1,1,3,3-tetramethylguanidinium dihydrogenorthophosphate

In the title compound, C 5 H 14 N 3 + H 2 PO 4 ˇ , the cation has a central guanidinium fragment with a planar geometry, as expected for a central C sp 2 atom with a small charge delocalization along the three C–N bonds. The crystal packing is governed by hydrogen bonds so that the phosphate anions are linked head to tail, forming chains running parallel to the c direction. These chains in turn are interconnected by hydrogen bonds to intermediate tetramethylguanidinium cations forming hydrogen-bonded molecular layers stacked parallel to the bc crystal planesCICYT PB98-112

European Red List of Marine Fishes

The European Red List is a review of the conservation status of European species according to IUCN regional Red Listing guidelines. It identifies those species that are threatened with extinction at the regional level, so that appropriate conservation action can be taken to improve their status. This Red List publication summarises results for all described native European marine fishes.Postprin

Convergence among Non-Sister Dendritic Branches: An Activity-Controlled Mean to Strengthen Network Connectivity

The manner by which axons distribute synaptic connections along dendrites remains a fundamental unresolved issue in neuronal development and physiology. We found in vitro and in vivo indications that dendrites determine the density, location and strength of their synaptic inputs by controlling the distance of their branches from those of their neighbors. Such control occurs through collective branch convergence, a behavior promoted by AMPA and NMDA glutamate receptor activity. At hubs of convergence sites, the incidence of axo-dendritic contacts as well as clustering levels, pre- and post-synaptic protein content and secretion capacity of synaptic connections are higher than found elsewhere. This coupling between synaptic distribution and the pattern of dendritic overlapping results in ‘Economical Small World Network’, a network configuration that enables single axons to innervate multiple and remote dendrites using short wiring lengths. Thus, activity-mediated regulation of the proximity among dendritic branches serves to pattern and strengthen neuronal connectivity

Evaluation of appendicitis risk prediction models in adults with suspected appendicitis

Background Appendicitis is the most common general surgical emergency worldwide, but its diagnosis remains challenging. The aim of this study was to determine whether existing risk prediction models can reliably identify patients presenting to hospital in the UK with acute right iliac fossa (RIF) pain who are at low risk of appendicitis. Methods A systematic search was completed to identify all existing appendicitis risk prediction models. Models were validated using UK data from an international prospective cohort study that captured consecutive patients aged 16–45 years presenting to hospital with acute RIF in March to June 2017. The main outcome was best achievable model specificity (proportion of patients who did not have appendicitis correctly classified as low risk) whilst maintaining a failure rate below 5 per cent (proportion of patients identified as low risk who actually had appendicitis). Results Some 5345 patients across 154 UK hospitals were identified, of which two‐thirds (3613 of 5345, 67·6 per cent) were women. Women were more than twice as likely to undergo surgery with removal of a histologically normal appendix (272 of 964, 28·2 per cent) than men (120 of 993, 12·1 per cent) (relative risk 2·33, 95 per cent c.i. 1·92 to 2·84; P < 0·001). Of 15 validated risk prediction models, the Adult Appendicitis Score performed best (cut‐off score 8 or less, specificity 63·1 per cent, failure rate 3·7 per cent). The Appendicitis Inflammatory Response Score performed best for men (cut‐off score 2 or less, specificity 24·7 per cent, failure rate 2·4 per cent). Conclusion Women in the UK had a disproportionate risk of admission without surgical intervention and had high rates of normal appendicectomy. Risk prediction models to support shared decision‐making by identifying adults in the UK at low risk of appendicitis were identified

Complete multilineage CD4 expression defect associated with warts due to an inherited homozygous CD4 gene mutation

