InAs/InP/InSb Nanowires as Low Capacitance n-n Heterojunction Diodes

Nanowire diodes have been realized by employing an axial heterojunction between InAs and InSb semiconductor materials. The broken-gap band alignment (type III) leads to a strong rectification effect when the current-voltage (I-V) characteristic is inspected at room temperature. The additional insertion of a narrow InP barrier reduces the thermionic contribution, which results in a net decrease of leakage current in the reverse bias with a corresponding enhanced rectification in terms of asymmetry in the I-V characteristics. The investigated diodes compare favorably with the ones realized with p-n heterostructured nanowires, making InAs/InP/InSb devices appealing candidates to be used as building blocks for nanowire-based ultrafast electronics and for the realization of photodetectors in the THz spectral range

Индекс стабилизации Фабри (FASTEX): инновационный инструмент для оценки клинической стабилизации при болезни Фабри

На сегодняшний день предложены 2 системы количественной оценки бремени гликогеноза с дефицитом α-галактозидазы: индекс оценки степени тяжести Майнца (MSSI) и система балльной оценки тяжести болезни Фабри (DS3). Сделана попытка разработать динамическую математическую модель FASTEX (от англ. FAbry STabilization indEX, индекс стабилизации Фабри) для оценки клинической стабильности состояния. Мультидисциплинарная группа экспертов по болезни Фабри впервые предложила новую шкалу оценки тяжести заболевания по предварительной оценке (от англ. raw score, RS), основанную на 3 доменах (домен нервной системы (боль, цереброваскулярные события), почечный домен (протеинурия, скорость клубочковой фильтрации), сердечный домен (параметры эхокардиографии, электрокардиографии и степень сердечной недостаточности по классификации Нью-Йоркской кардиологической ассоциации)) с небольшим числом пунктов в каждом из них и оценкой клинической стабильности во времени. RS протестирована на 28 пациентах (15 мужчин и 13 женщин) с классической формой болезни Фабри. Получена сильная корреляционная связь предложенной оценки RS и взвешенной оценки (от англ. weighted score, WS) с DS3 и MSSI (r2 = 0,914; 0,949; 0,910 и 0,938 соответственно). Для уточнения RS была рассчитана WS, выражаемая в процентах. WS была основана на относительной клинической значимости каждого пункта в пределах домена, при этом группа экспертов согласовывала присвоение разного веса клинического вреда конкретной системе органов. Для определения динамики тяжести заболевания RS была повторно определена через 1 год. Группа экспертов согласилась с пороговым ограничением в 20 % от исходного уровня в качестве клинической WS для определения клинической стабильности. Модель FASTEX показала хорошую корреляцию с клинической оценкой и клиническим изменением на протяжении времени у всех пациентов. 

Nervous system and Fabry disease, from symptoms to diagnosis: damage evaluation and follow-up in adult patients, enzyme replacement, and support therapy

The X-linked genetic Fabry disease causes multiorgan lesions due to intracellular storage of the substrate globotriaosylceramide. Neurological involvement ranges from painful, small fiber neuropathy to cerebrovascular disorders to multifocal aggressive forms. Disease identification through proper differential diagnosis and timely assessment of organ damage should guide a careful treatment planning. Mainstay treatment, include enzyme replacement and support therapy. Neurologists have a pivotal role in early instrumental and clinical detection of organ damage. A panel of experts has developed a set of consensus recommendations to guide the approach of neurologists to Fabry disease

Genetic Association and Altered Gene Expression of Mir-155 in Multiple Sclerosis Patients

