A Case of Gorham-Stout Disease Treated with Fistula Closure by Transmeatal Approach

Gorham-Stout disease, a rare and intractable disease of unknown etiology, causes systemic bone lysis and replacement with lymphoid tissue. Here, we report a case of Gorham-Stout disease with cerebrospinal fluid leakage in a 16-year-old boy. The patient complained of nasal discharge, right ear obstruction, fever, and headache. A computed tomography scan of the head showed osteolysis around the right internal carotid artery, vestibule, and cochlea and osteolytic changes in the left parietal bone. It was suggested that the patient had bacterial meningitis owing to the leakage of cerebrospinal fluid from the fistula caused by the temporal bone osteolysis. He was treated with meropenem, and a transmeatal fistula closure and a bone biopsy of the left parietal bone were performed. Intraoperatively, osteolysis was observed on the promontory and around the internal carotid artery. The fistula was closed by dense filling and compression around the fistula, in the middle ear cavity, and in the external auditory canal. The symptoms disappeared after the surgery. Bone biopsy showed the presence of a lymphangioma, and Gorham-Stout disease was diagnosed. Prophylactic bisphosphonate therapy was initiated. A 4-year follow-up revealed no progression of the disease

GEM-TREND: a web tool for gene expression data mining toward relevant network discovery

<p>Abstract</p> <p>Background</p> <p>DNA microarray technology provides us with a first step toward the goal of uncovering gene functions on a genomic scale. In recent years, vast amounts of gene expression data have been collected, much of which are available in public databases, such as the Gene Expression Omnibus (GEO). To date, most researchers have been manually retrieving data from databases through web browsers using accession numbers (IDs) or keywords, but gene-expression patterns are not considered when retrieving such data. The Connectivity Map was recently introduced to compare gene expression data by introducing gene-expression signatures (represented by a set of genes with up- or down-regulated labels according to their biological states) and is available as a web tool for detecting similar gene-expression signatures from a limited data set (approximately 7,000 expression profiles representing 1,309 compounds). In order to support researchers to utilize the public gene expression data more effectively, we developed a web tool for finding similar gene expression data and generating its co-expression networks from a publicly available database.</p> <p>Results</p> <p>GEM-TREND, a web tool for searching gene expression data, allows users to search data from GEO using gene-expression signatures or gene expression ratio data as a query and retrieve gene expression data by comparing gene-expression pattern between the query and GEO gene expression data. The comparison methods are based on the nonparametric, rank-based pattern matching approach of Lamb et al. (Science 2006) with the additional calculation of statistical significance. The web tool was tested using gene expression ratio data randomly extracted from the GEO and with in-house microarray data, respectively. The results validated the ability of GEM-TREND to retrieve gene expression entries biologically related to a query from GEO. For further analysis, a network visualization interface is also provided, whereby genes and gene annotations are dynamically linked to external data repositories.</p> <p>Conclusion</p> <p>GEM-TREND was developed to retrieve gene expression data by comparing query gene-expression pattern with those of GEO gene expression data. It could be a very useful resource for finding similar gene expression profiles and constructing its gene co-expression networks from a publicly available database. GEM-TREND was designed to be user-friendly and is expected to support knowledge discovery. GEM-TREND is freely available at <url>http://cgs.pharm.kyoto-u.ac.jp/services/network</url>.</p

Mid-Ventricular Obstructive Hypertrophic Cardiomyopathy Associated with an Apical Aneurysm: Evaluation of Possible Causes of Aneurysm Formation

Mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHCM) is a rare type of cardiomyopathy, associated with apical aneurysm formation in some cases. We report a patient presenting with ventricular fibrillation, an ECG with an above normal ST segment, and elevated levels of cardiac enzymes but normal coronary arteries. Left ventriculography revealed a left ventricular obstruction without apical aneurysm. There was a significant pressure gradient between the apical and basal sites of the left ventricle. Cine magnetic resonance imaging (MRI), performed on the 10th hospital day, showed asymmetric septal hypertrophy, mid-ventricular obstruction, and an apical aneurysm with a thrombus. The first evaluation by contrast-enhanced imaging showed a subendocardial perfusion defect and delayed enhancement. It was speculated that the intraventricular pressure gradient, due to mid-ventricular obstruction, triggered myocardial infarction, which subsequently resulted in apical aneurysm formation

Electrical storm after cardiac resynchronization therapy in a patient with nonischemic cardiomyopathy: Signal-averaged vector-projected 187-channel electrocardiogram-based risk stratification for lethal arrhythmia

AbstractWe describe treatment of atrial flutter and electrical storm presenting as incessant ventricular tachycardia (VT) after implantation of a cardiac resynchronization therapy defibrillator (CRT-D) in a patient with dilated cardiomyopathy. No prior arrhythmic event had occurred. Our treatment strategy, including amiodarone administration, was guided in part by signal-averaged vector-projected 187-channel electrocardiogram (SAVP-ECG)-based risk stratification for ventricular arrhythmia. Corrected recovery time (RTc) dispersion and Tpeak-end dispersion were used to evaluate transmural dispersion of repolarization. RTc and Tpeak-end dispersion increased during the period of electrical storm. Values were improved 2 years after CRT-D implantation, and the amiodarone was discontinued. The VT has not recurred despite discontinuation of the antiarrhythmic agent. SAVP-ECG-based risk stratification for ventricular arrhythmia proved useful for the management of antiarrhythmic therapy

Dynamics of laser-induced cavitation bubble during expansion over sharp-edge geometry submerged in liquid - an inside view by diffuse illumination

Laser ablation in liquids is growing in popularity for various applications including nanoparticle production, breakdown spectroscopy, and surface functionalization. When laser pulse ablates the solid target submerged in liquid, a cavitation bubble develops. In case of "finite" geometries of ablated solids, liquid dynamical phenomena can occur inside the bubble when the bubble overflows the surface edge. To observe this dynamics, we use diffuse illumination of a flashlamp in combination with a high-speed videography by exposure times down to 250 ns. The developed theoretical modelling and its comparison with the experimental observations clearly prove that this approach widens the observable area inside the bubble. We thereby use it to study the dynamics of laser-induced cavitation bubble during its expansion over a sharp-edge ("cliff-like" 90°) geometry submerged in water, ethanol, and polyethylene glycol 300. The samples are 17 mm wide stainless steel plates with thickness in the range of 0.025-2 mm. Bubbles are induced on the samples by 1064-nm laser pulses with pulse durations of 7-60 ns and pulse energies of 10-55 mJ. We observe formation of a fixed-type secondary cavity behind the edge where low-pressure area develops due to bubble-driven flow of the liquid. This occurs when the velocity of liquid overflow exceeds [app.] 20 m s-1. A re-entrant liquid injection with up to [app.] 40 m s-1 velocity may occur inside the bubble when the bubble overflows the edge of the sample. Formation and characteristics of the jet evidently depend on the relation between the breakdown-edge offset and the bubble energy, as well as the properties of the surrounding liquid. Higher viscosity of the liquid prevents the generation of the jet