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Τετάρτη 27 Φεβρουαρίου 2019

Genetic analysis in Egyptian patients with Chediak–Higashi syndrome reveals new LYST mutations



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Comment on ‘Baseline patients’ characteristics as predictors for therapeutic survival and response in patients with psoriasis on biological treatments'



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Review of Health Problems in Adult Patients with Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Exp Clin Endocrinol Diabetes 2019; 127: 171-177
DOI: 10.1055/a-0820-2085

The introduction of newborn screening programmes in most Western countries for classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH) enables timely introduction of life-saving glucocorticoid replacement in affected babies. Early diagnosis and optimised pediatric care not only successfully led to survival but also allow that almost all patients reach adulthood. Cohort studies in adults, however, provided evidence for significant health problems and co-morbidities of adult patients such as life-threatening adrenal crises, cardiovascular and metabolic health problems, fertility problems, benign endocrine tumours, and osteopenia and osteoporosis. This review summarises the current state of knowledge aiming to emphasize the neccessity of primary and secondary prevention of additional long-term health issues as a major task of health professionals in the care of CAH.
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© Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
Table of contents  |  Abstract  |  Full text



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The Adrenal Gland: Central Relay in Health and Disease

Exp Clin Endocrinol Diabetes 2019; 127: 81-83
DOI: 10.1055/a-0752-4176

Diseases of the adrenal gland are as important for the general practitioner as for the endocrine specialist. The high prevalence of some adrenal endocrinopathies, such as adrenal incidentalomas (1–2% of the population) and primary aldosteronism (6% of hypertensives), which affect millions of patients, makes adrenal diseases a relevant health issue. The high morbidity and mortality of some of the rarer adrenal diseases, i. e., Addison's disease and Cushing's syndrome (Table 1), make early detection and appropriate treatment such a challenge for the health care system.
[...]

© Georg Thieme Verlag KG Stuttgart · New York

Article in Thieme eJournals:
Table of contents  |  Abstract  |  Full text



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Surgical Management of Pediatric Lymphedema: A Systematic Review

10-1055-s-0039-1681068_180208-1.jpg

J reconstr Microsurg
DOI: 10.1055/s-0039-1681068

Introduction Lymphedema is a rare, progressive, and debilitating condition caused by failure of the lymphatic system to adequately drain the protein-rich fluid exiting the capillaries. Conservative management is often emphasized in pediatric patients, resulting in a paucity of literature describing surgical treatment in this population. Methods A systematic review was performed using PubMed, ScienceDirect, and Google Scholar to identify all studies describing surgical management of lymphedema in the pediatric population. Results Of the 343 relevant articles identified, 14 met the criteria for full review. Articles were divided into the following treatment categories: genital lymphedema, excisional procedures for extremity lymphedema, and physiologic procedures for extremity lymphedema. Outcomes for genital lymphedema were overall positive. For extremity lymphedema, excisional procedures yielded good results overall and included the majority of patients in this study. Physiologic procedures had mixed outcomes in the small population included in this study, but definitive conclusions cannot be drawn due to the paucity of existing data. Conclusion While conservative management of pediatric lymphedema is well described, the literature remains sparse regarding surgical treatment. Further studies are needed to evaluate efficacy and outcomes in all three categories in this population.
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Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Article in Thieme eJournals:
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Characteristics of single atmospheric particles in a heavily polluted urban area of China: size distributions and mixing states

Abstract

To investigate the chemical composition, size distribution, and mixing state of aerosol particles on heavy pollution days, single-particle aerosol mass spectrometry was conducted during 9–26 October 2015 in Xi'an, China. The measured particles were classified into six major categories: biomass burning (BB) particles, K-secondary particles, elemental carbon (EC)–related particles, metal-containing particles, dust, and organic carbon (OC) particles. BB and EC-related particles were the dominant types during the study period and mainly originated from biomass burning, vehicle emissions, and coal combustion. According to the ambient air quality index, two typical episodes were defined: clean days (CDs) and polluted days (PDs). Accumulation of BB particles and EC-related particles was the main reason for the pollution in Xi'an. Most types of particle size were larger on PDs than CDs. Each particle type was mixed with secondary species to different degrees on CDs and PDs, indicating that atmospheric aging occurred. The mixing state results demonstrated that the primary tracers were oxidized or vanished and that the amount of secondary species was increased on PDs. This study provides valuable information and a dataset to help control air pollution in the urban areas of Xi'an.

