Nasal glioma- a diagnostic challenge

Summary  In this unusual case there was a difficulty in diagnosis on the basis of histopathological reporting. On the grounds of clinical suspiscion one has to try to find out the proper diagnosis by advanced diagnostic methods like immunohistochemistry.     

Catheter ablation of the atrioventricular node using radiofrequency energy

Background: Catheter ablation of the atrioventricular (AV) junction using stored direct current (DC) energy from a standard DC Cardioverter defibrillator was first reported in 1982. Since then many patients have been treated using this procedure for refractory supraventricular arrhythmias, usually atrial fibrillation and flutter. Undesirable thermal effects such as barotrauma and arcing are largely responsible for complications associated with the use of DC energy. This report details our experience of catheter ablation of the AV junction using radiofrequency (RF) energy in a series of 30 consecutive patients. Methods: RF ablations were performed using steerable Mansfield (Webster Laboratories) 4 mm tipped electrodes and locally assembled RF energy delivery system. Results: The procedure was successful in 27/30 (90%) patients using RF energy, while three patients required DC energy to achieve successful AV junction ablation. General anaesthesia was required in nine patients, six of whom required this for cardioversion to sinus rhythm so that an adequate His Bundle spike could be recorded and three for DC ablation. Dual chamber permanent pacemakers with automatic mode switching were implanted in four patients who had paroxysmal atrial fibrillation or flutter and the remainder had ventricular rate responsive pacemakers. Conclusions: In patients with drug refractory paroxysmal atrial fibrillation and flutter and in patients with established atrial fibrillation where control of the ventricular rate is difficult, catheter ablation of the AV junction using RF energy is a safe and effective procedure with a high success rate.     

Ultrasensitive and bifunctional ZnO nanoplates for an oxidative electrochemical and chemical sensor of NO2: implications towards environmental monitoring of the nitrite reaction

Herein, we focused on the one pot synthesis of ZnO nanoplates (NP edge thickness of ∼100 nm) using a chemical emulsion approach for chemical (direct) and electrochemical (indirect) determination of NO₂. The structural and morphological elucidation of the as-synthesized ZnO NPs was carried out by X-ray diffraction (XRD), scanning electron microscopy (SEM), energy dispersive analysis of X-ray (EDAX), thermogravimetric analysis (TGA) and BET-surface area measurements. The XRD studies of the as-synthesised NPs reveal that ZnO NPs have a Wurtzite type crystal structure with a crystallite size of ∼100 nm. Such ZnO NPs were found to be highly sensitive to NO₂ gas at an operating temperature of 200 °C. Electrocatalytic abilities of these ZnO NPs towards NO₂/NO₂⁻ were verified through cyclic voltammetry (CV) and linear sweep voltammetry (LSV) using aqueous 1 mM NO₂⁻ (nitrite) in phosphate buffer (pH 7) solution. The results revealed enhanced activity at an onset potential of 0.60 V vs. RCE, achieved at a current density of 0.14 mA cm⁻². These ZnO NPs show selective NO₂ detection in the presence of other reactive species including CO, SO₂, CH₃OH and Cl₂. These obtained results show that this chemical route is a low cost and promising method for ZnO NPs synthesis and recommend further exploration into its applicability towards tunable electrochemical as well as solid state gas sensing of other toxic gases.

Herein, we focused on the one pot synthesis of ZnO nanoplates (NP edge thickness of ∼100 nm) using a chemical emulsion approach for chemical (direct) and electrochemical (indirect) determination of NO₂.      

Initial and long-term results of percutaneous transluminal balloon angioplasty for chronic total occlusions: an analysis of 184 procedures

