Kawasaki disease in Sicily: clinical description and markers of disease severity

Background: Kawasaki disease (KD) is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin (IVIG) develop coronary artery lesions (CAL). Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL. Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity. Methods: We studied the clinical data of 70 KD Sicilian children (36 males: 51 %; 34 females: 49 %), analysed retrospectively, including: demographic and laboratory parameters; echocardiographic findings at diagnosis, at 2, 6 and 8 weeks, and at 1 year after the onset of the illness. Results: Forty-seven had Typical KD, three Atypical KD and twenty Incomplete KD. Age at the disease onset ranged from 0.1 to 8.9 years. IVIG were administered 5 \ub1 2 days after the fever started. Defervescence occurred 39 \ub1 26 hours after the first IVIG infusion. Fifty-six patients (80 %) received 1 dose of IVIG (responders); 14 patients (20 %) had a resistant KD, with persistent fever after the first IVIG dose (non responders). Ten (14 %) non responders responded to the second dose, 4 (5 %) responded to three doses; one needed treatment with high doses of steroids and Infliximab. Cardiac involvement was documented in twenty-two cases (eighteen with transient dilatation/ectasia, fifteen with aneurysms). Pericardial effusion, documented in eleven, was associated with coronaritis and aneurysms, and was present earlier than coronary involvement in seven. Hypoalbuminemia, D-dimer pre-IVIG, gamma-GT pre-IVIG showed a statistically significant direct correlation with IVIG doses, highlighting the role of these parameters as predictor markers of refractory disease. The persistence of elevated CRP, AST, ALT levels, a persistent hyponatremia and hypoalbuminemia after IVIG therapy, also had a statistical significant correlation with IVIG doses. Non responders showed higher levels of D-dimer and gamma-GT pre-IVIG, persistent high levels of D-dimer, CRP, AST, ALT, hypoalbuminemia and hyponatremia after IVIG. Conclusions: This is the first study on KD in Sicily. We suggest some laboratory parameters as predictive criteria for resistant KD. Patients who show early pericarditis need careful surveillance for coronary lesions

Systemic lupus erythematosus and bullous pemphigoid with dramatic response to dapsone

Objective: Unusual clinical course Background: Bullous pemphigoid is an autoimmune blistering disease, with relapses, isolated or associated with other autoimmune diseases such as systemic lupus erythematosus (SLE). Joint manifestations rapidly respond to small or moderate doses of corticosteroids, whereas skin manifestations usually respond to antimalarial drugs. Case Report: We describe the clinical case of an 11-year-old girl with SLE. She showed bullous skin lesions with arthralgia, mild proteinuria, resolved after steroid treatment. At the tapering of her prednisone dose, the patient had new skin lesions requiring an increased dose of prednisone. She started dapsone at the dosage of 1 mg/kg/day, maintaining low dose prednisone; this treatment was successfully followed by the dramatic disappearance of skin lesions and limb pain. Conclusions: Bullous skin lesions can represent the first clinical presentation of pediatric SLE and could influence the treatment and the outcome of these patients. This case showed an atypical course as both skin manifestations and arthritis promptly and persistently resolved with dapsone without the use of high-dose glucocorticoids. Only a few cases of patients with SLE associated with bullous pemphigoid have been reported in the literature, and very few in the pediatric population

INTEGRATED SURVEY AND SMART NAVIGATION OF COMPLEX TERRITORIAL MODELS

Abstract. The study examines the numerous medieval fortified sites that are in ruins throughout the Sicilian territory. Their landscape and historical value is significant, but they are unfortunately abandoned, not in stable conditions and often no metrically reliable graphic representation exists.The methodology we chose required a combined 3D survey campaign and data processing aimed at obtaining 2D and 3D drawings, as well as a virtual reality application to provide knowledge and virtual fruition of the sites.The entire experimental procedure was optimised to provide multi-scalar readings of the sites under investigation, that include their details, the location, territory, and landscape.The article presents the results of a case study carried out on the Maletto Castle in Sicily (Italy)

Vortex Structure in Superconducting Stripe States

The vortex structure in superconducting stripe states is studied according to the Bogoliubov-de Gennes theory on the two-dimensional Hubbard model with nearest-neighbor sites pairing interaction. The vortex is trapped at the outside region of the stripe line, where the superconductivity is weak. The superconducting coherence length along the stripe direction becomes long. There are no eminent low-energy electronic states even near the vortex core. These characters resemble the Josephson vortex in layered superconductors under a parallel field.Comment: LaTeX 5 pages (using jpsj macros) with 3 figure

