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Epidemiological profile of the homeless population assisted by voluntary initiative and the social impact on access to healthcare: an investigative study in Curitiba, Brazil

Published on: 7th July, 2021

OCLC Number/Unique Identifier: 9137583522

Objective: The study aimed to describe the epidemiological profile of the homeless population and report the social impact on healthcare services provided by a voluntary initiative during one-year of follow-up. Method: This is an investigational study, carried out in an important capital-city of southern Brazil, which included 509 individuals in 2019. Findings were divided into three segments: sociodemographic, substance use/abuse and impact on healthcare. Results: The sociodemographic profile found was predominantly male, white, single, heterosexual, educated in elementary or high school, in regular use of legal and/or illegal substances, and living as homeless for less than a year. The impact of assistance provided on the perception of individuals who returned in subsequent actions was evident. Conclusion: The significant increase in the number of homeless people in large urban centers can benefit from the support of non-profit organizations to address the deficiencies faced in accessing basic health services. Based on this, we seek to advance in the development of new policies that improve local healthcare for the vulnerable population, to be more assertive to their needs.
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A retrospective review of the factors that negatively influenced the eradication of polio in South-South, Nigeria

Published on: 30th July, 2021

OCLC Number/Unique Identifier: 9186964198

Background: The endemic nature of poliomyelitis in Nigeria was a source of concern to many given the global efforts aimed at eradicating the scourge. In this study, the factors that influenced the eradication of this malady in South-South Nigeria (Akwa Ibom and Cross River State) were investigated. Four null hypotheses, drawn on these factors (including culture belief, religious difference, language and the prevalence of ethnic conflict) guided the investigation. The main thrust of this study was to examine how socio-cultural factors negatively influenced the eradication of polio in the study area. Methods: Through stratified random and purposive sampling techniques, 600 adult men and women who had children were selected for the study. A structured questionnaire constructed by the researcher was used in data collection. Data analysis was carried out using one-way ANOVA and independent t-test statistical tools. Results: The findings were that cultural belief and religious affiliation exerted significant influence on the people’s effort towards polio eradication. Conclusion: The study indicated that the low use of health services by the people was brought about by variation in these variables. Among other things, it was suggested that continuous awareness campaigns be carried out to educate the people on the need for immunization anchored in science and technology.
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Heat transfer investigation of Non-Newtonian Fluid between two vertically infinite flat plates by numerical and analytical solutions

Published on: 18th May, 2017

OCLC Number/Unique Identifier: 7317592196

In natural convection, the fluid motion occurs by natural means such as buoyancy. Heat transfer by natural convection happens in many physical problems and engineering applications such as geothermal systems, heat exchangers, petroleum reservoirs and nuclear waste repositories. These problems and phenomena are modeled by ordinary or partial differential equations. In most cases, experimental solutions cannot be applied to these problems, so these equations should be solved using special techniques. In this paper, natural convection of a non-Newtonian fluid flow between two vertical flat plates is investigated analytically and numerically. Collocation Method (CM) and fourth-order Runge -Kutta numerical method (NUM) are used to solve the present problem. These methods are powerful and convenient algorithms in finding the solutions for the equations. While these are capable of reducing the size of calculations. In order to compare with exact solution, velocity and temperature profiles are shown graphically. The obtained results are valid with significant accuracy.
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Wifi and health: Perspectives and risks

Published on: 12th October, 2017

OCLC Number/Unique Identifier: 7317602372

Increased exposure to electromagnetic fields such as radio frequencies used by Wifi technology raise questions and concerns about their impact on health. For answer these questions, several scientific studies have carried out followed by results publication in prestigious scientific revues. Literature conducted on the effects of non-ionizing radiation and Wifi waves is vast and sometimes controversial. Epidemiological studies and the results of in vitro and in vivo experimental studies have showed the biological effects of electromagnetic field in different frequencies range. These effects caused disorders at the molecular and behavioral level. However, these studies were insufficient to confirm the directly related effects to the cause. Therefore, further research must be done to raise the controversy about the safety of wireless waves.
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Naphazoline nitrate treat the Frey effect of microwave and other sonic weapon’s damages in Human’s Internal, Endogenous Organs