Multiple sclerosis (MS) is a complex autoimmune disease of the central nervous system characterized by chronic inflammation, demyelination, and axonal damage. As microRNA (miRNA)-dependent alterations in gene expression in hematopoietic cells are critical for mounting an appropriate immune response, miRNA deregulation may result in defects in immune tolerance. In this frame, we sought to explore the possible involvement of miRNAs in MS pathogenesis by monitoring the differential expression of 22 immunity-related miRNAs in peripheral blood mononuclear cells of MS patients and healthy controls, by using a microbead-based technology. Three miRNAs resulted >2 folds up-regulated in MS vs controls, whereas none resulted down-regulated. Interestingly, the most up-regulated miRNA (mir-155; fold change = 3.30; P = 0.013) was previously reported to be up-regulated also in MS brain lesions. Mir-155 up-regulation was confirmed by qPCR experiments. The role of mir-155 in MS susceptibility was also investigated by genotyping four single nucleotide polymorphisms (SNPs) mapping in the mir-155 genomic region. A haplotype of three SNPs, corresponding to a 12-kb region encompassing the last exon of BIC (the B-cell Integration Cluster non-coding RNA, from which mir-155 is processed), resulted associated with the disease status (P = 0.035; OR = 1.36, 95% CI = 1.05–1.77), suggesting that this locus strongly deserves further investigations

Incentivizing the Dynamic Workforce: Learning Contracts in the Gig-Economy

In principal-agent models, a principal offers a contract to an agent to perform a certain task. The agent exerts a level of effort that maximizes her utility. The principal is oblivious to the agent's chosen level of effort, and conditions her wage only on possible outcomes. In this work, we consider a model in which the principal is unaware of the agent's utility and action space. She sequentially offers contracts to identical agents, and observes the resulting outcomes. We present an algorithm for learning the optimal contract under mild assumptions. We bound the number of samples needed for the principal obtain a contract that is within $\epsilon$ of her optimal net profit for every $\epsilon>0$

Natural history of motor neuron disease in adult onset GM2-gangliosidosis: a case report with 25 years of follow-up

An adult with Sandhoff disease presented with pure lower motor neuron phenotype. Twenty years later, he showed signs of upper motor neuron involvement. 25 years from the onset, his muscle weakness slightly worsened but he was fully independent in activities of daily living.GM2-gangliosidosis can manifest as a motor neuron disease with a slowly progressive course. The correct knowledge of the natural history can be really important to achieve the diagnosis, design new therapies and evaluate clinical trials

Growth and characterization of red-green-blue cathodoluminescent ceramic films

Gd2O3 and Y2O3 films, respectively, doped with Eu3+, Tb3+, and Tm3+ have been grown by the electron beam physical vapor codeposition technique on optically polished quartz substrates. The film samples have been doped at different concentrations by the corresponding rare-earth oxides. The concentration range explored is from 0.9% to 9.8% on 18 samples. For each film sample an extended characterization has been performed by thin film-x-ray diffraction, scanning electron microscopy, energy dispersion spectroscopy, cathodoluminescence spectroscopy, and color coordinate analysis. Y2O3 films display the most intense red-green-blue (RGB) emission and their film morphology and structure are more compact and crystalline with respect to Gd2O3 monoclinic films. Eu3+ and Tb3+ doped Y2O3 films grow oriented along the (222) direction. The ratios between the intensities of the electric dipole and magnetic dipole transitions have been also evaluated. The blue emission of Tm3+ doped Gd2O3 is lacking in the dopant concentration range from 1.6% to 7.6%, whereas it is present in Tm3+ doped Y2O3 films, at approximately the same dopant concentration range (1.9%-9.8%). Commission Internationale de l'Eclairage plot of the color coordinates of all the RGB film samples has been reported together with the RGB phosphor standard used in cathodic ray tube TV screens

Teaching NeuroImages: Brain MRI and DaT-SPECT imaging in adult GM1 gangliosidosis

[no abstract available

A case of Fabry disease with central nervous system (CNS) demyelinating lesions: a double trouble?

We present the case of a 36-year-old woman affected with Fabry disease (FD), with neuroradiologic and laboratory tests suggestive of a coexistent inflammatory demyelinating disease. Since the age of 23, she presented recurrent neurologic deficits, such as right limb paresthesias, diplopia, and right leg weakness. Magnetic resonance imaging revealed multiple demyelinating lesions in periventricular areas, corpus callosum, and spinal cord. Cerebrospinal fluid analysis showed the presence of oligoclonal bands, while visual-evoked potentials were delayed with preserved morphology. FD is usually considered as a differential diagnosis of multiple sclerosis, but we think that the best explanation of all pathological features in this case is the coexistence of the two diseases