Dal fegato al muscolo: una rara malattia a esordio tardivo

We describe the case of a seven-year-old child who, following the incidental finding of hypertransaminasemia in the blood tests performed for a lymphadenopathy, is subjected to other blood tests that show a persistent moderate increase in CPK values, supporting the hypothesis of a muscle disease even in the absence of specific symptoms. The diagnostic path seems to point towards a late-onset metabolic myopathy. The occasional finding of hypertransaminases , associated with the finding of high CPK levels in an asymptomatic or paucisymptomatic child, orients the diagnostic path. Excluding hepatic storage disease or viral infectious disease, the differential diagnosis leads to a complex late-onset myopathy such as Pompe disease, a rare but important diagnosis whose prognosis could be dramatically improved by the enzyme replacement therapy

Forme dell’intreccio per ri-cucire memorie di antico impianto in Basilicata/Twine forms to re-stitch memories of an ancient plan in Basilicata

The research, starting from the knowledge of territories and fragile heritages in Lucana land, tries to re-configure a possible transformation to re-inhabit the abandoned ancient city center of Craco, hypothesizing new opportunities for cultural, tourist and economic enhancement. The project imagines and designs a widespread system of small refuges and places to stop and enjoy the landscape. These small architectures will be able to give visitors the most sublime of experiences: living among the rocks and falling asleep under the uncovered vaults of a ghost town, among the ruins of ancient Craco and the badlands of these lands. The artifacts designed as temporary and temporary works, work and graft through the concept of ‘suspension’ and ‘lightness’, between the wrecks, alleys and streets, the skeletons of the houses, the flattened earth of rubble of the remains of architecture, materials of the art of building that once designed these places. The shape of the city and of the architectural materials of which it is composed characterized the project that took place around the study and the creation of prototypes to design wooden and iron crossing devices for the consolidation of the ruins and also to be replicable in other areas with similar characters and morphologies.La ricerca, partendo dalla conoscenza di territori e patrimoni fragili in terra Lucana, tenta di ri-configurare una possibile trasformazione per ri-abitare il borgo antico abbandonato della città di Craco, ipotizzando nuove opportunità di valorizzazione culturale, turistica ed economica. Il progetto immagina e disegna un sistema diffuso di piccoli rifugi e luoghi di sosta e fruizione del paesaggio. Queste piccole architetture saranno in grado di donare ai visitatori la più sublime delle esperienze: abitare fra le rocce e addormentarsi sotto le volte scoperte di una città fantasma, fra le rovine di Craco antica ed i calanchi di queste terre. I manufatti disegnati come opere provvisionali e provvisorie, lavorano e si innestano mediante il concetto di ‘sospensione’ e ‘leggerezza’, tra i relitti, i vicoli e le strade, gli scheletri delle case, la terra spianata di macerie dei resti di architetture, materiali dell’arte del costruire che un tempo hanno disegnato questi luoghi. La forma della città e dei materiali architettonici di cui essa si compone hanno caratterizzato il progetto che si è svolto intorno allo studio ed alla realizzazione di prototipi per disegnare dispositivi di attraversamento in legno e ferro per il consolidamento dei ruderi ed essere anche replicabili in altre zone con caratteri e morfologie analoghe

Exploring the use of cluster analysis to assess antibiotic stewardship in critically-ill neonates in a low resource setting

Abstract Background Sepsis is the third leading cause of neonatal death in low and middle-income countries, accounting for one third of all deaths in Ethiopia. A concerning issue is the increasing number of multidrug-resistant microorganisms facilitated by suboptimal antibiotic stewardship. The study aims to identify clusters of newborns switching antibiotic lines for sepsis in a neonatal intensive care unit (NICU) in Ethiopia, and to explore their potential association with sepsis outcomes. Methods A retrospective cohort study was conducted including all newborns discharged with a diagnosis of probable neonatal sepsis from the St. Luke Catholic Hospital NICU between April and July 2021. The antibiotic management protocol included two lines according to WHO guidelines and a third line based on internal hospital guidelines. In the cluster analysis, the Gower distance was estimated based on the antibiotics employed in the different lines and the duration of each line. Mortality and respiratory distress (RD) were the response variables. Results In the study period, 456 newborns were admitted to the NICU and 196 (42.8%) had probable neonatal sepsis. Four antibiotic management clusters were identified. Cluster 1 (n = 145, 74.4%) had no antibiotic switches, using only the first line. Cluster 2 (n = 26, 13.3%) had one switch from the first to the second line. Cluster 4 (n = 9, 4.6%) had two switches: from first to second and then to third line. In cluster 3 (n = 15, 7.7%), newborns were switched from ceftriaxone/cloxacillin as second line to off-protocol antibiotics. There were no differences in sex, age, weight on admission or crude mortality between clusters. Cluster 3 included a higher frequency of infants who did not breathe at birth (53.3%, p = 0.011) and that necessitated bag ventilation (46.7%, p = 0.039) compared to the other clusters. Conclusions The first antibiotic line failed in one out of four newborns with probable sepsis while third-generation cephalosporins were insufficient in one in ten patients. Cluster analysis can provide valuable insights into antibiotic treatment patterns and their potential implications. This approach may support antibiotic stewardship and aid in contrasting antimicrobial resistance in limited resource settings