Published on: 1st February, 2019

OCLC Number/Unique Identifier: 8017063333

State Department had evacuated a number of Americans from the U.S. consulate in Guangzhou, China after they experienced unexplained health issues. A group of U.S. diplomats stationed in China have been brought back to the states after being inflicted by a mystery illness that reportedly resembles the brain injuries previously suffered by staff in Cuba. At the end of the December 2018 we have found a medicine fully treating the damages caused the Frey Effect of Microwave and other types of Sonic Weapons in Human’s internal, endogenous organs. I am proposing to use Naphasoline nitrate, (former) nasal decongestant, to treat Carcinogenesis of the Human’s internal, endogenous organs caused by Sonic Weapons through the release and cleaning of the Lymphatic ways in patients with colorectal, colon, pancreatic, breast, etc., cancer. I have proved this healing effect of the Naphazoline nitrate on myself during treatment in last months of the year 2018.
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Nanotherapeutic agent for cancer: Miracle or catastrophe

Published on: 28th June, 2019

OCLC Number/Unique Identifier: 8172439175

Nanotechnology is a smart technology in the field of biomedical engineering used for the diagnosis and treatment of diseases. Nanodrugs provide better encapsulation of drug and efficiency at low dosage to kill the targeted tissue/cells. However, the chances of chronic toxicity and high cost of treatment limits its applicability [1]. To overcome these problems still, the experts of the scientific community have been working on it, to design the best one and cost-effective treatment for the human welfare.
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Synthesis of NaYF4:Yb,Er@SiO2@Ag core-shell nanoparticles for plasmon-enhanced upconversion luminescence in bio-applications

Published on: 12th October, 2019

OCLC Number/Unique Identifier: 8278640404

The present report highlights our results on synthesis of NaYF4:Yb,Er@SiO2@Ag core–shell nanoparticles (CSNPs) for plasmon-enhanced upconversion luminescence (UCL). Hydrophilic surface UCL nanoparticles (UCLNPs) as cores were obtained by precipitation of Rare Earth Elements (REE) chlorides from water-alcohol solutions. The formation of a hydrophobic surface of α-NaYF4:Yb,Er NPs was achieved by thermolysis method at 280 °C and β-NaYF4:Yb,Er by precipitation method in nonpolar medium at 320 °C. Silica shell was formed by the modified Stöber method on the surfaces of UCLNPs with different polarity and phase composition. A mixture of hexane-cyclohexane-isopropyl alcohol was used as a medium for the formation of mononuclear CSNPs on hydrophobic surfaces of cores with different thicknesses of the silica shell: 5 nm and 14 nm. Formation of a predetermined thickness of silica shell was carried out by introducing a precise quantity of TEOS taking into account the size of core NPs with molar ratio TEOS: H2O equal to 1:6. The morphology and phase composition of cores and CSNPs were examined by transmission electron microscopy and selected area electron diffraction, respectively. The insertion of Ag NPs into the structure of NaYF4:Yb,Er@SiO2 was carried out in parallel at the stage of shell formation, which made this synthesis a one-step process. The control of the size of Ag NPs was implemented through the use of a colloidal solution of NPs of the cluster structure by changing the polarity of the medium. The highest intensity enhancement of 85-fold with 5 nm and 29-fold with 14 nm shell thickness was recorded, respectively. For the first time, tests on bioimaging of neutrophil cells by those CSNPs are demonstrated.
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Peptide-based antifouling aptasensor for cardiac troponin I detection by surface plasmon resonance applied in medium sized Myocardial Infarction