Theory of vortex lattice effects on STM spectra in d-wave superconductors

Theory of scanning tunneling spectroscopy of low energy quasiparticle (QP) states in vortex lattices of d-wave superconductors is developed taking account of the effects caused by an extremely large extension of QP wavefunctions in the nodal directions and the band structure in the QP spectrum. The oscillatory structures in STM spectra, which correspond to van Hove singularities are analysed. Theoretical calculations carried out for finite temperatures and scattering rates are compared with recent experimental data for high temperature cuprates.Comment: 4 pages, 3 eps figures, M2S-HTSC-VI conference paper, using Elsevier style espcrc2.st

HYPERPLASIA OF THYMIC GLAND: LEFT VIDEO-ASSISTED THORACOSCOPIC APPROACH

Hyperplasia of thymic gland is a rare benign entity that should be considered in the differential diagnosis of anterior mediastinal masses in children and young adolescents. We report a case of a patient with a thymic mass, diagnosed occasionally for respiratory symptoms and treated by video-assisted thoracoscopic surgery. A previously healthy 10 years-old boy presented to our hospital for retrosternal pain and dyspnea with restriction to daily activities from four months. Diagnostic imaging was performed, including a chest x-ray and a magnetic resonance imaging, showing a large homogeneous anterosuperior mediastinal mass, more extended on the left side. The additional laboratory analysis, considered essential for differential diagnosis with myasthenia gravis and lymphoma, resulted negative. In view of these findings, our patient underwent to video assisted thoracoscopy with left-sided approach for a total resection of thymus and perithymic fat. The patient made an excellent recovery without postoperative complications and was discharged from the hospital four days later. Histopathological examination showed a normal thymic architecture like a true thymic hyperplasia. At follow up, chest x-ray was normal in absence of pleural and parenchimal alterations. Thoracoscopic thymectomy is a safe technique that allows to achieve the goal of early thymectomy with the advantages of less invasive procedure

Simple Ginzburg-Landau Theory for Vortices in a Crystal Lattice

We study the Ginzburg-Landau model with a nonlocal quartic term as a simple phenomenological model for superconductors in the presence of coupling between the vortex lattice and the underlying crystal lattice. In mean-field theory, our model is consistent with a general oblique vortex lattice ranging from a triangular lattice to a square lattice. This simple formulation enables us to study the effect of thermal fluctuations in the vortex liquid regime. We calculate the structure factor of the vortex liquid nonperturbatively and find Bragg-like peaks with four-fold symmetry appearing in the structure factor even though there is only a short-range crystalline order.Comment: Revised version with new title and additional results for the vortex liquid regime, to be published in Phys. Rev. Lett. 5 pages RevTeX, 1 figure include

Theory of vortex excitation imaging via an NMR relaxation measurement

The temperature dependence of the site-dependent nuclear spin relaxation time T_1 around vortices is studied in s-wave and d-wave superconductors.Reflecting low energy electronic excitations associated with the vortex core, temperature dependences deviate from those of the zero-field case, and T_1 becomes faster with approaching the vortex core. In the core region, T_1^{-1} has a new peak below T_c. The NMR study by the resonance field dependence may be a new method to prove the spatial resolved vortex core structure in various superconductors.Comment: 5 pages, 3 figure

A Frailty Index based on clinical data to quantify mortality risk in dogs

Frailty is defined as a decline in an organism's physiological reserves resulting in increased vulnerability to stressors. In humans, a single continuous variable, the so-called Frailty Index (FI), can be obtained by multidimensionally assessing the biological complexity of an ageing organism. Here, we evaluate this variability in dogs and compare it to the data available for humans. In dogs, there was a moderate correlation between age and the FI, and the distribution of the FI increased with age. Deficit accumulation was strongly related to mortality. The effect of age, when combined with the FI, was negligible. No sex-related differences were evident. The FI could be considered in epidemiological studies and/or experimental trials to account for the potential confounding effects of the health status of individual dogs. The age-related deficit accumulation reported in dogs is similar to that demonstrated in humans. Therefore, dogs might represent an excellent model for human aging studies

POTENZIALI EVOCATI VISIVI DA FLASH IN GEMELLI IN EPOCA NEONATALE. CORRELAZIONI CON LA ADEGUATEZZA DEL PESO CON LA ETA' GESTAZIONALE (AGA VS SGA)

Neonati AGA, SGA, PE