Graphical abstract


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Effects of operating parameters on in situ NH 3 emission control during kitchen waste composting and correlation analysis of the related microbial communities

Abstract

Ammonia emission during composting results in anthropogenic odor nuisance and reduces the agronomic value of the compost due to the loss of nitrogen. Adjusting the operating parameters during composting is an emerging in situ odor control technique that is cheap and highly efficient. The effects of in situ NH3 emission control were investigated in this study by simultaneously adjusting key operating parameters (such as C/N ratio, aeration rate, and moisture content) during the composting processes (C1–C9). Results showed that the average NH3 emission concentrations for different treatments were in the order of C1 > C4 > C2 > C5 > C3 > C6 > C7 > C8 > C9. The total content of NH3 emission (21.02 g/kg) in C9 (C/N ratio = 35, aeration rate = 15 L/min, and moisture content = 60%) was much lower than that (65.95 g/kg) in C1 (C/N ratio = 15, aeration rate = 5 L/min, and moisture content = 60%). The nitrogen loss ratio was 27.36% for C1, while 16.15% for C9. The microbial diversity and abundance in C9 and C1 were compared using high-throughput sequencing. The relationship between NH3 emission, operating parameters, and the related functional microbial communities was also investigated. Results revealed that Nitrosospira, Nitrosomonas, Nitrobacter, Pseudomonas, Methanosaeta, Rhodobacter, Paracoccus, and Sphingobacterium were negatively related to NH3 emission. According to the above results, the optimal values for different operating parameters for the in situ NH3 control during kitchen waste composting were, respectively, moisture content of 70%, C/N ratio of 35, and aeration rate of 15 L/min, with the order of effectiveness from high to low being aeration rate > C/N > moisture. This information could be used as a valuable reference for the in situ NH3 emission control during kitchen waste composting.



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The effects of QMix and EndoActivator on postoperative pain in mandibular molars with nonvital pulps: a randomized clinical trial

Abstract

Aim

The purpose of this study was to compare the effects of different irrigation solutions when used EndoActivator on postoperative pain.

Methodology

A total of 104 subjects with nonvital pulps were selected and randomly assigned to four experimental groups; (G) 1 (n = 26) 2.5% NaOCl + conventional endodontic syringe, (G) 2 (n = 26) 2.5% NaOCl + EndoActivator, (G) 3 (n = 26) 2.5% NaOCl + QMix + conventional endodontic syringe, (G) 4 (n = 26) 2.5% NaOCl + QMix + EndoActivator. All the patients were prescribed ibuprofen 400 mg to be taken every 8 h in case of pain. The patients were asked to rate the severity of the postoperative pain on a visual analog scale (VAS) for 8th, 24th, 48th, and 72nd hours after the visit. The data were analyzed using Kruskal-Wallis, Mann-Whitney U, and Chi-Square tests.

Results

G4 resulted in lesser postoperative pain levels on 8th, 24th, 48th, and 72nd hours than the G2, G3, and G1 did. There was no significant difference between G4 and G2 and between G3 and G1 in terms of the postoperative pain levels at any time interval (P > 0.05).

Conclusion

It was determined that the postoperative values of the groups, in which EA was used, were statistically significantly lower than in the other groups. The combined use of QMix and EA provided the most significant decrease in the postoperative pain values.

Clinical relevance

Successful management of postoperative pain is an important issue for the clinician. The combined use of Qmix and EA can be recommended to the clinicians.



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Effects of secondary metabolites produced by different cyanobacterial populations on the freshwater zooplankters Brachionus calyciflorus and Daphnia pulex

Abstract

Cyanobacterial blooms in eutrophic water bodies are a worldwide problem. Combined effects of mixtures of secondary metabolites produced by different cyanobacterial species on aquatic fauna are still not well recognised. We compared the survivorship of Brachionus calyciflorus Pallas (Rotifera) and Daphnia pulex Leyding (Cladocera) exposed to pure microcystin LR (MC-LR), anatoxin-a (ANTX) and to five extracts obtained from bloom-forming cyanobacteria Microcystis, Planktothrix and Dolichospermum. The obtained results revealed different response of the organisms to high concentrations of pure MC-LR, ANTX and complex cyanobacterial extracts. The extracts' toxicity to invertebrates was higher than that exerted by pure cyanotoxins and was dependent on the composition of cyanobacterial metabolites: Microcystis spp. extract containing anabaenopeptins A and B, aeruginosamide, four variants of cyanopeptolins and five MCs was not toxic to either of the organisms, whereas Planktothrix agardhii extract (I), containing anabaenopeptins A, B, F, 915, oscillamide Y, five different aeruginosins and four variants of MC was more toxic to daphnids than to rotifers. The extracts of another P. agarhdii (II) biomass and two different biomass samples of Dolichospermum spp. also affected survivorship of the rotifer and cladoceran, however, to various extent. It strongly suggests that non-ribosomal oligopeptides, other than MCs, had essential contribution to the observed toxicity to invertebrates and their effects on particular species or populations can vary depending on the secondary metabolite profiles of cyanobacteria.