Published on: 14th February, 2020

OCLC Number/Unique Identifier: 8541469721

Highly selective and sensitive detection of cardiac troponin I (cTnI) is a powerful complement to clinical diagnosis of acute myocardial infarction (AMI). In this study, a strategy for cTnI detection was developed by constructing a universal biosensing interface composed of zwitterionic peptides and aptamers. The peptides were self-assembled onto gold chips, and some of them were biotinylated. The cTnI-specific binding aptamers were immobilized through the streptavidin-biotin system. Surface plasmon resonance (SPR) measurements revealed the preparation process. The developed aptasensor presents a linear detection with cTnI ranging from 20 ng/ml to 600 ng/ml and a detection limit of 20 ng/ml. The high immobilization of the aptamer enhances the sensitivity of the aptasensor and the calculated KD was 6.75 nM. Due to the outstanding antifouling property of the zwitterionic peptide, the developed aptasensor possesses a high resistance towards protein fouling. Moreover, the aptasensor has excellent selectivity and specificity towards cTnI in complex media. Hence, the proposed peptide-based aptasensor shows great potential for practical application in medium sized Myocardial Infarction (MI).
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Mesoscopic irreversible thermodynamics of morphological evolution kinetics of helical conformation in bioproteins ‘DNA’ under the isothermal isobaric conditions

Published on: 11th March, 2020

OCLC Number/Unique Identifier: 8570215259

The morphological evolution kinetics and instabilities of alpha helical peptide 3.613, which involves large amount of stored torsional elastic deformation energy (3-40 eV/molecule), is formulated by the variational method based on the connection between the rates of internal entropy production and the changes in the global Gibbs free energy, assuming that one has isobaric irreversible processes under the isothermal conditions. The present mesoscopic nonequilibrium thermodynamic approach relies on the fact that the global Gibbs free energy of helical conformation involves not only the bulk Gibbs free energy of the amino-acid back bone structure but also the interfacial Gibbs free energy of the enclosing cylindrical shell or the cage associated with the side-wall molecular branches, and their interactions with the immediate surroundings. The proposed variational analysis applied directly on the proposed macro-model has furnished a nonlinear integral equation in terms of the normalized and scaled internal and external variables. This allows us to track down the motion of the total pitch height of the alpha polypeptide along the well-defined trajectories in the displacement-time space, dictated not only by the initial configuration of the helix but also through the gradients of the global Gibbs free energy of the strained helical conformation as the main driving force. In the negative manifold, there is a well-defined region below the dynamic instability regime, in which the helical conformation can evolve towards the nonequilibrium stationary states by expanding, or contracting, depending upon whether the interfacial free energy and/or the applied stress system are below or above the well-defined thresholds level dictated by the initial pitch height. The highest life time may be realized along that trajectory, which follows up the threshold level of the interfacial specific Gibbs free energy, which is gs = -315 erg/cm2. In the upper region of the negative manifold, the helical conformations are driven by the very large applied uniaxial tension or the negative pressure induced by the thermal expansion, in the range of p > 1GPa and/or the strong negative interfacial free energies [3-4 pH] or their combinations, they show strong kinematic instabilities, which can cause not only the accelerated unfolding phenomenon but also cause large extensions that end up with the catastrophic decimations by ruptures and fragmentations. In the positive manifold, the aging behavior of the polypeptide follows up a S-shape path having rather speedy aging behavior compared to the negative manifold, which is separated from by a well-defined boundary, which represents the isochoric path having longest relaxation times, which can be achieved with great stability. Finally, one could attempt to estimate the upper limit of the relaxation time of aging for the modern hominin, from samples of exceptional preservations, relying on the present nonequilibrium theory as well as on the very limited knowledge on the post-mortem DNA and the present pitch heights of the modern hominin, which is found to be about 25,840 yrs, with a life expectation of 451,800 yrs. These figures are very close to those calculated for Neanderthals (SH), which are found to be 31,820 yrs and 499,100 yrs, respectively.
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The Trans-zoonotic Virome interface: Measures to balance, control and treat epidemics