On behalf of the EuroFlow Consortium.Idiopathic T-CD4 lymphocytopenia (ICL) is a rare and heterogeneous syndrome characterized by opportunistic infections due to reduced CD4 T-lymphocytes (<300 cells/μl or <20% T-cells) in the absence of HIV infection and other primary causes of lymphopenia. Molecular testing of ICL has revealed defects in genes not specific to CD4 T-cells, with pleiotropic effects on other cell types. Here we report for the first time an absolute CD4 lymphocytopenia (<0.01 CD4+ T-cells/μl) due to an autosomal recessive CD4 gene mutation that completely abrogates CD4 protein expression on the surface membrane of T-cells, monocytes, and dendritic cells. A 45-year-old female born to consanguineous parents consulted because of exuberant, relapsing, and treatment-refractory warts on her hands and feet since the age of 10 years, in the absence of other recurrent infections or symptoms. Serological studies were negative for severe infections, including HIV 1/2, HTLV-1, and syphilis, but positive for CMV and EBV. Blood analysis showed the absence of CD4+ T-cells (<0.01%) with repeatedly increased counts of B-cells, naïve CD8+ T-lymphocytes, and particularly, CD4/CD8 double-negative (DN) TCRαβ+ TCRγδ− T-cells (30% of T-cells; 400 cells/μl). Flow cytometric staining of CD4 using monoclonal antibodies directed against five different epitopes, located in two different domains of the protein, confirmed no cell surface membrane or intracytoplasmic expression of CD4 on T-cells, monocytes, and dendritic cells but normal soluble CD4 plasma levels. DN T-cells showed a phenotypic and functional profile similar to normal CD4+ T-cells as regards expression of maturation markers, T-helper and T-regulatory chemokine receptors, TCRvβ repertoire, and in vitro cytokine production against polyclonal and antigen-specific stimuli. Sequencing of the CD4 gene revealed a homozygous (splicing) mutation affecting the last bp on intron 7–8, leading to deletion of the juxtamembrane and intracellular domains of the protein and complete abrogation of CD4 expression on the cell membrane. These findings support previous studies in CD4 KO mice suggesting that surrogate DN helper and regulatory T-cells capable of supporting antigen-specific immune responses are produced in the absence of CD4 signaling and point out the need for better understanding the role of CD4 on thymic selection and the immune response.EB was supported by a grant from the Junta de Castilla y León (Fondo Social Europeo, ORDEN EDU/346/2013, Valladolid, Spain). This work was supported by: the CB16/12/00400 grant (CIBERONC, Instituto de Salud Carlos III, Ministerio de Economía y Competitividad, Madrid, Spain and FONDOS FEDER), the FIS PI12/00905-FEDER grant (Fondo de Investigación Sanitaria of Instituto de Salud Carlos III, Madrid, Spain), and a grant from Fundación Mutua Madrileña (Madrid, Spain).Peer reviewe

Melanoma cutâneo: estudo prospectivo de 65 casos Cutaneous melanoma: prospective study of 65 cases

FUNDAMENTOS: A incidência e a mortalidade por melanoma cutâneo vêm aumentando em todo o mundo. Os registros brasileiros de bases populacionais não refletem precisamente a real dimensão do problema. OBJETIVOS: Estudo prospectivo de 65 casos de melanoma cutâneo observados no Hospital Universitário Clementino Fraga Filho no período de 1993 a 2003. MÉTODOS: Foram analisadas as variáveis idade, sexo, cor, localização, tipos clínico-histológicos e estadiamento. RESULTADOS: 64,7% na faixa etária de 40 a 69 anos, distribuição etária homogênea entre o sexo masculino (49,2%) e o sexo feminino (50,8%), predominância de brancos (83%), localização no tronco (35,3%), tipo clínico-histológico expansivo superficial (63%/30,7%) e relação de significância entre tipo acral localizado no pé em não brancos. Segundo o American Joint Committee on Cancer, em 2002, 22 casos (33,8%) no estádio IA, 14 (21,5%) melanomas in situ e um caso indeterminado. CONCLUSÕES: O melanoma cutâneo primário na amostra estudada mostrou padrões semelhantes aos classicamente reconhecidos e maior freqüência do estádio IA e melanoma in situ.<br>BACKGROUND: Incidence and mortality of cutaneous melanoma are increasing all over the world. The data base for the Brazilian population is still inadequate. OBJECTIVES: Prospective study of 65 cases seen at University Hospital Clementino Fraga Filho, from 1993 to 2003. METHODS: Patient's age, sex, ethnic group, anatomic site, clinical histological presentation and staging were analyzed. RESULTS: The case distribution was 64.7% aged 40 to 69 years, males (49.2%) and females (50.8%), majority white (83.1%), most lesions in the trunk (35.3%), more frequently of the clinical histological superficial spreading type (63%/30.7%) and significant relationship between foot acral type in non-whites. According to American Joint Committee on Cancer 2002 system, 22 cases (33.8%) in stage IA, 14 (21.5%) melanomas in situ, and one indeterminate case. CONCLUSIONS: Primary cutaneous melanoma in the present study has a similar pattern to other published series and higher frequency of stage IA and in situ melanomas