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Congo red dye diversely affects organisms of different trophic levels: a comparative study with microalgae, cladocerans, and zebrafish embryos

Abstract

Global consumption of synthetic dyes is roughly 7 × 105 tons per year, of which the textile industry expends about two-thirds. Consumption of synthetic dyes produces large volumes of wastewater discharged into aquatic ecosystems. Colored effluents produce toxic effects in the hydrobionts, reduce light penetration, and alter the photosynthetic activity, causing oxygen depletion, among other effects. Some dyes, such as Congo red (CR), are elaborated with benzidine, a known carcinogenic compound. Information regarding dye toxicity in aquatic ecosystems is scarce; therefore, our study was aimed at evaluating the toxicity of CR on a battery of bioassays: the microalga Pseudokirchneriella subcapitata, the cladocerans Daphnia magna and Ceriodaphnia rigaudi, and the zebrafish Danio rerio. P. subcapitata was the most sensitive species to CR (IC50, 3.11 mg L−1); in exposed individuals, population growth was inhibited, but photosynthetic pigments and macromolecule concentrations were stimulated. D. magna was tolerant to high dye concentrations, the determined LC50 (322.9 mg L−1) is not an environmentally relevant value, but for C. rigaudi, LC50 was significantly lower (62.92 mg L−1). In zebrafish embryos, exposure to CR produced yolk sac edema, skeletal deformities, and stopped larvae hatching; lack of heart beating was the only observed lethal effect. CR affected organisms of different trophic levels diversely. Particularly, the effects observed in microalgae confirm the vulnerability of primary producers to dye-polluted wastewaters, because dyes produced toxic effects and interfered with photosynthesis. Different cladoceran species displayed different acute effects; thus, species sensitivity must also be considered when toxicity of dyes is assessed. Inhibition of fish larvae hatching is a significant effect not previously reported that warns about the toxicity of dyes in fish population dynamics. Synthetic azo colorants should be considered as emerging pollutants because they are discharged into the aquatic environment and are not currently included in the environmental regulation of several countries.



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Comparison of multi-criteria analysis methodologies for the prioritization of arsenic-contaminated sites in the southwest of China

Abstract

The issue of contaminated sites has been highlighted as an immediate priority in the 13th Five-Year Plan of China. Identification and prioritization of contaminated sites are of key importance for proposing effective strategies for the regional management of contaminated sites. In this study, three advanced multi-attribute methodologies, the risk-based priority methodology, the regional risk assessment methodology, and the dominance-based rough set approach (DRSA), were comparatively employed to screen contaminated sites in, Guangxi, Southwest of China. The results of the three prioritizations show that the highest ranking site identified by the three methods had great agreement. In regard to the screening attributers, while the risk-based prioritization methodology and regional risk assessment methodology allowed a high discrimination in the screening of contaminated sites associated with different attributes, such as farmland, residential areas, contaminant level, number of people, area, storage quality, site service life, and surrounding communities, the DRSA allowed the identification of contamination strength (CS) and contamination potential (CP).



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Catalytic CO 2 gasification of rubber seed shell-derived hydrochar: reactivity and kinetic studies

Abstract

In this study, hydrothermal carbonization (HTC) of a biomass was used as a means to improve the physicochemical properties of rubber seed shell (RSS) and enhance its reactivity in the char-CO2 gasification reaction, known as the Boudouard reaction (C + CO2 ↔ 2CO). Hydrochar samples were developed by hydrothermal treatment of RSS, without separating the solid residue from the liquid product, at 433, 473, 513, and 553 K under autogenous pressure. The CO2 gasification reactivity of the developed hydrochars was then investigated at different heating rates (5, 10, 20, and 30 K/min) by the non-isothermal thermogravimetric method. The hydrochars revealed higher reactivity and improved gasification characteristics compared to the untreated biomass, while the hydrochar which was filtered from the liquid slurry showed lower reactivity compared to the untreated biomass. This was due to the chemical and structural evolutions of the biomass during hydrothermal treatment as indicated by various analyses. The gasification reactivity of the hydrochar was substantially enhanced by introduction of a catalyst (NaNO3) during HTC. Kinetic analysis of the char-CO2 gasification reaction was carried out by applying Flynn-Wall-Ozawa (FWO), Kissinger-Akahira-Sunose (KAS), and Starink isoconversional methods, and thermodynamic parameters were also determined. The activation energy of the Na-loaded RSS hydrochar in CO2 gasification (120–154 kJ/mol) was considerably lower than that of the untreated biomass (153–172 kJ/mol). Thermodynamic studies also confirmed the promoting effect of hydrothermal treatment and catalyst impregnation on enhancement of reactivity of the virgin biomass and reduction of gasification temperature.



