Case of bullous pemphigoid in a 28-year-old woman affected by tuberous sclerosis complex

[No abstract available

Una strana placca eritematosa parzialmente ulcerata

Presentiamo il caso di una donna di 38 anni affetta da sclerosi tuberosa ed in terapia immunosoppressiva dal 2006 per trapianto renale. La paziente presentava grave deficit cognitivo e di coordinazione motoria. All’esame obiettivo si rilevava la presenza di una placca eritematosa in regione pretibiale sinistra, parzialmente ulcerata, di cui la paziente riferiva comparsa e persistenza da circa 3 anni. Nel resto del corpo la cute si presentava integra eccetto alcune lesioni da grattamento. In passato erano state eseguite due biopsie cutanee per un miglior inquadramento diagnostico il cui referto istologico era risultato completamente aspecifico. La madre riferiva di aver notato la comparsa talvolta di alcune bolle sul corpo, che si rompevano e guarivano spontaneamente senza lasciare esiti cicatriziali. Nel sospetto di una patologia bollosa si eseguiva un prelievo ematico per immunofluorescenza indiretta e due biopsie cutanee per esame istologico e immunofluorescenza diretta. Al momento della consegna degli esiti degli esami, che deponevano per un quadro di pemfigoide bolloso, si osservava un’intensa area disepitelizzata nella regione pretibiale sinistra e due piccole bolle tese sulla gamba destra. Localmente si applicavano steroide topico sulle lesioni bollose e medicazione a base di idrofibra con argento sull’area pretibiale sinistra con risoluzione del quadro cutaneo

Rethinking neuroprotection in severe traumatic brain injury : toward bedside neuroprotection

Neuroprotection after traumatic brain injury (TBI) is an important goal pursued strenuously in the last 30 years. The acute cerebral injury triggers a cascade of biochemical events that may worsen the integrity, function, and connectivity of the brain cells and decrease the chance of functional recovery. A number of molecules acting against this deleterious cascade have been tested in the experimental setting, often with preliminary encouraging results. Unfortunately, clinical trials using those candidate neuroprotectants molecules have consistently produced disappointing results, highlighting the necessity of improving the research standards. Despite repeated failures in pharmacological neuroprotection, TBI treatment in neurointensive care units has achieved outcome improvement. It is likely that intensive treatment has contributed to this progress offering a different kind of neuroprotection, based on a careful prevention and limitations of intracranial and systemic threats. The natural course of acute brain damage, in fact, is often complicated by additional adverse events, like the development of intracranial hypertension, brain hypoxia, or hypoperfusion. All these events may lead to additional brain damage and worsen outcome. An approach designed for early identification and prompt correction of insults may, therefore, limit brain damage and improve results

Current and emerging technologies for probing molecular signatures of traumatic brain injury

Traumatic brain injury (TBI) is understood as an interplay between the initial injury, subsequent secondary injuries, and a complex host response all of which are highly heterogeneous. An understanding of the underlying biology suggests a number of windows where mechanistically inspired interventions could be targeted. Unfortunately, biologically plausible therapies have to-date failed to translate into clinical practice. While a number of stereotypical pathways are now understood to be involved, current clinical characterization is too crude for it to be possible to characterize the biological phenotype in a truly mechanistically meaningful way. In this review, we examine current and emerging technologies for fuller biochemical characterization by the simultaneous measurement of multiple, diverse biomarkers. We describe how clinically available tech- niques such as cerebral microdialysis can be leveraged to give mechanistic insights into TBI pathobiology and how multiplex proteomic and metabolomic techniques can give a more complete description of the underlying biology. We also describe spatially resolved label-free multiplex techniques capable of probing structural differences in chemical signatures. Finally, we touch on the bioinformatics challenges that result from the acquisition of such large amounts of chemical data in the search for a more mechanistically complete description of the TBI phenotype.GV received a fellowship by “Associazione Amici del Mario Negri.

