78%)% -at week14 (six patients were dropped out for either absence of active mucosal inflammation at week 0 (1 patient), treatment discontinuation before week 14 (2 patients) endoscopic activity – 1, withdrawal of consent (1 patient) or loss to follow-up (1 patient)). After 14 weeks of VDZ treatment, there was a significant improvement in LS (median of 900 (interquartile ration (IQR)- 1143)), vs 450 (IQR-750), p=0.013), ES (15 (IQR-18) vs 10 (IQR-11), p=0.012) and FC (996 (IQR-455) vs 742 (IQR -740), p=0.012), while CRP levels were not significantly different. Conclusion VDZ induces significant improvement in mucosal inflammation evaluated by panenteric capsule endoscopy, in addition to a significant improvement in FC levels, after 14 weeks of therapy *The study was supported by IIR grant from Takeda and Medtronic

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Prasetyo, Eko, Raden Dimas Adityo, Nanik Suciati i Chastine Fatichah. "Partial Centroid Contour Distance (PCCD) in Mango Leaf Classification". International Journal on Advanced Science, Engineering and Information Technology 10, nr 5 (21.10.2020): 1920. http://dx.doi.org/10.18517/ijaseit.10.5.8047.

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Zhang, Jinglin, Jian Shang i Guoyu Zhang. "Verification for Different Contrail Parameterizations Based on Integrated Satellite Observation and ECMWF Reanalysis Data". Advances in Meteorology 2017 (2017): 1–11. http://dx.doi.org/10.1155/2017/8707234.

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Aviation induced cloud termed contrail plays a more and more important role in the climate change, which makes a significant contribution to anthropogenic climate forcing through impacting the coverage of cirrus in the intersection of troposphere and stratosphere. In this paper, we propose one novel automatic contrail detecting method based on Himawari-8 stationary satellite imagery and two kinds of potential contrail coverage (PCC1 and PCC2) from contrail parameterization in ECHAM4 and HadGEM2. In addition, we propose one new climatological index called contrail occurrence and persistence (COP). According to the algorithm identification (AI) and artificial visual inspection (AVI), COP measured from Himawari-8 stationary satellite imagery is related to upper tropospheric relative humidity over ice (RHI) computed with the ECMWF reanalysis data by simple linear regression. Similarly, we compared the linear correlation between COP and PCCs fractions and found that PCC1 has better correspondence with COP than PCC2.

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Montri, P., P. W. J. Taylor i O. Mongkolporn. "Pathotypes of Colletotrichum capsici, the Causal Agent of Chili Anthracnose, in Thailand". Plant Disease 93, nr 1 (styczeń 2009): 17–20. http://dx.doi.org/10.1094/pdis-93-1-0017.

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Eleven isolates of Colletotrichum capsici were screened on nine chili genotypes derived from four cultivated species of Capsicum: Capsicum annuum, C. baccatum, C. chinense, and C. frutescens. Host reactions were assessed 9 days after inoculation by microinjection of spores into the pericarp of red fruit. A set of disease scales, with 0 to 9 scores, were developed for anthracnose infection of each Capsicum sp. based on percent lesion size in relation to fruit size, appearance of necrotic or water-soaked tissue, and presence of acervuli. Three pathotypes, PCc1, PCc2, and PCc3, were identified according to differential qualitative infection of fruit of C. chinense genotypes PBC932 and C04714. PCc1 was the most virulent pathotype, infecting all genotypes of C. annuum, C. chinense, and C. frutescens, whereas PCc3 was the least virulent pathotype, infecting only the genotypes C. annuum and C. frutescens. Quantitative infection occurred in all chili genotypes except for genotypes of C. baccatum, where no infection occurred, demonstrating various levels of aggressiveness of isolates within pathotypes.

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Nowak, Artur, Örs Legeza i Katharina Boguslawski. "Orbital entanglement and correlation from pCCD-tailored coupled cluster wave functions". Journal of Chemical Physics 154, nr 8 (28.02.2021): 084111. http://dx.doi.org/10.1063/5.0038205.

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Lahl, Uwe. "Sintering plants of steel industry - PCCD/F emission status and perspectives". Chemosphere 29, nr 9-11 (listopad 1994): 1939–45. http://dx.doi.org/10.1016/0045-6535(94)90359-x.

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Mezgec, Zdenko, Amor Chowdhury, Bojan Kotnik i Rajko Svečko. "Implementation of PCCD-OFDM-ASK Robust Data Transmission over GSM Speech Channel". Informatica 20, nr 1 (1.01.2009): 51–78. http://dx.doi.org/10.15388/informatica.2009.237.

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Totaro, Grazia, Paola Marchese, Maria Barbara Banella, Laura Sisti, Annamaria Celli, Vincent Verney i Sophie Commereuc. "Ageing of PCCD aliphatic polyesters: Effect of stereochemistry and ionic chain terminals". Journal of Photochemistry and Photobiology A: Chemistry 292 (październik 2014): 42–48. http://dx.doi.org/10.1016/j.jphotochem.2014.06.019.

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Alonso, Daniela, i Silvina Brussino. "Political Culture and the Crisis of Democracy. An Analysis of Citizenship Motivations to Justify a Coup D’état in Argentina". Universitas Psychologica 20 (15.12.2021): 1–16. http://dx.doi.org/10.11144/javeriana.upsy20.pccd.

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Latin American democracies are experiencing high instability, signed by economic and political crises and increasing social conflict. In this scenario, the continuity and further development of democracies are far from being granted. From a political psychology perspective, we are interested in understanding public motivations for justifying an authoritarian interruption of the democratic order. On a 454 Cordoba citizens sample (mean age= 37.3, SD=14.22; 52.7% women, 47.3% men), we conducted a Discriminant Analysis to identify the more relevant dimensions for classifying people according to their attitudes towards a coup d’état. Six variables constituted the linear discriminant function resulting from a stepwise procedure (λ = 0.67, X2 = 151.5, gl = 6, p = 0) and enabled to classify 77.3% of the cases correctly. Conservative, normative, and ideological orientations were the more relevant dimensions to predict the justification of a state coup. Additionally, we identified two different attitudinal profiles among the people who are willing to justify a coup, mainly differentiated by their ideological orientations through a two-step cluster analysis. We accounted for a small group of people who justify a non-democratic exit to the socio-political conflict based on progressive ideological attitudes more than conservative or authoritarian orientations. Theoretical and practical implications of these findings are discussed.