Background: Coronary angioplasty, although of proven use in partial occlusion, has not been shown to be of similar benefit in chronic total occlusion. Aims: To assess the utility of coronary angioplasty in chronically totally occluded vessels in patients undergoing angioplasty and to determine the success of TIMI-I flow before angioplasty compared to those patients with TIMI-O flow. Methods: A group of 178 consecutive patients (from 1984 to 1992), who underwent angioplasty of a chronic occlusion, were analysed. There were 136 males and 42 females with a mean age of 56.9 years. Results: Initial technical success was achieved in 65%. Patients with TIMI-I flow before angioplasty had a higher chance of success (700%) compared to those with TIMI-O flow (53%), p < 0.04. During hospitalisation six patients suffered myocardial infarction (MI), two required surgery and one patient died. During a mean follow-up of 2.8 years the overall survival rate was 95% for the group as a whole. Freedom from coronary surgery was significantly greater in patients with successful angioplasty (93%) than those without (66%, p < 0.002). The above two populations also showed a significant difference in the incidence of angina (35%vs 56%, p < 0.0003). However, the incidence of MI (6%vs 5%, p > O.5) and cardiac survival (98%vs 94%, p > 0.l) did not differ significantly in the two groups. Restenosis occurred in 63% of the 95 patients (82%) who returned for follow-up angiography. Eighteen of the 59 patients (28%) with restenosis had a reocclusion. Conclusion: The success rate for angioplasty of chronic total occlusions is acceptable. Long-term clinical benefit in patients with successful angioplasty is suggested by the high freedom from angina and the lesser need for coronary surgery. No major impact on either the incidence of MI or cardiac survival was noted when patients who had coronary surgery were included, although it must be emphasised that the sample size in this study was insufficient to detect a difference in these outcome variables.     

Cytoplasmic RNA quality control failure engages mTORC1-mediated autoinflammatory disease

Inborn errors of nucleic acid metabolism often cause aberrant activation of nucleic acid sensing pathways, leading to autoimmune or autoinflammatory diseases. The SKIV2L RNA exosome is cytoplasmic RNA degradation machinery that was thought to be essential for preventing the self-RNA–mediated interferon (IFN) response. Here, we demonstrate the physiological function of SKIV2L in mammals. We found that Skiv2l deficiency in mice disrupted epidermal and T cell homeostasis in a cell-intrinsic manner independently of IFN. Skiv2l-deficient mice developed skin inflammation and hair abnormality, which were also observed in a SKIV2L-deficient patient. Epidermis-specific deletion of Skiv2l caused hyperproliferation of keratinocytes and disrupted epidermal stratification, leading to impaired skin barrier with no appreciable IFN activation. Moreover, Skiv2l-deficient T cells were chronically hyperactivated and these T cells attacked lesional skin as well as hair follicles. Mechanistically, SKIV2L loss activated the mTORC1 pathway in both keratinocytes and T cells. Both systemic and topical rapamycin treatment of Skiv2l-deficient mice ameliorated epidermal hyperplasia and skin inflammation. Together, we demonstrate that mTORC1, a classical nutrient sensor, also senses cytoplasmic RNA quality control failure and drives autoinflammatory disease. We also propose SKIV2L-associated trichohepatoenteric syndrome (THES) as a new mTORopathy for which sirolimus may be a promising therapy.     

Acute Confusional Migraine May Be a Presenting Feature of CADASIL

Objective.— Characterize the phenomenon of acute confusional migraine (ACM) among Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) patients and emphasize the possibility of CADASIL in adults with ACM.
Background.— ACM, well described in children, has rarely been reported in adults. Although 30-60% of CADASIL patients have migraine, acute confusional state during migraine has not been described. We describe 7 patients with ACM that complicated up to 50% of the migraine episodes.
Design/Methods.— Detailed neurologic evaluation was performed in 20 CADASIL patients; International Classification of Headache Disorders 2nd edition criteria were used to diagnose migraine.
Results.— The mean age was 51 years. Fourteen patients reported headache and 11 met the criteria for migraine (mean age of onset 25). Seven patients experienced concomitant confusion, within 3 years of migraine onset. Confusion occurred either abruptly or insidiously, at the onset of aura or headache, lasting for 2-48 hours, and ending abruptly. These episodes were stereotypic, characterized by disorientation with agitation, and retrograde amnesia for the episodes. Patients reported disorientation to time and place, inability to recognize friends and relatives, difficulty with finding directions home, fear of getting lost, inability to analyze traffic lights or tell time. Patients reliably predicted the episodes and felt the need to seek a safe place for protection. Severity of the episodes progressed, but a striking improvement occurred after the first stroke.
Conclusion.— ACM may be a presenting feature and important clue, enabling CADASIL to be recognized up to a decade or earlier than at present. Therefore, a brain MRI and/or testing for Notch3 mutations should be considered in adult patients with ACM.