The disruption of proteostasis in neurodegenerative diseases

Cells count on surveillance systems to monitor and protect the cellular proteome which, besides being highly heterogeneous, is constantly being challenged by intrinsic and environmental factors. In this context, the proteostasis network (PN) is essential to achieve a stable and functional proteome. Disruption of the PN is associated with aging and can lead to and/or potentiate the occurrence of many neurodegenerative diseases (ND). This not only emphasizes the importance of the PN in health span and aging but also how its modulation can be a potential target for intervention and treatment of human diseases.info:eu-repo/semantics/publishedVersio

Aspectos epidemiológicos de pacientes com lesões ungueais e cutâneas causadas por Scytalidium spp Epidemiological aspects of patients with ungual and cutaneous lesions caused by Scytalidium spp

FUNDAMENTO: As dermatomicoses causadas por fungos filamentosos não dermatófitos são infecções raras, exceto as onicomicoses, cuja prevalência vem crescendo nos últimos anos. Dentre esses agentes etiológicos destacam-se o Scytalidium dimidiatum e o S. hyalinum, fungos emergentes responsáveis por micoses em unhas e pele. OBJETIVO: Investigar as características epidemiológicas das onicomicoses e micoses de outras localizações causadas pelos fungos do gênero Scytalidium, utilizando-se como parâmetros sexo, idade e localizações das lesões. MÉTODOS: Avaliaram-se 81 amostras com cultura positiva para o gênero em estudo, oriundas de 74 pacientes encaminhados ao Laboratório de Investigação em Dermatologia (ID) situado na cidade do Rio de Janeiro (RJ), no período de 1997 a 2006. As amostras foram submetidas a confirmação diagnóstica por exame direto e cultura. RESULTADOS: A prevalência de onicomicoses por Scytalidium spp. foi de 0,87%, entre as idades de 41 e 60 anos (48,64%). Em relação à localização das lesões, os pés foram mais acometidos (91,36%), com predomínio do hálux esquerdo. No exame direto, as estruturas mais encontradas foram hifas hialinas; na cultura, a espécie S. dimidiatum foi a mais frequente. CONCLUSÃO: As onicomicoses por Scytalidium spp. são raras e o S. dimidiatum foi a espécie mais isolada neste laboratório no período em estudo.<br>BACKGROUND: Dermatomycoses caused by non-dermatophyte filamentous fungi are rare infections, except for onychomycosis, whose prevalence has increased over the past few years. Among these etiologic agents, we highlight Scytalidium dimidiatum and S. hyalinum, emergent fungi that cause mycoses that affect the nails and skin. OBJECTIVE: To investigate the characteristics of onychomycosis and other mycoses caused by the fungi Scytalidium spp, using sex, age and site of infection as parameters. METHODS: Eighty-one samples were evaluated showing positive culture for Scytalidium spp, obtained from 74 patients referred to the Laboratory of Investigation in Dermatology (ID) located in the city of Rio de Janeiro, RJ, between 1997 and 2006. The samples were submitted to diagnostic confirmation through direct exam and culture. RESULTS: The prevalence of onychomycosis caused by Scytalidium spp. was of 0,87%. The most prevalent age was between 41-60 years (48.64%). Regarding the site of infection, the feet (91.36%) were most affected, with predominance of the left hallux. Hyaline hyphae were the most common structures in direct examination and the species S. dimidiatum was the most frequent in culture. CONCLUSION: Onychomycosis caused by Scytalidium spp. is rare and S. dimidiatum was the most isolated species in this laboratory during the period of the study

MR of the kidneys, liver, and spleen in paroxysmal nocturnal hemoglobinuria

The magnetic resonance (MR) findings in the liver, kidneys, and spleen in eight patients with paroxysmal nocturnal hemoglobinuria (PNH) were retrospectively reviewed to determine whether characteristic features could be demonstrated. Eight patients underwent abdominal MR examinations by gradient echo sequences (seven patients), spin-echo sequences (seven patients), and inversion recovery (one patient). Signal intensities of the kidneys, liver, and spleen were visually evaluated. Autopsy and liver biopsy correlation were available in one case each. Renal signal intensity was decreased in all eight patients by either gradient-echo or T2-weighted sequences and in the single inversion recovery sequence. Hepatic signal intensity was decreased in three of eight patients on spin- and gradient-echo images. Splenic signal intensity was decreased in three of eight patients on spin- and gradient-echo images, and in two of these was manifest as focal low signal spots (Gamna-Gandy bodies). While the signal intensity in the renal cortex is typically decreased in patients with PNH, signal intensities in the liver and spleen are variable. Low signal intensity in the kidneys is due to hemosiderin deposition resulting from intravascular hemolysis, whereas low signal intensity in the liver or spleen may be due to either transfusion siderosis, or as a consequence of hepatic or portal venous thrombosis.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/48141/1/261_2004_Article_BF00203497.pd