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Brunelle, Daniel J., i Taeseok Jang. "Optimization of poly(1,4-cyclohexylidene cyclohexane-1,4-dicarboxylate) (PCCD) preparation for increased crystallinity". Polymer 47, nr 11 (maj 2006): 4094–104. http://dx.doi.org/10.1016/j.polymer.2006.02.070.

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Makita, Naomasa, Takeru Makiyama, Akihiko Nogami, Hiroshi Watanabe, Akiko Seki, Naokata Sumitomo, Wataru Shimizu i Minoru Horie. "Clinical Features and Genetic Basis of Progressive Cardiac Conduction Defect: Japanese PCCD Registry". Journal of Arrhythmia 27, Supplement (2011): JJJS_1. http://dx.doi.org/10.4020/jhrs.27.jjjs_1.

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Seraphin, Hugues, Anca C. Yallop, Alexandru Capatîna i Vanessa GB Gowreesunkar. "Heritage in tourism organisations’ branding strategy: the case of a post-colonial, post-conflict and post-disaster destination". International Journal of Culture, Tourism and Hospitality Research 12, nr 1 (5.03.2018): 89–105. http://dx.doi.org/10.1108/ijcthr-05-2017-0057.

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Purpose The purpose of this study is to investigate the link between history (heritage) and tourism marketing (destination branding). More specifically, the paper focusses on how heritage is used by private- and public-sector tourism organisations of post-colonial, post-conflict and post-disaster (PCCD) destinations in their branding strategy. In particular, the paper investigates how these organisations use heritage in their branding and logo design. Design/methodology/approach Within the paradigm of theory building and exploratory approach, this conceptual study is based on a narrative literature review and analysis of research and secondary data on Haiti. The study uses visual research methods to examine and reveal the basis and composition of logos of both private- and public-sector organisations in Haiti. Findings The findings of this paper suggest that capturing the essence of the destination is critical for any visual identification (i.e. logos), and that the visual identification can either adjust representation of past events to the time being (heritage) or move away from the past with clear expectations for the future. Such findings are reflected within the new marketing strategies adopted by the Haitian destination marketing organisation (DMO) and a private resort that we used as examples. Both moved from an idiosyncratic identity-based logo to a universal “sea-and-sun” stereotyped one that goes against heritage for which authenticity is the most important criterion. Practical implications The findings of this research may help destination managers in general, and DMOs in PCCD destinations in particular, to design logos aligned with their marketing and branding strategies. The findings of the paper may also assist industry experts in designing logos that communicate with potential tourists, by leveraging heritage to influence their emotion and decision making. Originality/value This paper represents one of the first papers in tourism research that examines branding strategies of both public and private sectors in the context of Haiti. The research contributes to the body of knowledge on heritage and destination marketing by exploring the role of heritage in the Caribbean area’s branding and marketing strategies.

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Storring, N., C. Hammerton, C. Lawn, R. Fernandez i H. Rabe. "G274(P) Simulation Training - Transition from Straight to Pigtail Catheter Chest Drain (PCCD) Insertion". Archives of Disease in Childhood 99, Suppl 1 (1.04.2014): A118. http://dx.doi.org/10.1136/archdischild-2014-306237.271.

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Berti, Corrado, Maurizio Fiorini, Paola Marchese i Laura Sisti. "Synthesis and characterisation of novel PBT and PCCD telechelic ionomers prepared by end capping". European Polymer Journal 43, nr 9 (wrzesień 2007): 3943–52. http://dx.doi.org/10.1016/j.eurpolymj.2007.06.034.

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Галушкин, А. С., Е. Ю. Пыркова, М. В. Куркина, С. В. Михайлова, В. П. Воронцова, Д. И. Грибов, М. В. Заживихина i in. "Biochemical and genetic characterization of 16 patients with propionic acidemia". Nauchno-prakticheskii zhurnal «Medicinskaia genetika, nr 7 (31.07.2021): 3–14. http://dx.doi.org/10.25557/2073-7998.2021.07.3-14.

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Пропионовая ацидемия (ПА) - редкое наследственное заболевание с аутосомно-рецессивным типом наследования, относится к «классическим», т.е. наиболее часто диагностируемым органическим ацидемиям. В данной работе представлена биохимическая и молекулярно-генетическая характеристика 16 пациентов с ПА, выявленных в Российской Федерации. У большинства обследованных пациентов первые симптомы проявились в первые месяцы жизни, наиболее частые из них: нарушения вскармливания, судороги, мышечная гипотония, угнетение сознания. При биохимическом исследовании (ГХ-МС и МС/МС) у пациентов было выявлено повышение концентрации 3-гидроксипропионой кислоты, метилцитрата, пропионилглицина, C3, глицина, которые являются патогномоничными маркерами этой патологии. В результате молекулярно-генетического анализа в генах PCCA и PCCB было выявлено 6 неописанных ранее вариантов нуклеотидной последовательности (4 варианта в гене PCCA, 2 - в гене PCCB), один из которых (PCCB: c.655-2A>G) имеет высокую частоту (17,2%, 5/29 мутантных аллелей генов PCCA и PCCB) в исследуемой выборке. Локализация миссенс-вариантов и их влияние на структуру белка были продемонстрированы на 3D-модели фермента пропионил-КоА-карбоксилазы. Выявленным вариантам были присвоены критерии патогенности в соответствии с рекомендациями American College of Medical Genetics and Genomics (ACMG). Propionic acidemia is a rare autosomal recessive metabolic disorder, characterized as classic organic acidemia. The article represents biochemical and molecular characterization of 16 patients diagnosed with propionic acidemia in Russia. Symptoms appeared during the first months after birth in most cases. Poor feeding, seizures, hypotonia, lethargy were the most frequent symptoms. Biochemical tests (GC-MS and MS/MS) showed elevated 3-hydroxypropionic acid, methylcitrate, propionylglycine, C3 in patients blood. Six undescribed earlier variants were found as a result of PCCA & PCCB (4 variants - PCCA, 2 - PCCB) genes DNA-tests. Variant c.655-2A>G in PCCB is the most frequent in this group (17.2%). Localization of missense variants and their effect on protein was demonstrated using propionyl-CoA carboxylase 3D model. New variants were classified according to the American College of Medical Genetics and Genomics (ACMG) guidelines.

