How should eosinophilic cystitis be treated in patients with chronic granulomatous disease?

Chronic granulomatous disease (CGD) is a primary immunodeficiency resulting from the absence or malfunction of oxidative mechanism in phagocytic cells. The disease is due to a mutation in one of four genes that encode subunits of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Affected patients experience severe infections and granuloma formation due to exuberant inflammatory responses. Some evidence suggests that eosinophilic cystitis (EC) is included in the spectrum of inflammatory manifestations. EC is an inflammatory disease, rare in childhood, which may require different, nonstandardized therapeutic approaches, ranging from antihistamines to cyclosporine

Volcanology and magma geochemistry of the present day activity: constraints on the feeding system

Stromboli volcano is famous in the scientific literature for its persistent state of activity, which began about 1500 years ago and consists of continuous degassing and mild intermittent explosions (normal Strombolian activity). Rare lava emissions and sporadic more violent explosive episodes (paroxysms) also occur. Since its formation, the present-day activity has been dominated by the emission of two basaltic magmas, differing chiefly in their crystal and volatile contents, whose characteristics have remained constant until now. The normal Strombolian activity and lava effusions are fed by a crystal-rich, degassed magma, stored within the uppermost part of the plumbing system, whereas highly vesicular, crystal-poor light-colored pumices are produced during paroxysms testifying to the ascent of volatile-rich magma batches from deeper portions of the magmatic system. Mineralogical, geochemical, and isotopic data, together with data on the volatile contents of magmas, are presented here with the aim of discussing (1) the relationships between the different magma batches erupted at Stromboli, (2) the mechanisms of their crystallization and transfer, (3) the plumbing system and triggering mechanisms of Strombolian eruptions.Unpublished203.5. Geologia e storia dei sistemi vulcanici4.3. TTC - Scenari di pericolosità vulcanicareserve

The Impact of Specific Viruses on Clinical Outcome in Children Presenting with Acute Heart Failure

Abstract: The presence and type of viral genomes have been suggested as the main etiology for inflammatory dilated cardiomyopathy. Information on the clinical implication of this finding in a large population of children is lacking. We evaluated the prevalence, type, and clinical impact of specific viral genomes in endomyocardial biopsies (EMB) collected between 2001 and 2013 among 63 children admitted to our hospital for acute heart failure (median age 2.8 years). Viral genome was searched by polymerase chain reaction (PCR). Patients underwent a complete two-dimensional echocardiographic examination at hospital admission and at discharge and were followed-up for 10 years. Twenty-seven adverse events (7 deaths and 20 cardiac transplantations) occurred during the follow-up. Viral genome was amplified in 19/63 biopsies (35%); PVB19 was the most commonly isolated virus. Presence of specific viral genome was associated with a significant recovery in ejection fraction, compared to patients without viral evidence (p < 0.05). In Cox-regression analysis, higher survival rate was related to virus-positive biopsies (p < 0.05). When comparing long-term prognosis among different viral groups, a trend towards better prognosis was observed in the presence of isolated Parvovirus B19 (PVB19) (p = 0.07). In our series, presence of a virus-positive EMB (mainly PVB19) was associated with improvement over time in cardiac function and better long-term prognosis

Magma dynamics during the 2007 Stromboli eruption (Aeolian Islands. Italy): mineralogical, geochemical and isotopic data

After the 6 month-long effusive event of 2002-2003, a new lava effusion occurred at Stromboli between 27 February and 2 April 2007. Despite the different durations, approximately the same volume of magma was emitted in both eruptions, in the order of 107 m3 . A paroxysmal eruption occurred at the summit craters in both the 2002–2003 and 2007 episodes, during which a significant amount of low porphyritic (LP), volatile- rich magma was erupted. In both cases, the paroxysm did not interrupt the lava emission. Here, we present compositional data, including texture, mineralogy, chemistry and Sr and Nd isotope ratios of bulk-rock, groundmass and separated minerals of lavas erupted in 2007, together with chemistry and Sr and Nd isotope composition of the pumices emitted during the 15 March paroxysm. As a whole, the lavas have the same texture and chemistry that characterize the highly porphyritic (HP) products usually erupted at Stromboli during normal Strombolian activity and effusive events. Compared to the previous HP products, the 2007 lavas show minor but systematic mineralogical and isotopic variations which are consistent with a modest increase of the magma supply rate of the volcano. Compositional variations during the entire duration of the event are very modest. Glass chemistry changes in lavas erupted in the second half of March can be explained by theminormixing between the volatile-rich LPmagma rising through the shallowmagmatic systemduring the 15 March paroxysm and the degassed residing HP magma. A first conclusion of this study is that there is no compositional evidence supporting major changes in the magma dynamics of the volcano accompanying the effusive activity, as also suggested for the 2002–2003 event. The activity of Stromboli is controlled by a steady state feeding system in which refilling, mixing, degassing and crystallization at shallow level continuously operate,withmodest oscillations in themagma supply rate. Switching between normal Strombolian and effusive activity is related to periods of relatively more vigorous refilling of the shallow system, leading to progressive pressure increase in the upper conduits associated with only minor compositional variations in the erupted products

New Onset Cardiac Murmur and Exertional Dyspnea in an Apparently Healthy Child: A Rare Localization of Obstructive Myxoma in the Right Ventricle Outflow Tract without Pulmonary Embolization-A Case Report and Literature Review

Myxomas are slowly growing benign neoplasms which are rare in children. Up to 80% can be located in the left atrium and generate symptoms such as embolism, cardiac failure, fever and weight loss. Rarely, myxomas can be detected in the right ventricle outflow tract, causing arrhythmias, pulmonary emboli and sudden death. We report the case of a 13-year-old healthy child brought to the Emergency Department (ED) of the Children's Hospital Bambino Gesu, Rome, for recent dyspnea, chest pain on exertion and new onset cardiac murmur. Patient underwent medical examination and echocardiogram with the finding of a rounded and lobulated voluminous mass in the right ventricle outflow tract (RVOT) which caused severe obstruction. The contrast computed tomography (CT) scan confirmed the presence of a heterogeneously enhancing soft-tissue mass occupying the RVOT with no evidence of pulmonary embolization. The mass was surgically excised, and the pathologic examination confirmed our suspicion of myxoma. Our experience suggests that myxoma can have mild clinical symptoms, the presentation may be non-specific, and diagnosis can be a challenge Careful examination and a diagnostic imaging workup, primarily with the transthoracic echocardiogram, are needful to make a rapid differential diagnosis and to better manage surgical treatment and follow-up