Published on: 9th April, 2020

OCLC Number/Unique Identifier: 8870064677

The global virome: The viruses have a global distribution, phylogenetic diversity and host specificity. They are obligate intracellular parasites with single- or double-stranded DNA or RNA genomes, and afflict bacteria, plants, animals and human population. The viral infection begins when surface proteins bind to receptor proteins on the host cell surface, followed by internalisation, replication and lysis. Further, trans-species interactions of viruses with bacteria, small eukaryotes and host are associated with various zoonotic viral diseases and disease progression. Virome interface and transmission: The cross-species transmission from their natural reservoir, usually mammalian or avian, hosts to infect human-being is a rare probability, but occurs leading to the zoonotic human viral infection. The factors like increased human settlements and encroachments, expanded travel and trade networks, altered wildlife and livestock practices, modernised and mass-farming practices, compromised ecosystems and habitat destruction, and global climate change have impact on the interactions between virome and its hosts and other species and act as drivers of trans-species viral spill-over and human transmission. Zoonotic viral diseases and epidemics: The zoonotic viruses have caused various deadly pandemics in human history. They can be further characterized as either newly emerging or re-emerging infectious diseases, caused by pathogens that historically have infected the same host species, but continue to appear in new locations or in drug-resistant forms, or reappear after apparent control or elimination. The prevalence of zoonoses underlines importance of the animal–human–ecosystem interface in disease transmission. The present COVID-19 infection has certain distinct features which suppress the host immune response and promote the disease potential. Treatment for epidemics like covid-19: It appears that certain nutraceuticals may provide relief in clinical symptoms to patients infected with encapsulated RNA viruses such as influenza and coronavirus. These nutraceuticals appear to reduce the inflammation in the lungs and help to boost type 1 interferon response to these viral infections. The human intestinal microbiota acting in tandem with the host’s defence and immune system, is vital for homeostasis and preservation of health. The integrity and balanced activity of the gut microbes is responsible for the protection from disease states including viral infections. Certain probiotics may help in improving the sensitivity and effectivity of immune system against viral infections. Currently, antiviral therapy is available only for a limited number of zoonotic viral infections. Because viruses are intracellular parasites, antiviral drugs are not able to deactivate or destroy the virus but can reduce the viral load by inhibiting replication and facilitating the host’s innate immune mechanisms to neutralize the virus. Conclusion: Lessons from recent viral epidemics - Considering that certain nutraceuticals have demonstrated antiviral effects in both clinical and animal studies, further studies are required to establish their therapeutic efficacy. The components of nutraceuticals such as luteolin, apigenin, quercetin and chlorogenic acid may be useful for developing a combo-therapy. The use of probiotics to enhance immunity and immune response against viral infections is a novel possibility. The available antiviral therapy is inefficient in deactivating or destroying the infecting viruses, may help in reducing the viral load by inhibiting replication. The novel efficient antiviral agents are being explored.
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C3D data based on 2-dimensional images from video camera

Published on: 13th January, 2021

OCLC Number/Unique Identifier: 8899341920

The Human three-dimensional (3D) musculoskeletal model is based on motion analysis methods and can be obtained by particular motion capture systems that export 3D data with coordinate 3D (C3D) format. Unique cameras and specific software are essential for analyzing the data. This equipment is quite expensive, and using them is time-consuming. This research intends to use ordinary video cameras and open source systems to get 3D data and create a C3D format due to these problems. By capturing movements with two video cameras, marker coordination is obtainable using Skill-Spector. To create C3D data from 3D coordinates of the body points, MATLAB functions were used. The subject was captured simultaneously with both the Cortex system and two video cameras during each validation test. The mean correlation coefficient of datasets is 0.7. This method can be used as an alternative method for motion analysis due to a more detailed comparison. The C3D data collection, which we presented in this research, is more accessible and cost-efficient than other systems. In this method, only two cameras have been used.
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Fecal storage condition induces variations of microbial composition and differential interpretation of metagenomic analysis