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Loyola, Cristina Douat, Rosane Mara Pontes de Oliveira i Virginia Faria Damásio Dutra. "Caring for the future: decrease in infant mortality in Maranhão State". Revista Brasileira de Enfermagem 71, suppl 3 (2018): 1479–84. http://dx.doi.org/10.1590/0034-7167-2017-0245.

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ABSTRACT Objective: To report on the experience and care provided by the “Caring for the future” program to decrease infant mortality in 17 municipalities of Maranhão, in a proposal work of the Secretariat of Basic Care, in partnership with the Non-Governmental Organization called Centro Popular de Cultura e Desenvolvimento (Popular Center for Culture and Development/PCCD) from 2009 to 2015. Method: Experience Report of community care that made it possible to create and strengthen solidary relationships among community members. Results: The program decreased infant mortality rate and created in the supported communities a platform of health support, uniting information and care in Primary Care. The program trained 34 “Guardian Angels” and 170 “Supportive Caregivers”, mapped seven thousand “luminous spots” and trained more than 7,000 caregivers in health. It served 17 municipalities, 27,191 pregnant women, 291,266 families. Final considerations: The program was a catalyst for actions: it unveiled hidden resources in the community, awakened collective responsibility, offered training and built solidary relationships.

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Song, Shunxi, Jiantao Liang, Lin Li, Meiyun Zhang, Jingyi Nie i Xiaoli Zhen. "Selection of filler particle size for maximizing the critical properties of cellulosic paper by filler pre-flocculation". Nordic Pulp & Paper Research Journal 33, nr 4 (19.12.2018): 603–9. http://dx.doi.org/10.1515/npprj-2018-0044.

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AbstractFiller pre-flocculation has been extensively studied in the past to increase filler content while maintaining the physical strength properties of paper. However, optical properties of paper could be negatively affected due to the filler pre-flocculation. In this work, two kinds of precipitated calcium carbonate (PCC) fillers with different particle sizes, namely PCC1 (4.2 μm) and PCC2 (7.7 μm), were used to investigate the effects of filler pre-flocculation on the strength and optical properties of paper. Results showed that pre-flocculated fillers with smaller particle size (PCC1) could improve the tensile strength of handsheets more effectively, since the tensile index was increased by 22.9 %. Meanwhile, for a given floc size, the handsheets with PCC1 flocs exhibited a better light scattering ability comparing to the ones filled with PCC2 flocs. The improved light scattering ability of handsheets filled with 24 μm PCC1 flocs compared to 24 μm flocs produced with PCC2 can be attributed to the complex micro void structure of flocs. This finding can be used as a guidance for the selection of filler particle size when using filler pre-flocculation to optimize the mechanical and optical properties of paper.

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Moraleda Barreno, Enrique, Modesto Jesús Romero López i María José Cayetano Menéndez. "La parálisis cerebral como una condición dinámica del cerebro: un estudio secuencial del desarrollo de niños hasta los 6 años de edad". Universitas Psychologica 12, nr 1 (20.06.2012): 119–27. http://dx.doi.org/10.11144/javeriana.upsy12-1.pccd.

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La parálisis cerebral es un trastorno motor debido a inmadurez cerebral con numerosos déficits asociados, incluidos los adaptativos, sociales, motores, cognitivos y de la comunicación, y con gran impacto en el desarrollo. El objetivo del presente estudio fue estudiar el desarrollo durante un año de niños con parálisis cerebral de uno a seis años. El método empleado fue un diseño ex post facto evolutivo secuencial. Se utilizó la prueba de screening del Inventario de Desarrollo de Battelle en un pretest y en un postest transcurrido un año. La muestra estuvo compuesta por 41 niños de entre 1 y 6 años de edad con diagnóstico de parálisis cerebral infantil. Los niños mostraban puntuaciones en el postest significativamente superiores en todas las áreas estudiadas. Se observó una mejora generalizada en las capacidades de la mayoría de los sujetos. Se discuten las posibles influencias que contribuyen a estas mejoras en los cocientes de desarrollo en la obtención de estos resultados.

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Bernard, Alfred. "Dermal Exposure to Hazardous Chemicals in Baby Diapers: A Re-Evaluation of the Quantitative Health Risk Assessment Conducted by The French Agency for Food, Environmental and Occupational Health and Safety (ANSES)". International Journal of Environmental Research and Public Health 19, nr 7 (31.03.2022): 4159. http://dx.doi.org/10.3390/ijerph19074159.

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In January 2019, the French Agency for Food, Environmental and Occupational Health and Safety (ANSES) published an opinion on risks related to the presence of hazardous chemicals in infant diapers. ANSES found that health reference values were largely exceeded for polycyclic aromatic hydrocarbons (PAHs), dioxins (PCCD/Fs) and dioxin-like polychlorobiphenyls (DL-PCBs). The levels of formaldehyde and some fragrances were also considered potentially unsafe. Therefore, ANSES concluded that actions have to be taken to restrict levels of these contaminants in diapers. Under the exposure scenario deemed the most reliable by ANSES, estimates of cancer risks of the most potent PAHs detected in diapers exceeded 10−3 and hazard quotients for neurobehavioral effects attained values up to 66. Regarding dioxins and DL-PCBs, ANSES derived a hazard quotient of 12 for the risk of decreased sperm count at adult age. The aim of this study was to examine whether the exposure and risk assessment conducted by ANSES contained potential flaws that could explain such a high exceedance of health reference values. This study also put into perspective the exposure from diapers with that from breast milk whose benefits for children’s health are undisputable despite contamination by PAHs, dioxins and DL-PCBS.

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Choi, Se-Jin, Sung-Ho Bae, Dong-Min Ji i Sung-Hoon Kim. "Effects of Capsule Type on the Characteristics of Cement Mortars Containing Powder Compacted Capsules". Materials 15, nr 19 (29.09.2022): 6773. http://dx.doi.org/10.3390/ma15196773.

