Avaliação de risco em mioloma múltiplo: resultados preliminares do grupo brasileiro de estudos de mieloma

The Durie/Salmon staging system continues to be used worldwide in patients with multiple myeloma. However, in recent years, new systems have been proposed. The International Myeloma Working Group performed a retrospective study with 11,179 patients and proposed an "International Staging System" utilizing serum levels of â2 microglobulin and albumin. In addition, current research has focused on the usefulness of cytogenetic and molecular data as prognostic factors. These data suggest that these parameters are powerful discriminators of a poor prognostic group of myeloma patients. Indeed, these prognostic indexes have been utilized in clinical trials, with interesting and encouraging results.O esquema de Durie / Salmon continua a ser utilizado para estadiar os pacientes com mieloma múltiplo. Recentemente, um novo sistema mais simples e eficaz foi proposto. O "International Myeloma Working Group" realizou um estudo retrospectivo com 11.179 pacientes e a partir destes dados propôs a criação de um "International Staging System (ISS)" utilizando os níveis séricos de ß2 microglobulina e de albumina ao diagnóstico. Além do ISS a pesquisa está voltada para identificar alterações citogenéticas e moleculares que se correlacionem com o prognóstico no mieloma múltiplo. Estes fatores prognósticos têm sido utilizados para estratificar pacientes em ensaios clínicos com resultados promissores

Impact Factor: outdated artefact or stepping-stone to journal certification?

A review of Garfield's journal impact factor and its specific implementation as the Thomson Reuters Impact Factor reveals several weaknesses in this commonly-used indicator of journal standing. Key limitations include the mismatch between citing and cited documents, the deceptive display of three decimals that belies the real precision, and the absence of confidence intervals. These are minor issues that are easily amended and should be corrected, but more substantive improvements are needed. There are indications that the scientific community seeks and needs better certification of journal procedures to improve the quality of published science. Comprehensive certification of editorial and review procedures could help ensure adequate procedures to detect duplicate and fraudulent submissions.Comment: 25 pages, 12 figures, 6 table

Optimal NOR Networks for Self-Dual Functions

Coordinated Science Laboratory was formerly known as Control Systems LaboratoryJoint Services Electronics Program / DAAB-07-72-C-025

Spoken term detection ALBAYZIN 2014 evaluation: overview, systems, results, and discussion

The electronic version of this article is the complete one and can be found online at: http://dx.doi.org/10.1186/s13636-015-0063-8Spoken term detection (STD) aims at retrieving data from a speech repository given a textual representation of the search term. Nowadays, it is receiving much interest due to the large volume of multimedia information. STD differs from automatic speech recognition (ASR) in that ASR is interested in all the terms/words that appear in the speech data, whereas STD focuses on a selected list of search terms that must be detected within the speech data. This paper presents the systems submitted to the STD ALBAYZIN 2014 evaluation, held as a part of the ALBAYZIN 2014 evaluation campaign within the context of the IberSPEECH 2014 conference. This is the first STD evaluation that deals with Spanish language. The evaluation consists of retrieving the speech files that contain the search terms, indicating their start and end times within the appropriate speech file, along with a score value that reflects the confidence given to the detection of the search term. The evaluation is conducted on a Spanish spontaneous speech database, which comprises a set of talks from workshops and amounts to about 7 h of speech. We present the database, the evaluation metrics, the systems submitted to the evaluation, the results, and a detailed discussion. Four different research groups took part in the evaluation. Evaluation results show reasonable performance for moderate out-of-vocabulary term rate. This paper compares the systems submitted to the evaluation and makes a deep analysis based on some search term properties (term length, in-vocabulary/out-of-vocabulary terms, single-word/multi-word terms, and in-language/foreign terms).This work has been partly supported by project CMC-V2 (TEC2012-37585-C02-01) from the Spanish Ministry of Economy and Competitiveness. This research was also funded by the European Regional Development Fund, the Galician Regional Government (GRC2014/024, “Consolidation of Research Units: AtlantTIC Project” CN2012/160)

Concomitant essential thrombocythemia with jak2 v617f mutation in a patient with chronic myeloid leukemia with major molecular response with imatinib and long-term follow-up

The association of chronic myeloid leukemia (CML) with other myeloproliferative neoplasms (MPNs), in particular with the V617F mutation in the Janus kinase 2 (JAK2) gene, is very uncommon, and there are only a few cases reported in the literature. In the present study, the case of a 73-year-old man with CML and persistent thrombocytosis, is reported. The patient achieved a complete cytogenetic response and major molecular response (MR) with imatinib. The patient presented JAK2 V617F mutation, and bone marrow morphology was consistent with essential thrombocythemia. The patient was treated with imatinib and hydroxyurea to control the platelet count, and maintains complete MR with imatinib upon 10 years of follow-up. Although rare, the association of breakpoint cluster region-Abelson rearrangement and JAK2 V617F mutation should be investigated in patients with MPN, since both genetic anomalies may be present at diagnosis or may emerge during treatment, and require different therapeutic approaches.The association of chronic myeloid leukemia (CML) with other myeloproliferative neoplasms (MPNs), in particular with the V617F mutation in the Janus kinase 2 (JAK2) gene, is very uncommon, and there are only a few cases reported in the literature. In the121485487sem informaçãosem informaçã