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Impact of phosphate additive on organic carbon component degradation during pig manure composting

Abstract

Phosphate, as an additive to composting, could significantly reduce ammonia emission and nitrogen loss but may also cause adverse effects on the degradation of organic matter. However, there is little information about the influence of pH change, salt content, and phosphate on different organic fraction degradation during composting with the addition of phosphate at a higher level. In this study, the equimolar phosphoric acid (H3PO4), sulfuric acid (H2SO4), and dipotassium phosphate (K2HPO4) were added into pig manure composting with 0.25 mol mass per kilogram of dry matter basis addition amount to evaluate the effect of H+, PO43−, and salinity on carbon component transformation and organic matter degradation. The results showed that both H3PO4 and K2HPO4 additives could lead to shorter duration in the thermophilic phase, lower degradation of lignocellulose, and lesser carbon loss compared to CK, even though had different pH, i.e., acidic and alkaline conditions, respectively. Besides, the addition of H3PO4, H2SO4, and K2HPO4 could increase the degradation of soluble protein and lipid during composting. Redundancy analysis demonstrated that the variation in different organic carbon fractions was significantly correlated with the changes of pH and the presence of PO43−, but not with SO42− and electrical conductivity, suggesting that pH and phosphate were the more predominant factors than salinity for the inhibition of organic matter degradation. Taken together, as acidic phosphate addition produces a true advantage of controlling nitrogen loss and lower inhibition of organics transformation during composting, the expected effects may result in more efficient composting products.



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Issue Information



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Multiple Keratin Granulomata: A Potential Histologic Clue to Cutaneous Squamous Cell Carcinoma Responding to PD‐1 Inhibitor Therapy

We present histologic features of a locally advanced cutaneous squamous cell carcinoma treated with programmed cell death protein ‐1 (PD‐1) antagonist, pembrolizumab, with a partial response. This contributes to a growing body of literature supporting pembrolizumab's efficacy in treatment of surgically‐unresectable cutaneous squamous cell carcinoma. We also provide a detailed description of the histologic features, particularly keratin granulomata with adjacent lymphocytic aggregates and fibrosis, observed in cutaneous squamous cell carcinoma under treatment with a PD‐1 antagonist.

This article is protected by copyright. All rights reserved.



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Are drug intradermal tests dangerous to explore cross‐reactivity and co‐sensitization in DRESS?

Abstract

Drug Reaction with Eosinophilia and Systemic Symptoms syndrome (DRESS) is a severe Cutaneous Adverse drug Reaction, for which allergological explorations are poorly codified.

Patch tests are usually well tolerated, and their sensitivity depends on the tested drug. Due to a potential risk of relapse during the tests, intradermal tests (IDTs) are contra‐indicated in severe cutaneous drug adverse reactions 1. Consequently, results of IDTs in DRESS allergological work‐up have been rarely reported.

The objective of this retrospective monocentric study was to assess the value and tolerance of drug IDTs in patients who had a DRESS and negative patch tests with the same drugs.

This article is protected by copyright. All rights reserved.



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Interaction of biologic therapy with apical periodontitis and periodontitis: A systematic review

Abstract

Biologic medications (BMs) are increasingly used for the management of systemic chronic inflammatory diseases. These diseases are often associated with a higher prevalence of apical periodontitis (AP) and periodontitis (P). The purpose of this systematic review was to ascertain the interactions between AP and/or P and BMs. The review was registered in the PROSPERO database (CRD42017054756). Electronic searches were performed on Pubmed Medline, Scopus and The Web of Science from their inception through to 20 March 2018. The references of the articles selected were checked. The keywords were chosen based on a pilot search which aimed to find the most frequently prescribed BMs. The included studies were appraised qualitatively using appropriate tools. Thirty‐five articles met the inclusion criteria, comprising 16 non‐randomised clinical studies, 12 in vivo animal studies and 7 case reports. Quality of information was assessed as high in 18 articles, moderate in 16 articles and low in 1 article. BMs in patients suffering from chronic inflammatory diseases seem to inhibit the progression of AP and P, and to enhance the healing response to periodontal and endodontic treatment. A healthier condition of the periodontal tissues seems to be associated with a better response of the patient to BMs therapy.

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Abivertinib Maleate Versus Geifitinib in Patients With Advanced Non-small Cell Lung Cancer With Sensitive EGFR Mutation

Condition:   Advanced Non-small Cell Lung Cancer
Interventions:   Drug: Abivertinib Maleate Capsules;   Drug: Placebo Gefitinib Tablets;   Drug: Gefitinib Tablets;   Drug: Placebo Abivertinib Maleate Capsules
Sponsors:   Hangzhou ACEA Pharmaceutical Research Co., Ltd.;   Guangdong Provincial People's Hospital
Not yet recruiting

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Letter to “Successful radiofrequency ablation strategies for benign thyroid nodules”



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Letter to “Successful radiofrequency ablation strategies for benign thyroid nodules”



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