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Several studies have been reported on self-healing concrete using bacteria, admixtures, and microcapsules. Among these self-healing techniques, encapsulating cement-based materials is advantageous in that large amounts of self-healing material can be contained in a capsule and released at the cracked site for a targeted reaction. This study produced a powder compacted capsule (PCC) using the droplet and blended manufacturing methods to encapsulate cementitious materials. This study refers to the PCCs as droplet-PCC (D-PCC) and blended-PCC (B-PCC) according to the manufacturing method used. The fluidity, compressive strength, carbonation, drying shrinkage, and water permeability of cement mortar with PCCs were evaluated. The test results show that the flow of the mortar sample using D-PCC was slightly higher than that of the mortar using B-PCC. The compressive strength of the mortar sample with B-PCC was generally higher than that of the mortar sample with D-PCC. The compressive strength of the B-PCC2 sample (with 0.2% of B-PCC) was the highest at all curing ages. This may be because the B-PCC fracture load was higher than that of the D-PCC. In addition, more hydrates were observed in the B-PCC sample than in the D-PCC sample. A crack healing effect was observed in the samples with PCC, regardless of the PCC type. The effect was the greatest in the B-PCC6 sample (with 0.6% of B-PCC). The results of this study provide a reference for the PCC type and mix ratio that would yield the best mechanical properties and crack healing effect.

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Inoue, Yukiko. "Methodological Issues in the Evaluation of Intelligent Tutoring Systems". Journal of Educational Technology Systems 29, nr 3 (marzec 2001): 251–58. http://dx.doi.org/10.2190/b5vf-qk3f-pccd-64lb.

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Drill and practice, simulation, and tutorial are the main formats of computer-assisted instruction, yet tutorial format is particularly suitable to conceptual school subjects. When a knowledge module is added to the tutorial format, it is termed an intelligent tutoring system (ITS). Although ITS evaluation studies greatly influence interest in and support for the future ITS work, valid evaluation methodologies are lacking because ITS is fairly new. Consequently, this article examined the ITS evaluation studies, with the focus on the methodological issue. The critical component in ITS is the knowledge component that has not been evaluated adequately to verify the knowledge. Definitely summative evaluation is more difficult than formative evaluation since it involves the comparison of ITS with human tutors using traditional teaching methods across extensive subject domains.

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Hou, Jing, Hua Xiang i Jing Han. "Propionyl Coenzyme A (Propionyl-CoA) Carboxylase in Haloferax mediterranei: Indispensability for Propionyl-CoA Assimilation and Impacts on Global Metabolism". Applied and Environmental Microbiology 81, nr 2 (14.11.2014): 794–804. http://dx.doi.org/10.1128/aem.03167-14.

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ABSTRACTPropionyl coenzyme A (propionyl-CoA) is an important intermediate during the biosynthesis and catabolism of intracellular carbon storage of poly(3-hydroxybutyrate-co-3-hydroxyvalerate) (PHBV) in haloarchaea. However, the haloarchaeal propionyl-CoA carboxylase (PCC) and its physiological significance remain unclear. In this study, we identified a PCC that catalyzed propionyl-CoA carboxylation with an acetyl-CoA carboxylation side activity inHaloferax mediterranei. Gene knockout/complementation demonstrated that the PCC enzyme consisted of a fusion protein of a biotin carboxylase and a biotin-carboxyl carrier protein (PccA [HFX_2490]), a carboxyltransferase component (PccB [HFX_2478]), and an essential small subunit (PccX [HFX_2479]). Knockout ofpccBXled to an inability to utilize propionate and a higher intracellular propionyl-CoA level, indicating that the PCC enzyme is indispensable for propionyl-CoA utilization. Interestingly,H. mediterraneiDBX (pccBX-deleted strain) displayed multiple phenotypic changes, including retarded cell growth, decreased glucose consumption, impaired PHBV biosynthesis, and wrinkled cells. A propionyl-CoA concentration equivalent to the concentration that accumulated in DBX cells was demonstrated to inhibit succinyl-CoA synthetase of the tricarboxylic acid cyclein vitro. Genome-wide microarray analysis showed that many genes for glycolysis, pyruvate oxidation, PHBV accumulation, electron transport, and stress responses were affected in DBX. This study not only identified the haloarchaeal PCC for the metabolism of propionyl-CoA, an important intermediate in haloarchaea, but also demonstrated that impaired propionyl-CoA metabolism affected global metabolism inH. mediterranei.

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Villarreal-Molina, Teresa, Gabriela Paola García-Ordóñez, Álvaro E. Reyes-Quintero, Mayra Domínguez-Pérez, Leonor Jacobo-Albavera, Santiago Nava, Alessandra Carnevale, Argelia Medeiros-Domingo i Pedro Iturralde. "Clinical Spectrum of SCN5A Channelopathy in Children with Primary Electrical Disease and Structurally Normal Hearts". Genes 13, nr 1 (22.12.2021): 16. http://dx.doi.org/10.3390/genes13010016.

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Sodium voltage-gated channel α subunit 5 (SCN5A)-mutations may cause an array of arrhythmogenic syndromes most frequently as an autosomal dominant trait, with incomplete penetrance, variable expressivity and male predominance. In the present study, we retrospectively describe a group of Mexican patients with SCN5A-disease causing variants in whom the onset of symptoms occurred in the pediatric age range. The study included 17 patients with clinical diagnosis of primary electrical disease, at least one SCN5A pathogenic or likely pathogenic mutation and age of onset <18 years, and all available first- and second-degree relatives. Fifteen patients (88.2%) were male, and sixteen independent variants were found (twelve missense, three truncating and one complex inframe deletion/insertion). The frequency of compound heterozygosity was remarkably high (3/17, 17.6%), with early childhood onset and severe disease. Overall, 70.6% of pediatric patients presented with overlap syndrome, 11.8% with isolated sick sinus syndrome, 11.8% with isolated Brugada syndrome (BrS) and 5.9% with isolated type 3 long QT syndrome (LQTS). A total of 24/45 SCN5A mutation carriers were affected (overall penetrance 53.3%), and penetrance was higher in males (63.3%, 19 affected/30 mutation carriers) than in females (33.3%, 5 affected/15 carriers). In conclusion, pediatric patients with SCNA-disease causing variants presented mainly as overlap syndrome, with predominant loss-of-function phenotypes of sick sinus syndrome (SSS), progressive cardiac conduction disease (PCCD) and ventricular arrhythmias.

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Castillejo Becerra, Clara, Adam Guenzel i Michael Barry. "Immunodeficiency in a hypomorphic mouse model of propianic acidemia (HUM4P.300)". Journal of Immunology 194, nr 1_Supplement (1.05.2015): 122.7. http://dx.doi.org/10.4049/jimmunol.194.supp.122.7.