Fractal dimension of chromatin is an independent prognostic factor for survival in melanoma

<p>Abstract</p> <p>Background</p> <p>Prognostic factors in malignant melanoma are currently based on clinical data and morphologic examination. Other prognostic features, however, which are not yet used in daily practice, might add important information and thus improve prognosis, treatment, and survival. Therefore a search for new markers is desirable. Previous studies have demonstrated that fractal characteristics of nuclear chromatin are of prognostic importance in neoplasias. We have therefore investigated whether the fractal dimension of nuclear chromatin measured in routine histological preparations of malignant melanomas could be a prognostic factor for survival.</p> <p>Methods</p> <p>We examined 71 primary superficial spreading cutaneous melanoma specimens (thickness ≥ 1 mm) from patients with a minimum follow up of 5 years. Nuclear area, form factor and fractal dimension of chromatin texture were obtained from digitalized images of hematoxylin-eosin stained tissue micro array sections. Clark's level, tumor thickness and mitotic rate were also determined.</p> <p>Results</p> <p>The median follow-up was 104 months. Tumor thickness, Clark's level, mitotic rate, nuclear area and fractal dimension were significant risk factors in univariate Cox regressions. In the multivariate Cox regression, stratified for the presence or absence of metastases at diagnosis, only the Clark level and fractal dimension of the nuclear chromatin were included as independent prognostic factors in the final regression model.</p> <p>Conclusion</p> <p>In general, a more aggressive behaviour is usually found in genetically unstable neoplasias with a higher number of genetic or epigenetic changes, which on the other hand, provoke a more complex chromatin rearrangement. The increased nuclear fractal dimension found in the more aggressive melanomas is the mathematical equivalent of a higher complexity of the chromatin architecture. So, there is strong evidence that the fractal dimension of the nuclear chromatin texture is a new and promising variable in prognostic models of malignant melanomas.</p

Иммуногистохимическая детекция каппа-опиоидных рецепторов в коже человека

The imbalance of p- and kappa-opioid receptors in the skin or central nervous system is currently deemed to be one of the reasons of chronic pruritus. A number of studies demonstrated a positive effect of system agonists of kappa-opioid receptors in the treatment of uremic pruritus, nodular pruritus, paraneoplastic and cholestatic pruritus. This research demonstrates an expression of kappa-opioid receptors in human skin (basal keratinocytes, dendritic cells, epidermal melanocytes and fibroblasts of the upper dermis) detected with the use of different immunochemistry methods.В качестве одной из причин хронического зуда в настоящее время предполагается дисбаланс системы мю- и каппа-опиоидных рецепторов в коже или в центральной нервной системе. В нескольких исследованиях продемонстрирован положительный эффект системных агонистов каппа-опиоидных рецепторов в лечении уремического зуда, узловатой почесухи, паранеопластического и холестатического зуда. В настоящей работе демонстрируется экспрессия каппа-опиоидных рецепторов в коже человека (базальных кератиноцитах, дендритных клетках, меланоцитах эпидермиса и фибробластах сосочкового слоя дермы), определенная с помощью различных иммуногистохимических методик

Quality of life and clinical characteristics of self-improving congenital ichthyosis within the disease spectrum of autosomal recessive congenital ichthyosis

Background Autosomal-recessive congenital ichthyosis (ARCI) is a heterogeneous group of ichthyoses presenting at birth. Self-improving congenital ichthyosis (SICI) is a subtype of ARCI and is diagnosed when skin condition improves remarkably (within years) after birth. So far, there are sparse data on SICI and quality of life (QoL) in this ARCI subtype. This study aims to further delineate the clinical spectrum of SICI as a rather unique subtype of ARCI. Objectives This prospective study included 78 patients (median age: 15 years) with ARCI who were subdivided in SICI (n = 18) and non-SICI patients (nSICI, n = 60) by their ARCI phenotype. Methods Quality of life (QoL) was assessed using the (Children's) Dermatology Life Quality Index. Statistical analysis was performed with chi-squared and t-Tests. Results The genetically confirmed SICI patients presented causative mutations in the following genes: ALOXE3 (8/16; 50.0%), ALOX12B (6/16; 37.5%), PNPLA1 (1/16; 6.3%) and CYP4F22 (1/16; 6.3%). Hypo-/anhidrosis and insufficient vitamin D levels (<30 ng/mL) were often seen in SICI patients. Brachydactyly (a shortening of the 4th and 5th fingers) was statistically more frequent in SICI (P = 0.023) than in nSICI patients. A kink of the ear's helix was seen in half of the SICI patients and tends to occur more frequently in patients with ALOX12B mutations (P = 0.005). QoL was less impaired in patients under the age of 16, regardless of ARCI type. Conclusions SICI is an underestimated, milder clinical variant of ARCI including distinct features such as brachydactyly and kinking of the ears. Clinical experts should be aware of these features when seeing neonates with a collodion membrane. SICI patients should be regularly checked for clinical parameters such as hypo-/anhidrosis or vitamin D levels and monitored for changes in quality of life