Training on the Silverman and Andersen score improved how special care unit nurses assessed neonatal respiratory distress in a low-resource setting

Aim Identifying the severity of neonatal respiratory distress (RD) is essential, so that resources can be appropriately allocated. We assessed the ability of nurses to grade neonatal RD in a low-resource setting before and after they were trained to use a dedicated scoring tool. Methods The study was conducted in the Special Care Unit of St Luke Wolisso Hospital, Ethiopia. Ten nurses reviewed nine local video recordings and graded neonatal RD without a standardised method, which was current practice, and then after they were trained to use the Silverman and Andersen score. The data were analysed using the McNemar test and Cohen's kappa. Results Training increased the identification of mild RD from 63% to 93% (p = 0.008) and moderate RD from 40% to 73% (p = 0.03). Severe RD was 93% before and 90% after training (p = 0.99). Overall, the agreement improved from kappa 0.59 to 0.84, mainly by reducing the overestimation of milder degrees of RD. Conclusion Being trained on how to use the Silverman and Andersen score improved the ability of nurses to identify mild and moderate neonatal RD. This improvement has the potential to optimise the use of equipment, staff and time

Isolation and molecular characterization of free-living amoebae from different water sources in Italy

Free-living amoebae (FLA) are protozoa ubiquitous in Nature, isolated from a variety of environments worldwide. In addition to their natural distribution, some species have been found to be pathogenic to humans. In the present study a survey was conducted in order to evaluate the presence and to characterize at molecular level the isolates of amoebic organisms collected from different water sources in Italy. A total of 160 water samples were analyzed by culture and microscopic examination. FLA were found in 46 (28.7%) of the investigated water samples. Groundwater, well waters, and ornamental fountain waters were the sources with higher prevalence rates (85.7%, 50.0%, and 45.9%, respectively). Identification of FLA species/genotypes, based on the 18S rDNA regions, allowed to identify 18 (39.1%) Acanthamoeba isolates (genotypes T4 and T15) and 21 (45.6%) Vermamoeba vermiformis isolates. Other FLA species, including Vahlkampfia sp. and Naegleria spp., previously reported in Italy, were not recovered. The occurrence of potentially pathogenic free-living amoebae in habitats related to human population, as reported in the present study, supports the relevance of FLA as a potential health threat to humans

Dynamic NLR and PLR in Predicting COVID-19 Severity: A Retrospective Cohort Study

Abstract Introduction The hyperinflammation phase of severe SARS-CoV-2 is characterised by complete blood count alterations. In this context, the neutrophil-to-lymphocyte ratio (NLR) and the platelet-to-lymphocyte ratio (PLR) can be used as prognostic factors. We studied NLR and PLR trends at different timepoints and computed optimal cutoffs to predict four outcomes: use of continuous positive airways pressure (CPAP), intensive care unit (ICU) admission, invasive ventilation and death. Methods We retrospectively included all adult patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pneumonia admitted from 23 January 2020 to 18 May 2021. Analyses included non-parametric tests to study the ability of NLR and PLR to distinguish the patients’ outcomes at each timepoint. Receiver operating characteristic (ROC) curves were built for NLR and PLR at each timepoint (minus discharge) to identify cutoffs to distinguish severe and non-severe disease. Their statistical significance was assessed with the chi-square test. Collection of data under the SMACORE database was approved with protocol number 20200046877. Results We included 2169 patients. NLR and PLR were higher in severe coronavirus disease 2019 (COVID-19). Both ratios were able to distinguish the outcomes at each timepoint. For NLR, the areas under the receiver operating characteristic curve (AUROC) ranged between 0.59 and 0.81, and for PLR between 0.53 and 0.67. From each ROC curve we computed an optimal cutoff value. Conclusion NLR and PLR cutoffs are able to distinguish severity grades and mortality at different timepoints during the course of disease, and, as such, they allow a tailored approach. Future prospects include validating our cutoffs in a prospective cohort and comparing their performance against other COVID-19 scores