1,888 research outputs found

    Investigations on nucleophilic layers made with a novel plasma jet technique

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    In this work a novel plasma jet technique is used for the deposition of nucleophilic films based on (3-aminopropyl)trimethoxysilane at atmospheric pressure. Film deposition was varied with regard to duty cycles and working distance. Spectral ellipsometry and chemical derivatization with 4-(trifluoromethyl)benzaldehyde using ATR- FTIR spectroscopy measurements were used to characterize the films. It was found that the layer thickness and the film composition are mainly influenced by the duty cycle

    One Hundred Cases of Localized Laryngeal Amyloidosis - Evidence for Future Management

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    Objective/Hypothesis: To update the current understanding of localized laryngeal amyloidosis by analyzing the NHS National Amyloidosis Database and to further clarify the important ongoing management issues. Study Design: Retrospective review, case series. Methods: Patients with laryngeal amyloid were identified from the database of the NHS National Amyloidosis Center, UCL, Royal Free Hospital, London between 2000 and 2017. Patient demographics and disease profile were collated, including the exact location of amyloid deposit, treatments if any, and progression of disease. Results: One hundred and three patients with localized laryngeal amyloid where identified from the database, with a mean age of 54 at diagnosis and female to male ratio of 54:49. Three patients were excluded from further analysis due to limited database information. The majority of amyloid was found in either the supraglottis (44) or glottis (53) but all the laryngeal subsites were involved. One-third of the patients (34) had amyloid in more than one laryngeal subsite. No patients were found to progress to systemic amyloid, but many progressed locally to other subsites or further down the LTB tree (29%). Three patients were successfully treated with radiotherapy after other modalities had failed. Conclusions: This is the largest case series reported to date of localized laryngeal amyloidosis. It highlights the high incidence of multifocal disease and the significant proportion of patients who progressed, not to systemic amyloidosis but to more extensive localized amyloid. We recommend that in all cases of laryngeal amyloid, patients should undergo a thorough assessment of the upper and lower airways and have ongoing surveillance for at least 15 years

    Gene expression becomes heterogeneous with age

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    Assembly Theory Explains and Quantifies the Emergence of Selection and Evolution

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    Since the time of Darwin, scientists have struggled to reconcile the evolution of biological forms in a universe determined by fixed laws. These laws underpin the origin of life, evolution, human culture and technology, as set by the boundary conditions of the universe, however these laws cannot predict the emergence of these things. By contrast evolutionary theory works in the opposite direction, indicating how selection can explain why some things exist and not others. To understand how open-ended forms can emerge in a forward-process from physics that does not include their design, a new approach to understand the non-biological to biological transition is necessary. Herein, we present a new theory, Assembly Theory (AT), which explains and quantifies the emergence of selection and evolution. In AT, the complexity of an individual observable object is measured by its Assembly Index (a), defined as the minimal number of steps needed to construct the object from basic building blocks. Combining a with the copy number defines a new quantity called Assembly which quantifies the amount of selection required to produce a given ensemble of objects. We investigate the internal structure and properties of assembly space and quantify the dynamics of undirected exploratory processes as compared to the directed processes that emerge from selection. The implementation of assembly theory allows the emergence of selection in physical systems to be quantified at any scale as the transition from undirected-discovery dynamics to a selected process within the assembly space. This yields a mechanism for the onset of selection and evolution and a formal approach to defining life. Because the assembly of an object is easily calculable and measurable it is possible to quantify a lower limit on the amount of selection and memory required to produce complexity uniquely linked to biology in the universe.Comment: 22 pages, 7 figure

    Senile Systemic Amyloidosis: Clinical Features at Presentation and Outcome

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    Background Cardiac amyloidosis is a fatal disease whose prognosis and treatment rely on identification of the amyloid type. In our aging population transthyretin amyloidosis (ATTRwt) is common and must be differentiated from other amyloid types. We report the clinical presentation, natural history, and prognostic features of ATTRwt compared with cardiac‐isolated AL amyloidosis and calculate the probability of disease diagnosis of ATTRwt from baseline factors. Methods and Results All patients with biopsy‐proven ATTRwt (102 cases) and isolated cardiac AL (36 cases) seen from 2002 to 2011 at the UK National Amyloidosis Center were included. Median survival from the onset of symptoms was 6.07 years in the ATTRwt group and 1.7 years in the AL group. Positive troponin, a pacemaker, and increasing New York Heart Association (NYHA) class were associated with worse survival in ATTRwt patients on univariate analysis. All patients with isolated cardiac AL and 24.1% of patients with ATTRwt had evidence of a plasma cell dyscrasia. Older age and lower N‐terminal pro‐B‐type natriuretic peptide (NT pro‐BNP) were factors significantly associated with ATTRwt. Patients aged 70 years and younger with an NT pro‐BNP <183 pmol/L were more likely to have ATTRwt, as were patients older than 70 years with an NT pro‐BNP <1420 pmol/L. Conclusions Factors at baseline associated with a worse outcome in ATTRwt are positive troponin T, a pacemaker, and NYHA class IV symptoms. The age of the patient at diagnosis and NT pro‐BNP level can aid in distinguishing ATTRwt from AL amyloidosis
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