Published on: 17th March, 2021

OCLC Number/Unique Identifier: 8999916387

Advances in metagenomics have facilitated population studies of associations between microbial compositions and host properties, but strategies to minimize biases in these population analyses are needed. However, the effects of storage conditions, including freezing and preservation buffer, on microbial populations in fecal samples have not been studied sufficiently. In this study, we investigated metagenomic differences between fecal samples stored in different conditions. We collected 46 fecal samples from patients with lung cancer. DNA quality and microbial composition within different storage Methods were compared throughout 16S rRNA sequencing and post analysis. DNA quality and sequencing results for two storage conditions (freezing and preservation in buffer) did not differ significantly, whereas microbial information was better preserved in buffer than by freezing. In a metagenomic analysis, we observed that the microbial compositional distance was small within the same storage condition. Taxonomic annotation revealed that many microbes differed in abundance between frozen and buffer-preserved feces. In particular, the abundances of Firmicutes and Bacteroidetes varied depending on storage conditions. Microbes belonging to these phyla differed, resulting in biases in population metagenomic analysis. We suggest that a unified storage Methods is requisite for accurate population metagenomic studies.
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A review article on artificial intelligence

Published on: 19th May, 2021

OCLC Number/Unique Identifier: 9048248509

Artificial intelligence (AI) is the emulation of human intelligence in computers that have been trained to think and behave like humans. The word may also refer to any computer that exhibits human-like characteristics like learning and problem-solving. Artificial intelligence is intelligence demonstrated by machines, as opposed to natural intelligence, which involves consciousness and emotionality and is demonstrated by humans and animals [1].
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Assessment and sensitive analysis of biological water risks in water resources with application of classical mass transfer computations

Published on: 9th June, 2021

OCLC Number/Unique Identifier: 9124650394

Due to the urgent need for water in all parts of industrial or developing societies, water supply, and transmission facilities are suitable targets for biological risks. Given that even a short interruption in water supply and water supply operations has a great impact on daily activities in the community, the deliberate contamination of urban water resources has irreparable consequences in the field of public health, and the economy of society will follow. Unfortunately, most officials in the public health control departments in our country have received limited training in detecting accidental or intentional contamination of water resources and dealing with the spread of waterborne diseases both naturally and intentionally. For this reason, there is low preparedness in the responsible agencies to deal with waterborne diseases during biological risks. In the first step of this research, a review study has been conducted on water biological risks and operational strategies to deal with them. In the following, it has studied how Escherichia coli (E. coli) bacteria spread in aqueous media. In this regard, the kinetic model of the studied microorganism was analyzed based on the implementation of (Fick Law) in polar coordinates and the combination of (Dirac Distribution) with (Legendre polynomial) distribution. Finally, after studying the factors affecting the microbial pollutant emission coefficient, the effects of all three factors of linear velocity, linear motion time period, and angle of motion on the pollutant emission flux and biofilm diffusion time in the water supply network environment were investigated. Studies have shown that the linear velocity parameter of Escherichia coli with a nonlinear relationship has the greatest effects on the release of microbial contaminants.
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Simulating the dispersion of poisonous organic chemical compounds in wastewater treatment process through the active sludge method using the TOXChem model

Published on: 24th June, 2021

OCLC Number/Unique Identifier: 9124851637

Naturally, microorganisms decompose the organic material existing in nature, both in the presence or absence of oxygen. The majority of materials such as poisonous chemical compounds, heavy metals, would prevent the treatment process from taking place, lead to the entry of these contaminants into the environment results in the emergence of numerous diseases. In the present study, using the TOXChem4.1 simulation model, attempts were made to simulate a wastewater treatment plant and then assess the dispersions of contaminants including 1,2-Dimethylnaphthalene, 1,3-Dinitropyrene, 1,6-Dimethylnaphthalene, 1,6-Dinitropyrene, and 17a-ethinylestradiol (EE2) in concentrations of a common scenario. The results of computer simulations showed that the EE2 contaminant is of the highest percentage of decomposition among others, due to its wider chemical structure. Consequently, it is clear that such contaminant is of the highest mass in the sludge exiting the treatment plant. In addition, the results of the simulations demonstrated that the highest volumes of gaseous pollutants take place in the modulation and initial sedimentation units.
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Pure Erythroid Leukemia: The Sole Acute Erythroid Leukemia