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Abstract Propionic Acidemia (PA) is a recessive disorder resulting from mutations in the PCCA or PCCB genes encoding the two subunits of propionyl-CoA carboxylase. Patients with PA generate propionate and potentially toxic metabolites after consumption of certain amino acids and odd-chain fatty acids leading to systemic health complications. In addition, PA patients are exceptionally sensitive to infections due to a compromised immune system. We developed a hypomorph mouse model of PA (Pcca-/-(A138T) that recapitulates many phenotypes associated with the human disease. In this study, we tested A138T mouse immune function and their susceptibility to infections. A138T mice had an overall reduction in white blood cells, lymphocytes, monocytes, granulocytes, and immunoglobulins when compared to control mice. When wild type and A138T mice were infected i.v. with adenovirus 5 (Ad5) expressing GFP-Luciferase, we observed higher luciferase expression in the livers of the Pcca-/-(A138T) suggesting that the mutant mice were more susceptible to the virus due to defects in the reticuloendothelial system. The A138T mice also generated lower levels of anti-GFP antibodies than control mice 7 weeks after injection suggesting weaker adaptive immune responses. Overall, our results show that the Pcca-/-(A138T) mouse model recapitulates the immunodefiency seen in humans and can be used to further explore their susceptibility to infections as well as the benefits or dangers of vaccination to PA patients.

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Alonso-Barroso, Esmeralda, Belén Pérez, Lourdes Ruiz Desviat i Eva Richard. "Cardiomyocytes Derived from Induced Pluripotent Stem Cells as a Disease Model for Propionic Acidemia". International Journal of Molecular Sciences 22, nr 3 (25.01.2021): 1161. http://dx.doi.org/10.3390/ijms22031161.

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Propionic acidemia (PA), one of the most frequent life-threatening organic acidemias, is caused by mutations in either the PCCA or PCCB genes encoding both subunits of the mitochondrial propionyl-CoA carboxylase (PCC) enzyme. Cardiac alterations (hypertrophy, dilated cardiomyopathy, long QT) are one of the major causes of mortality in patients surviving the neonatal period. To overcome limitations of current cellular models of PA, we generated induced pluripotent stem cells (iPSCs) from a PA patient with defects in the PCCA gene, and successfully differentiated them into cardiomyocytes. PCCA iPSC-derived cardiomyocytes exhibited reduced oxygen consumption, an accumulation of residual bodies and lipid droplets, and increased ribosomal biogenesis. Furthermore, we found increased protein levels of HERP, GRP78, GRP75, SIG-1R and MFN2, suggesting endoplasmic reticulum stress and calcium perturbations in these cells. We also analyzed a series of heart-enriched miRNAs previously found deregulated in the heart tissue of a PA murine model and confirmed their altered expression. Our novel results show that PA iPSC-cardiomyocytes represent a promising model for investigating the pathological mechanisms underlying PA cardiomyopathies, also serving as an ex vivo platform for therapeutic evaluation.

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Matyushkina, Yulia I., i Alexandr A. Shabarin. "EXTRACTION OF ANIONIC CADMIUM COMPLEXES WITH ORGANIC SOLUTIONS OF QUATERNARY AMMONIUM SALTS". IZVESTIYA VYSSHIKH UCHEBNYKH ZAVEDENII KHIMIYA KHIMICHESKAYA TEKHNOLOGIYA 63, nr 10 (8.09.2020): 30–35. http://dx.doi.org/10.6060/ivkkt.20206310.6224.

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The anion-exchange extraction of thiocyanate, chloride and iodide cadmium complexes by solutions of quaternary ammonium salts chlorides in organic solvents (toluene, carbon tetrachloride, ethyl acetate, isobutyl alcohol, nitrobenzene) was studied. Extraction involves solutions of alkyl dimethylbenzylammonium chlorides (R-N+(CH3)2-CH2C6H5-Cl-) and alkyl dimethylethyl-benzylammonium (R-N+(CH3)2-CH2-CH2C6H5-Cl-), where R is a straight alkyl chain, mainly C12 - C14. The composition of the cadmium anionic complexes was established by the analysis of the calibration curves E = f (pCCd (II)) constructed from cadmium sulfate solutions against the background of various contents of thiocyanate, chloride and iodide ions (ndicator electrode - ion-selective electrode with a membrane, which based on a nitrobenzene solution of tetradecylammonium bromide). The extraction process is estimated quantitatively using a distribution coefficient (D). The value of D is calculated taking into account the cadmium concentration in the aqueous phase before and after extraction. The dependence of the distribution coefficient on the organic solvent dielectric constant, the concentration and stability of the anionic complexes of cadmium is shown. So, for the indicated cadmium acidocomplexes, the minimum D values were obtained using low-polar toluene and carbon tetrachloride, and the maximum values were obtained using highly polar isobutyl alcohol and nitrobenzene. If the concentration of cadmium (II) is reduced by a factor of 100 for the cadmium rhodanide and iodide complexes, the value of D decreases by 1.6-1.9 times, for the chloride complex, by 1.2 times in the case of polar isobutyl alcohol and nitrobenzene, and 2.9-3.5 times in the case of low-polar solvents. It was experimentally established that in the series [Cd(SCN)4]2- - [CdI4]2-- [CdCl4]2- the value of D decreases for all the studied systems. The observed regularity is related both to the stability of the corresponding cadmium (II) complexes in aqueous solutions and to their hydrophobicity.

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Bychkov, Igor, Artur Galushkin, Alexandra Filatova, Andrey Nekrasov, Marina Kurkina, Galina Baydakova, Alexandra Ilyushkina, Mikhail Skoblov i Ekaterina Zakharova. "Functional Analysis of the PCCA and PCCB Gene Variants Predicted to Affect Splicing". International Journal of Molecular Sciences 22, nr 8 (16.04.2021): 4154. http://dx.doi.org/10.3390/ijms22084154.

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It is estimated that up to one-third of all variants causing inherited diseases affect splicing; however, their deleterious effects and roles in disease pathogenesis are often not fully characterized. Given their prevalence and the development of various antisense-based splice-modulating approaches, pathogenic splicing variants have become an important object of genomic medicine. To improve the accuracy of variant interpretation in public mutation repositories, we applied the minigene splicing assay to study the effects of 24 variants that were predicted to affect normal splicing in the genes associated with propionic acidemia (PA)—PCCA and PCCB. As a result, 13 variants (including one missense and two synonymous variants) demonstrated a significant alteration of splicing with the predicted deleterious effect at the protein level and were characterized as spliceogenic loss-of-function variants. The analysis of the available data for the studied variants and application of the American College of Medical Genetics and the Association for Molecular Pathology (ACMG/AMP) guidelines allowed us to precisely classify five of the variants and change the pathogenic status of nine. Using the example of the PA genes, we demonstrated the utility of the minigene splicing assay in the fast and effective assessment of the spliceogenic effect for identified variants and highlight the necessity of their standardized classification.