Uncertainty estimations for moment tensor inversions: the issue of the 2012 May 20 Emilia earthquake

This article has been accepted for publication in Geophysical Journal Internationa ©: 2016 Published by Oxford University Press on behalf of the Royal Astronomical Society. All rights reserved.Seismic moment tensor is one of the most important source parameters defining the earthquake dimension and style of the activated fault. Geoscientists ordinarily use moment tensor cata- logues, however, few attempts have been done to assess possible impacts of moment magnitude uncertainties upon their analysis. The 2012 May 20 Emilia main shock is a representative event since it is defined in literature with a moment magnitude value (Mw) spanning between 5.63 and 6.12. A variability of ∼0.5 units in magnitude leads to a controversial knowledge of the real size of the event and reveals how the solutions could be poorly constrained. In this work, we investigate the stability of the moment tensor solution for this earthquake, studying the effect of five different 1-D velocity models, the number and the distribution of the stations used in the inversion procedure. We also introduce a 3-D velocity model to account for structural hetero- geneity. We finally estimate the uncertainties associated to the computed focal planes and the obtained Mw. We conclude that our reliable source solutions provide a moment magnitude that ranges from 5.87, 1-D model, to 5.96, 3-D model, reducing the variability of the literature to ∼0.1. We endorse that the estimate of seismic moment from moment tensor solutions, as well as the estimate of the other kinematic source parameters, requires coming out with disclosed assumptions and explicit processing workflows. Finally and, probably more important, when moment tensor solution is used for secondary analyses it has to be combined with the same main boundary conditions (e.g. wave-velocity propagation model) to avoid conflicting results.Published792-8062T. Sorgente SismicaJCR Journa

Una strana placca eritematosa parzialmente ulcerata

Presentiamo il caso di una donna di 38 anni affetta da sclerosi tuberosa ed in terapia immunosoppressiva dal 2006 per trapianto renale. La paziente presentava grave deficit cognitivo e di coordinazione motoria. All’esame obiettivo si rilevava la presenza di una placca eritematosa in regione pretibiale sinistra, parzialmente ulcerata, di cui la paziente riferiva comparsa e persistenza da circa 3 anni. Nel resto del corpo la cute si presentava integra eccetto alcune lesioni da grattamento. In passato erano state eseguite due biopsie cutanee per un miglior inquadramento diagnostico il cui referto istologico era risultato completamente aspecifico. La madre riferiva di aver notato la comparsa talvolta di alcune bolle sul corpo, che si rompevano e guarivano spontaneamente senza lasciare esiti cicatriziali. Nel sospetto di una patologia bollosa si eseguiva un prelievo ematico per immunofluorescenza indiretta e due biopsie cutanee per esame istologico e immunofluorescenza diretta. Al momento della consegna degli esiti degli esami, che deponevano per un quadro di pemfigoide bolloso, si osservava un’intensa area disepitelizzata nella regione pretibiale sinistra e due piccole bolle tese sulla gamba destra. Localmente si applicavano steroide topico sulle lesioni bollose e medicazione a base di idrofibra con argento sull’area pretibiale sinistra con risoluzione del quadro cutaneo

Gamma dose rate calculation and mapping of Piemonte (North-West Italy) from gamma spectrometry soil data

In this study the air gamma dose rate map of Piemonte, a region in the North-West of Italy, was produced from gamma spectrometry soil data. Soil samples collected in 110 different sites of Piemonte were analysed with Hyperpure Germanium (HPGe) detectors (30% relative efficiency), which allow the evaluation of the activity concentrations of natural radionuclides and Cs-137. Then, using the available mathematical models, the gamma absorbed dose rate in air due to radionuclides was calculated. The contribution of the cosmic radiation to the total absorbed dose rate, which depend on the site altitude was also evaluated and added to the soil contribution. Finally, the map of the whole region was obtained by fitting the dose rate values of the different sites with kriging algorithms