Published on: 11th July, 2017

OCLC Number/Unique Identifier: 7317598453

Pure Erythroid Leukemia (PEL) is an aggressive and exceedingly rare form of acute leukemia. In the 2008 WHO classification PEL was one of the subtypes of acute erythroid leukemia the other subtype being erythroleukemia (erythroid/ myeloid). In the 2016 WHO classification update, erythroleukemia was merged into myelodysplastic syndrome and PEL now is the only type of acute erythroid leukemia.106 cases of acute myeloid leukemia were diagnosed in 28 months in children’s hospital Lahore and PEL constituted 0.94%. Diagnosis of PEL is made by the bone marrow morphology showing predominant Immature erythroid precursors (proerythroblastic or undifferentiated), Periodic Acid- Schiff staining and immunophenotyping. In PEL no specific genetic mutations have been described but complex karyotypes and TP53 mutations are frequently noted. Future collaborative studies to identify the molecular defects will contribute to the development of targeted therapies that might improve the prognosis.
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Comorbidity of alcohol dependence with attention-deficit/hyperactivity disorder and the role of executive dysfunctions

Published on: 30th January, 2018

OCLC Number/Unique Identifier: 7325429171

Background: This study aims to retrospectively investigate the comorbidity of ADHD multiple symptoms (behavioral) with alcohol addiction in a sample of adult alcohol-dependent patients and to test their current attentional skills (behavioral and cognitive). Methods: Thirty-two adult alcohol-dependent patients were examined for ADHD using a semi-structured interview and the Mini Mental State Examination to evaluate attention and inhibition functions. Brown ADD Scales were used to specifically examine ADHD syndrome. Patients were compared with thirty matched control participants selected from healthy population in few measures of attentional control and working memory. Results: 50% of patients showed evidence of primary ADHD symptoms: specifically, 28.12% showed criteria for ADHD highly probable, 12.50% for ADHD probable but not certain and 9.38% for ADHD possible but not likely. Patients also revealed several deficits in the selective visual attention, interference control and verbal working memory compared to the control group. Conclusions: These results revealed that adult alcohol-dependent patients had retrospectively high comorbidity with ADHD and significant current deficits of the executive functions. These findings suggest the importance of early diagnosis and treatment of ADHD in order to prevent the development of alcohol dependence.
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European Clinical Laboratory, Molecular and Pathological (ECMP) criteria for prefibrotic JAK2V617F-Thrombocythemia and Polycythemia Vera versus MPL515- and CALR-Thrombocythemia and Myelofibrosis: From Dameshek to Michiels 1950-2018

Published on: 1st March, 2019

OCLC Number/Unique Identifier: 8056299472

The broad spectrum of heterozygous versus homozygous JAK2V617F mutated MPN consists ET, ET with early features of PV (prodromal PV), classical PV, masked PV, advanced PV and post-PV myelofibrosis. Combined use of bone marrow histology and increased erythrocyte counts above 5.8x1012/L can replace increased red cell mass at time of presentation as the pathognomonic clue for the correct diagnosis of hetero/homozygous or homozygous mutated PV. Erythrocyte counts are in the normal range below 5.8x1012/L in heterozygous JAK2V617F mutated ET and prodromal PV but above 5.8x1012/L in heterozygous-homozygous or homozygous mutated PV. The bone marrow cellularity and morphology in pre-fibrotic ET, prodromal PV and PV carrying the JAK2V617F mutation are overlapping showing clustered increase of large mature pleomorphic megakaryocytes (M) with no increase of cellularity (<60%) in ET. The bone marrow is hypercellular (60%-80%) due to increased erythropoiesis megakaryopoiesis (EM) in prodromal and classical PV and trilinear hypercellular (80%-100% due increased megakaryopoiesis, erythropoiesis and granulopoiesis (EMG) in advanced PV and masked PV. Bone marrow cellularity ranging from normal (<60%) in ET to increased erythropoiesis (EM) in prodromal PV to hypercellular (80-100%) in advanced PV and masked PV largely depends on increasing JAK2V617F mutation load from low to high on top of other biological MPN variables like constitutional symptoms during long-term follow-up. MPL515 mutated ET is featured by an increase of clustered small and giant megakaryocytes with hyper-lobulated staghorn-like nuclei in a normal cellular bone marrow. The third entity of pronounced JAK2/MPL wild type ET associated with primary megakaryocytic granulocytic myeloproliferation (PMGM) without PV features proved to be caused by calreticulin (CALR) mutation. CALR mutated thrombocythemia is characterized by dual proliferation of megakaryocytic and granulocytic bone marrow proliferation of dense clustered large to giant immature dysmorphic megakaryocytes with bulky (bulbous) hyperchromatic nuclei, which are not seen in MPL515-mutated Thrombocythemia and JAK2V617F-Thrombocythemia, prodromal PV and classical PV. 
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Spinal muscular atrophy counteracted by Agrin biological NT-1654