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Прилуцкая, В. А., А. В. Сукало, Т. А. Деркач, И. В. Наумчик, Л. В. Шалькевич, А. В. Зиновик, Б. Л. Елиневский, В. П. Стефаненкова i И. П. Богданович. "Propionic Acidemia in Children: Literature Review and Clinical Observation". Педиатрия. Восточная Европа, nr 1 (11.05.2020): 92–105. http://dx.doi.org/10.34883/pi.2020.8.1.008.

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Пропионовая ацидемия редкое наследственное заболевание обмена веществ с аутосомно-рецессивным типом наследования, которое связано с нарушением метаболизма органических кислот. Заболевание обусловлено недостаточностью фермента пропионил-КоА карбоксилазы, которая приводит к накоплению в органах и тканях пропионовой кислоты и ее метаболитов. Клинически выделяют 2 основные формы болезни: острую неонатальную и младенческую (или позднюю) формы. Диагностика заболевания является сложной задачей, так как клинические симптомы, обусловленные дефектами метаболизма, часто встречаются при внутриутробных инфекциях, гипоксически-ишемическом поражении центральной нервной системы, пороках развития головного мозга. Лабораторная диагностика основана на увеличении содержания пропионовой кислоты, метаболитов, изолейцина, валина, метионина и треонина в крови. Для точной верификации необходимо молекулярно-генетическое исследование с поиском мутаций в генах PCCA и PCCВ. Подтверждение диагноза важно не только для тактики ведения пациентов, но и при планировании последующих деторождений в таких семьях. В статье представлено наблюдение пропионовой ацидемии у ребенка в возрасте 3 месяцев жизни. Данный случай продемонстрирован с целью привлечения внимания педиатров к проблеме наследственных болезней обмена веществ. Несмотря на объективные сложности в диагностике пропионовой ацидемии, такие опорные симптомы, как раннее начало заболевания с наличием характерных кризов, обусловленных токсической энцефалопатией, лабораторные изменения, регистрирующие метаболический ацидоз, специфические изменения в крови и моче при генетическом исследовании, помогают не только заподозрить и диагностировать заболевание, но и своевременно назначить специфическое лечение и сохранить жизнь ребенку. Propionic acidemia is a rare hereditary metabolic disease with autosomal recessive type of inheritance, which is associated with a violation of the metabolism of organic acids. The disease is caused by the deficiency of the propionyl CoA carboxylase enzyme, which leads to the accumulation of propionic acid and its metabolites in organs and tissues. Two main forms of the disease are clinically distinguished: acute neonatal one and infant (or late) one. Diagnostics of the disease is a difficult task, because clinical symptoms caused by metabolic defects are often found in intrauterine infections, hypoxic-ischemic damage to the central nervous system, and brain malformations. Laboratory diagnostics is based on the increase of the content of propionic acid, metabolites, isoleucine, valine, methionine, and threonine in the blood. For accurate verification, a molecular genetic study with the search of mutations in the PCCA and PCCB genes is required. Confirmation of the diagnosis is important not only for the management of patients, but also when planning subsequent births in such families. The article presents the observation of propionic acidemia in a child at the age of 3 months. This case was demonstrated in order to attract the attention of pediatricians to the problem of hereditary metabolic diseases. Despite objective difficulties in the diagnostics of propionic acidemia, such supporting symptoms as early onset of the disease with the presence of specific crises caused by toxic encephalopathy, laboratory changes that record metabolic acidosis, specific changes in blood and urine during the genetic study help to not only suspect and diagnose the disease, but also timely prescribe specific treatment and save the life of the child.

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Saldaña García, Natalia, Mº del Carmen López Castillo, Adrián Muñoz-Cruzado Rosete i Tomás Sánchez Tamayo. "Acidemia propiónica en prematuro con infección asociada. Informe de caso". Acta Pediátrica de México 42, nr 3 (11.05.2021): 112. http://dx.doi.org/10.18233/apm42no3pp112-1202142.

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ANTECEDENTES: Las acidemias orgánicas suponen el 30% de los errores innatos del metabolismo y pueden ser mortales en el periodo neonatal.CASO CLÍNICO: Recién nacida, por cesárea urgente por placenta previa con hemorragia materna sin factores de riesgo infeccioso a las 32 semanas de gestación, de 1970 gramos. Se diagnosticó infección nosocomial y ante la falta de respuesta al antibiótico se solicitó un hemograma que reportó, como dato sobresaliente: hiperamoniemia de 1920 mM/L. Se dejó en ayuno y se inciaron aportes elevados intravenosos de glucosa, soporte ventilatorio invasivo, tratamiento con quelantes de amonio y depuración extrarrenal. Pese al tratamiento, falleció a las 13 horas del diagnóstico de hiperamoniemia en el cuarto día de vida. El estudio bioquímico reportó: elevación de ácido 3 hidroxipropiónico, ácido metilcítrico, propionilglicina en orina y propionil carnitina y descenso de carnitina libre en sangre, con lo que se confirmó el diagnóstico de acidemia propiónica. En el análisis genético se detectó una mutación patogénica en homocigosis en el gen PCCB. La autopsia reveló: infección pulmonar por Enterobacter cloacae y la metabolopatía como causa de la muerte.CONCLUSIONES: La acidemia propiónica es consecuencia de un déficit de la propionil- CoA carboxilasa que interviene en la vía metabólica del propionato. Es una enfermedad autosómica recesiva, por mutación en los genes PCCA y/o PCCB. La presentación más frecuente es neonatal, con síntomas inespecíficos en la primera semana. Así, ante un neonato con síntomas inespecíficos, con respuesta inadecuada al tratamiento, a pesar de encontrar una causa que justifique el cuadro, debe sospecharse una enfermedad metabólica.

