Transcatheter closure of a fenestration in intracardiac Fontan circulation

We present a case of tricuspid atresia and the treatment that was used in a child now aged 12. The malformation was corrected at the age of five through intracardiac Fontan circulation. Later, the child developed a right-to-left shunt due to dehiscence in the intra-atrial conduit, causing significant desaturation. This was treated by transcatheter closure with a 10 mm Amplatzer device at 8 years of age. Currently the child is asymptomatic with no residual shunts, and the position of the device is normal. The percutaneous closure of Fontan fenestrations using the Amplatzer device is a therapeutic approach that provides good results. It results in normal oxygen saturation and has low morbidity. The technique is simple, available to most cardiac catheterization laboratories, safe, and offers a reasonable cost-benefit ratio. In addition, the design of the device is suitable for this type of defect

Using the Juvenile Arthritis Disease Activity Score based on erythrocyte sedimentation rate or C-reactive protein level: results from the Portuguese register

Our aims were to evaluate the correlation between Juvenile Arthritis Disease Activity Score 27-joint reduced count (JADAS27) with erythrocyte sedimentation rate (ESR) and JADAS27 with C-reactive protein (CRP) scores and to test the agreement of both scores on classifying each disease activity state. We also aimed at verifying the correlation of the 2 scores across juvenile idiopathic arthritis (JIA) categories and to check the correlation between JADAS27-ESR and clinical JADAS27 (JADAS27 without ESR)

Two new species of Phalangopsis Serville, 1831 (Orthoptera: Grylloidea: Phalangopsidae) from Brazilian Amazon Forest

We describe here two new species of the genus Phalangopsis Serville, 1831 from the Brazilian Amazon Forest. The male genitalia and the female copulatory papilla were described, and a combination of diagnostic characteristics was given to separate both new species from the other described species. The principal morphological characteristics of this genus were discussed.Aqui foram descritas duas espécies novas do gênero Phalangopsis Serville, 1831 da Floresta Amazônica brasileira. A genitália masculina e a papila copulatória feminina são descritas, bem como uma combinação de características diagnósticas para separar ambas as novas espécies das outras espécies descritas. As principais características morfológicas foram discutidas

Antimicrobial Peptides and Skin: A Paradigm of Translational Medicine

Antimicrobial peptides (AMPs) are small, cationic, amphiphilic peptides with broad-spectrum microbicidal activity against both bacteria and fungi. In mammals, AMPs form the first line of host defense against infections and generally play an important role as effector agents of the innate immune system. The AMP era was born more than 6 decades ago when the first cationic cyclic peptide antibiotics, namely polymyxins and tyrothricin, found their way into clinical use. Due to the good clinical experience in the treatment of, for example, infections of mucus membranes as well as the subsequent understanding of mode of action, AMPs are now considered for treatment of inflammatory skin diseases and for improving healing of infected wounds. Based on the preclinical findings, including pathobiochemistry and molecular medicine, targeted therapy strategies are developed and first results indicate that AMPs influence processes of diseased skin. Importantly, in contrast to other antibiotics, AMPs do not seem to propagate the development of antibiotic-resistant micro-organisms. Therefore, AMPs should be tested in clinical trials for their efficacy and tolerability in inflammatory skin diseases and chronic wounds. Apart from possible fields of application, these peptides appear suited as an example of the paradigm of translational medicine for skin diseases which is today seen as a `two-way road' - from bench to bedside and backwards from bedside to bench. Copyright (c) 2012 S. Karger AG, Base

GTPase regulator associated with the focal adhesion kinase (GRAF) transcript was down-regulated in patients with myeloid malignancies

<p>Abstract</p> <p>Background</p> <p>GTPase regulator associated with the focal adhesion kinase (<it>GRAF</it>), a putative tumor suppressor gene, is found inactivated in hematopoietic malignancies by either genetic or epigenetic abnormalities. However, the expression level of <it>GRAF </it>gene has not yet been studied in leukemia. The aim of this study was to investigate the expression level of <it>GRAF </it>gene in those patients with myeloid malignancies including acute myeloid leukemia (AML), myelodysplastic syndrome (MDS) and chronic myeloid leukemia (CML).</p> <p>Methods</p> <p>The expression levels of <it>GRAF </it>transcript were determined in 94 patients using real-time quantitative PCR (RQ-PCR). Clinical and laboratory data of these patients were collected and analyzed.</p> <p>Results</p> <p>The significantly decreased level of <it>GRAF </it>transcript was observed in three myeloid malignancies compared to controls. Within AML, there was no difference in the level of <it>GRAF </it>transcript among different FAB subtypes (<it>P </it>> 0.05). Difference was not observed in the amount of <it>GRAF </it>mRNA between CML at chronic phase and controls. As CML progressed, <it>GRAF </it>transcript significantly decreased. In MDS, three cases with 5q deletion had lower <it>GRAF </it>transcript than four without 5q deletion (median 0.76 vs 2.99) (<it>P </it>> 0.05).</p> <p>Conclusion</p> <p>our results demonstrate that the <it>GRAF </it>transcript is decreased in myeloid malignancies.</p