Published on: 20th April, 2018

OCLC Number/Unique Identifier: 7671782483

Spinal muscular atrophy (SMA) is a genetic and gravely disease, portrayed by motor neuron (MN) death, thereby leading to progressive and accelerating muscle fragility, respiratory collapse, and, in the most severe cases, it even pave the way to death. At the neuromuscular junction (NMJ), abnormally have been reported in SMA, including neurofilament (NF) aggregation at presynaptic terminals, immature and smaller endplates, lowered transmitter release, and, eventually, muscle denervation. In this review the role of Agrin in SMA is studied. This review highlights the antagonizing role of Agrin in SMA
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Primary myelofibrosis is not primary anymore since the discovery of MPL515 and CALR mutations as driver causes of mono-linear megakaryocytic and dual megakaryocytic granulocytic myeloproliferation and secondary myelofibrosis

Published on: 15th April, 2019

OCLC Number/Unique Identifier: 8164054496

Primary myelofibrosis (PMF) is a distinct clinicopathological myeloproliferatve disease (MPD) not preceded by any other MPD ET, PV, CML,... Combined use of bone marrow histology and increased erythrocyte counts above 5.8x1012/L can replace increased red cell mass at time of presentation as the pathognomonic clue for the correct diagnosis of hetero/homozygous or homozygous mutated PV. Erythrocyte counts are in the normal range below 5.8x1012/L in heterozygous JAK2V617F mutated ET and prodromal PV but above 5.8x1012/L in heterozygous-homozygous or homozygous mutated PV. The bone marrow cellularity and morphology in pre-fibrotic ET, prodromal PV and PV carrying the JAK2V617F mutation are overlapping showing clustered increase of large mature pleomorphic megakaryocytes (M) with no increase of cellularity (<60%) in ET. The bone marrow is hypercellular (60%-80%) due to increased erythropoiesis megakaryopoiesis (EM) in prodromal and classical PV and trilinear hypercellular (80%-100% due increased megakaryopoiesis, erythropoiesis and granulopoiesis (EMG) in advanced PV and masked PV. Bone marrow cellularity ranging from normal (<60%) in ET to increased erythropoiesis (EM) in prodromal PV to hypercellular (80-100%) in advanced PV and masked PV largely depends on increasing JAK2V617F mutation load from low to high on top of other biological MPN variables like constitutional symptoms during long-term follow-up. MPL515 mutated ET is featured by an increase of clustered small and giant megakaryocytes with hyper-lobulated staghorn-like nuclei in a normal cellular bone marrow. The third entity of pronounced JAK2/MPL wild type ET associated with primary megakaryocytic granulocytic myeloproliferation (PMGM) without PV features proved to be caused by calreticulin (CALR) mutation. CALR mutated thrombocythemia is characterized by dual proliferation of megakaryocytic and granulocytic bone marrow proliferation of dense clustered large to giant immature dysmorphic megakaryocytes with bulky (bulbous) hyperchromatic nuclei, which are not seen in MPL515-mutated Thrombocythemia and JAK2V617F-Thrombocythemia, prodromal PV and classical PV. 
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