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Yang, Xue, Osamu Sakamoto, Yoichi Matsubara, Shigeo Kure, Yoichi Suzuki, Yoko Aoki, Seiji Yamaguchi i in. "Mutation spectrum of the PCCA and PCCB genes in Japanese patients with propionic acidemia". Molecular Genetics and Metabolism 81, nr 4 (kwiecień 2004): 335–42. http://dx.doi.org/10.1016/j.ymgme.2004.01.003.

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Pazmino, Ana Veronica. "Design Social: desenvolvimento de material didático para Educação Básica". Extensio: Revista Eletrônica de Extensão 16, nr 33 (28.08.2019): 2–23. http://dx.doi.org/10.5007/1807-0221.2019v16n33p2.

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O trabalho mostra o resultado do projeto de extensão UFSC – 2018 “Design e Educação: objetos e materiais didáticos voltados para a educação básica”, em que houve a articulação da ação de extensão e o ensino com as disciplinas: metodologia de projeto EGR7112; Seminários de design social EGR7118; EGR7196; EGR7197 de PCC1 e PCC2 Projeto de conclusão de curso nos semestres de 2018/1 e 2018/2. O objetivo da ação de extensão foi: propor soluções por meio do design para contribuir com a ação educativa na Rede Municipal de Ensino de Florianópolis. Na busca de possibilitar a ação social os objetivos específicos foram: levantar informações sobre as necessidades de professores em creches e ou pré-escolas; desenvolver soluções para problemas identificados pelos professores e na escola durante a pesquisa; desenvolver materiais e entregar nas escolas. A metodologia adotada para o desenvolvimento do projeto foi o Design Thinking. Em um ano o projeto teve 20 materiais didáticos (jogos e livros) para crianças, adolescentes e professores de 16 escolas da rede Municipal de Florianópolis.

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Rodriguez-Pombo, P., C. Pérez-Cerdá, L. R. Desviat, B. Pérez, M. Ugarte i P. Rodríguez-Pombo. "Transfection Screening for Defects in the PCCA and PCCB Genes Encoding Propionyl-CoA Carboxylase Subunits". Molecular Genetics and Metabolism 75, nr 3 (marzec 2002): 276–79. http://dx.doi.org/10.1006/mgme.2001.3296.

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Dai, Jialin, Yanye Ruan, Ying Feng i Bin Li. "Physical Properties, α-Glucosidase Inhibitory Activity, and Digestive Stability of Four Purple Corn Cob Anthocyanin Complexes". Foods 11, nr 22 (16.11.2022): 3665. http://dx.doi.org/10.3390/foods11223665.

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In this study, pectin (PC), whey protein isolate (WPI), and chitosan (CS) were combined with purple corn cob anthocyanins (PCCA). Four complexes, PC−PCCA, WPI−PCCA, WPI−PC−PCCA, and CS−PC−PCCA were prepared to evaluate the improvement in the α-glucosidase inhibitory activity and digestive stability of PCCA. The encapsulation efficiency (EE), particle size, physical properties, and mode of action of the synthesized PCCA complexes were evaluated. Among them, CS−PC−PCCA had the highest EE (48.13 ± 2.73%) except for WPI−PC−PCCA; furthermore, it had a medium size (200−300 nm), the lowest hygroscopicity (10.23 ± 0.28%), lowest solubility (10.57 ± 1.26%), and highest zeta potential (28.20 ± 1.14). CS−PC−PCCA was multigranular and irregular in shape; X-ray diffraction showed that it was amorphous; and Fourier transform infrared spectroscopy confirmed that it was joined with PCCA through hydrogen bonds and electrostatic interactions. Compared with PCCA, the four complexes showed a higher α-glucosidase inhibition activity and digestive stability, except for WPI−PC−PCCA. Furthermore, CS−PC−PCCA exhibited the best α-glucosidase inhibition and simulated digestion stability.

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Nazli, Sukma Azureen, Yung-An Chua, Noor Alicezah Mohd Kasim, Zaliha Ismail, Ahmad Bakhtiar Md Radzi, Khairul Shafiq Ibrahim, Sazzli Shahlan Kasim, Azhari Rosman i Hapizah Nawawi. "Familial hypercholesterolaemia and coronary risk factors among patients with angiogram-proven premature coronary artery disease in an Asian cohort". PLOS ONE 17, nr 9 (2.09.2022): e0273896. http://dx.doi.org/10.1371/journal.pone.0273896.

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Background Familial hypercholesterolaemia (FH) patients have elevated levels of low-density lipoprotein cholesterol, rendering them at high risk of premature coronary artery disease (PCAD). However, the FH prevalence among angiogram-proven PCAD (AP-PCAD) patients and their status of coronary risk factors (CRFs) have not been reported in the Asian population. Objectives This study aimed to (1) determine the prevalence of clinically diagnosed FH among AP-PCAD patients, (2) compare CRFs between AP-PCAD patients with control groups, and (3) identify the independent predictors of PCAD. Methods AP-PCAD patients and FH patients without PCAD were recruited from Cardiology and Specialist Lipid Clinics. Subjects were divided into AP-PCAD with FH (G1), AP-PCAD without FH (G2), FH without PCAD (G3) and normal controls (G4). Medical records were collected from the clinic database and standardised questionnaires. FH was clinically diagnosed using Dutch Lipid Clinic Network Criteria. Results A total of 572 subjects were recruited (males:86.4%; mean±SD age: 55.6±8.5years). The prevalence of Definite, Potential and All FH among AP-PCAD patients were 6%(19/319), 16% (51/319) and 45.5% (145/319) respectively. G1 had higher central obesity, family history of PCAD and family history of hypercholesterolaemia compared to other groups. Among all subjects, diabetes [OR(95% CI): 4.7(2.9,7.7)], hypertension [OR(95% CI): 14.1(7.8,25.6)], FH [OR(95% CI): 2.9(1.5,5.5)] and Potential (Definite and Probable) FH [OR(95% CI): 4.5(2.1,9.6)] were independent predictors for PCAD. Among FH patients, family history of PCAD [OR(95% CI): 3.0(1.4,6.3)] and Definite FH [OR(95% CI): 7.1(1.9,27.4)] were independent predictors for PCAD. Conclusion Potential FH is common among AP-PCAD patients and contributes greatly to the AP-PCAD. FH-PCAD subjects have greater proportions of various risk factors compared to other groups. Presence of FH, diabetes, hypertension, obesity and family history of PCAD are independent predictors of PCAD. FH with PCAD is in very-high-risk category, hence, early management of modifiable CRFs in these patients are warranted.

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Huang, Ling-Hui, Lian-Shin Lin, Chun-Li Wang, Yu-Chen Chang, Lung-Chun Lee, Chung-Chieh Hu, Pi-Shan Hsu i Wei-Min Chu. "Palliative Care Consultation Services on Terminally Ill Cancer Patients and Non-Cancer Patients: Trend Analysis from a 9-Year-Long Observational Study in Taiwan". International Journal of Environmental Research and Public Health 18, nr 18 (19.09.2021): 9882. http://dx.doi.org/10.3390/ijerph18189882.

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Early integration of palliative care for terminally ill cancer and non-cancer patients improves quality of life. However, there are sparse data on results of palliative care consultation services (PCCS) between cancer and non-cancer patients. In this 9-year observational study, data were collected from the Hospice-Palliative Clinical Database (HPCD) of Taichung Veterans General Hospital (TCVGH). Terminally ill cancer and non-cancer patients who received PCCS during 2011 to 2019 were enrolled. Trend analysis was performed to evaluate differences in outcomes of PCCS, including duration of PCCS, the awareness of disease of patients and families before and after PCCS, status of PCCS termination, and DNR declaration before and after PCCS among cancer and non-cancer patients throughout study period. In total, 5223 cancer patients and 536 non-cancer patients received PCCS from 2011 to 2019. The number of people who received PCCS increased stably over the decade, both for cancer and non-cancer patients. The average duration of PCCS for cancer and non-cancer patients was 21.4 days and 18.4 days, respectively. Compared with non-cancer patients, cancer patients had longer duration of PCCS, less DNR declaration (82% vs. 98%, respectively), and more transfers to the palliative care unit (17% vs. 11%, respectively), or for palliative home care (12% vs.8%, respectively). Determinants of late referral to PCCS includes age (OR 0.992, 95% CI 0.987–0.996), DNR declaration after PCCS (OR 1.967, 95% CI 1.574–2.458), patients’ awareness after PCCS (OR 0.754, 95% CI 0.635–0.895), and status of PCCS termination. This 9-year observational study showed that the trend of PCCS among cancer and non-cancer patients had changed over the duration of the study, and early integration of PCCS to all patients is essential for both cancer and non-cancer patients.

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Gupta, Deepti, Sunita Bijarnia-Mahay, Sudha Kohli, Renu Saxena, Ratna Dua Puri, Yosuke Shigematsu, Seiji Yamaguchi i in. "Seventeen Novel Mutations in PCCA and PCCB Genes in Indian Propionic Acidemia Patients, and Their Outcomes". Genetic Testing and Molecular Biomarkers 20, nr 7 (lipiec 2016): 373–82. http://dx.doi.org/10.1089/gtmb.2016.0017.

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Xie, Qingtong, Xudong Zheng, Liuting Li, Liqun Ma, Qihui Zhao, Shiyuan Chang i Lijun You. "Effect of Curcumin Addition on the Properties of Biodegradable Pectin/Chitosan Films". Molecules 26, nr 8 (8.04.2021): 2152. http://dx.doi.org/10.3390/molecules26082152.

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A pectin/chitosan matrix-loaded curcumin film (PCCF) with a deep eutectic solvent (DES) as the solvent and plasticizer was prepared in this study. Different quantities of curcumin (identified as PCCF-0, PCCF-1, PCCF-2. PCCF-3) were loaded on the pectin/chitosan film in order to evaluate their effects on the film properties. Results showed that curcumin could interact with the pectin/chitosan matrix and form a complex three-dimensional network structure. PCCF could promote the thickness, tensile strength, thermal properties, antioxidant and antiseptic capacities, but deteriorate the light transmission and elongation at the same time. The addition of curcumin would change the color of the film, without significantly affecting the moisture content. The tensile strength of PCCF-3 reached the maximum value of 3.75 MPa, while the elongation decreased to 10%. Meanwhile, the water-resistance properties of PCCF-3 were significantly promoted by 8.6% compared with that of PCCF-0. Furthermore, PCCF showed remarkable sustained antioxidant activities in a dose-dependent manner. PCCF-3 could inhibit DPPH and ABTS free radicals by 58.66% and 29.07%, respectively. It also showed antiseptic capacity on fresh pork during storage. Therefore, curcumin addition could improve the barrier, mechanical, antioxidant and antiseptic properties of the polysaccharide-based film and PCCF has the potential to be used as a new kind of food packaging material in the food industry.

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Sandheimer, Christine, Cecilia Björkelund, Gunnel Hensing, Kirsten Mehlig i Tove Hedenrud. "Implementation of a care manager organisation and its association with antidepressant medication patterns: a register-based study of primary care centres in Sweden". BMJ Open 11, nr 3 (marzec 2021): e044959. http://dx.doi.org/10.1136/bmjopen-2020-044959.

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ObjectiveTo evaluate the implementation of a care manager organisation for common mental disorders and its association with antidepressant medication patterns on primary care centre (PCC) level, compared with PCCs without this organisation. Moreover, to determine whether a care manager organisation is associated with antidepressant medication patterns that is more in accordance with treatment guidelines.DesignRegister-based study on PCC level.SettingPrimary care in Region Västra Götaland, Sweden.ParticipantsAll PCCs in the region. PCCs were analysed in three subgroups: PCCs with a care manager organisation during 2015 and 2016 (n=68), PCCs without the organisation (n=92) and PCCs that shifted to a care manager organisation during 2016 (n=42).Outcome measuresProportion of inadequate medication users, defined as number of patients >18 years with a common mental disorder diagnosis receiving care at a PCC in the region during the study period and dispensed 1–179 defined daily doses (DDD) of antidepressants of total patients with at least 1 DDD. The outcome was analysed through generalised linear regression and a linear mixed-effects model.ResultsOverall, all PCCs had about 30%–34% of inadequate medication users. PCCs with a care manager organisation had significantly lower proportion of inadequate medication users in 2016 compared with PCCs without (−6.4%, p=0.02). These differences were explained by higher proportions in privately run PCCs. PCCs that shifted to a care manager organisation had a significant decrease in inadequate medication users over time (p=0.01).ConclusionsPublic PCCs had a more consistent antidepressant medication pattern compared with private PCCs that gained more by introducing a care manager organisation. It was possible to document a significant decrease in inadequate medication users, notwithstanding that PCCs in the region followed the guidelines to a comparatively high extent regardless of present care manager organisation.

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