Asymptomatic congenital cystic adenomatoid malformation of the lung: Is it time to operate?

ObjectiveThe optimal management of congenital adenomatoid malformation of the lung remains controversial. Prenatal ultrasonographic analysis has increasingly discovered asymptomatic lesions, raising questions about the need for and timing of surgical treatment for asymptomatic congenital adenomatoid malformation. The aim of our study was to analyze the short-term postoperative outcome of symptomatic congenital adenomatoid malformations compared with asymptomatic malformations.MethodsAll the data of patients presenting with congenital adenomatoid malformations histologically diagnosed and operated on between 1998 and 2005 at our institution were retrospectively reviewed. Patients were divided into 2 groups: group A comprised asymptomatic infants, and group B comprised symptomatic infants. Major outcomes considered were the length of ventilation, pleural drainage, and hospital stay. Postoperative morbidity and mortality were also evaluated. Asymptomatic patients were further stratified for age at the time of the operation to evaluate whether age at surgical intervention affects the outcome. The Fisher's exact and Mann–Whitney tests were used as appropriate.ResultsFifty-seven patients were consecutively treated. Thirty-five patients were given diagnoses of asymptomatic lesions and were enrolled into group A, whereas 22 patients presenting with symptoms were entered into group B. The lengths of ventilation, pleural drainage, and hospital stay were significantly longer in patients with symptomatic congenital adenomatoid malformations. Moreover, symptomatic patients presented with a higher postoperative complication rate. The age-based stratification of asymptomatic children did not show any difference on either postoperative mortality or major outcome considered.ConclusionChildren with congenital adenomatoid malformations operated on when asymptomatic present a better short-term outcome than symptomatic children. In addition, age at the time of the operation does not negatively affect the outcome. Our findings support early surgical treatment for asymptomatic congenital adenomatoid malformation

Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis

Importance: Juvenile amyotrophic lateral sclerosis (ALS) is a rare form of ALS characterized by age of symptom onset less than 25 years and a variable presentation.Objective: To identify the genetic variants associated with juvenile ALS.Design, Setting, and Participants: In this multicenter family-based genetic study, trio whole-exome sequencing was performed to identify the disease-associated gene in a case series of unrelated patients diagnosed with juvenile ALS and severe growth retardation. The patients and their family members were enrolled at academic hospitals and a government research facility between March 1, 2016, and March 13, 2020, and were observed until October 1, 2020. Whole-exome sequencing was also performed in a series of patients with juvenile ALS. A total of 66 patients with juvenile ALS and 6258 adult patients with ALS participated in the study. Patients were selected for the study based on their diagnosis, and all eligible participants were enrolled in the study. None of the participants had a family history of neurological disorders, suggesting de novo variants as the underlying genetic mechanism.Main Outcomes and Measures: De novo variants present only in the index case and not in unaffected family members.Results: Trio whole-exome sequencing was performed in 3 patients diagnosed with juvenile ALS and their parents. An additional 63 patients with juvenile ALS and 6258 adult patients with ALS were subsequently screened for variants in the SPTLC1 gene. De novo variants in SPTLC1 (p.Ala20Ser in 2 patients and p.Ser331Tyr in 1 patient) were identified in 3 unrelated patients diagnosed with juvenile ALS and failure to thrive. A fourth variant (p.Leu39del) was identified in a patient with juvenile ALS where parental DNA was unavailable. Variants in this gene have been previously shown to be associated with autosomal-dominant hereditary sensory autonomic neuropathy, type 1A, by disrupting an essential enzyme complex in the sphingolipid synthesis pathway.Conclusions and Relevance: These data broaden the phenotype associated with SPTLC1 and suggest that patients presenting with juvenile ALS should be screened for variants in this gene.</p

The reference site collaborative network of the european innovation partnership on active and healthy ageing

Seventy four Reference Sites of the European Innovation Partnership on Active and Healthy Ageing (EIP on AHA) have been recognised by the European Commission in 2016 for their commitment to excellence in investing and scaling up innovative solutions for active and healthy ageing. The Reference Site Collaborative Network (RSCN) brings together the EIP on AHA Reference Sites awarded by the European Commission, and Candidate Reference Sites into a single forum. The overarching goals are to promote cooperation, share and transfer good practice and solutions in the development and scaling up of health and care strategies, policies and service delivery models, while at the same time supporting the action groups in their work. The RSCN aspires to be recognized by the EU Commission as the principal forum and authority representing all EIP on AHA Reference Sites. The RSCN will contribute to achieve the goals of the EIP on AHA by improving health and care outcomes for citizens across Europe, and the development of sustainable economic growth and the creation of jobs

Combinazione di un vaccino genetico basato su DNA e adenovirus con un agonista del TLR9 nell'immunoterapia dei tumori

Argomento del presente lavoro è lo sviluppo di un vaccino antitumorale basato sulla combinazione di DNA, vettori virali ed immunomodulatori, contro tre antigeni tumorali, ossia HER2, CEA e TERT. Il DNA plasmidico è stato iniettato nel muscolo dell'animale mediante elettroporazione e la risposta immunitaria è stata misurata mediante ELISA, intracellular staining per IFNgamma ed ELISPOT. La strategia d’immunizzazione ha previsto un priming a DNA e, successivamente, un boost con un adenovirus replicazione-difettivo. Infine, come adiuvante del vaccino è stato utilizzato un ligando del recettore Toll-like 9, detto IMO. Per quanto concerne l’antigene HER2, è stato valutato l’effetto della combinazione di vaccino ed IMO nel modello dei topi BALB/NeuT, sia in uno studio profilattico che in uno terapeutico. Nel primo caso, il vaccino basato su DNA si è dimostrato sufficiente ad indurre una risposta immune, mentre nel secondo caso è stata necessaria la combinazione di vaccino eterologo, basato su DNA ed adenovirus, ed IMO per ottenere anche un consistente effetto antitumorale. Per quanto riguarda CEA invece, gli studi effettuati sui topi transgenici per CEA hanno dimostrato che la combinazione di vaccino eterologo ed IMO non è capace di indurre né una risposta immune né un effetto antitumorale. È stato quindi scelto TERT quale antigene target di un vaccino genetico antitumorale. Sia nel modello del melanoma murino sia in quello del carcinoma renale murino, è stata dimostrata l’efficacia della combinazione di vaccino eterologo contro TERT ed IMO nell’induzione di un forte effetto antitumorale. In particolare, l’analisi degli infiltrati tumorali ha dimostrato che l’effetto terapeutico osservato risulta correlato con l’induzione della risposta immune innata e con l’aumento delle cellule T e NK TERT-specifiche, richiamate all’interno del tumore grazie alla somministrazione peritumorale di IMO. Infine, dato che diversi studi hanno dimostrato l’esistenza di notevoli somiglianze tra l’uomo ed il cane per quanto concerne l’evoluzione e le caratteristiche generali tumorali, è stata sperimentata, nel modello del linfoma canino, l’efficacia del vaccino contro la forma canina di TERT nell’induzione di una forte risposta immune e soprattutto di un effetto antitumorale. Il vaccino è risultato in grado di indurre una risposta immune forte e durevole contro TERT ed un suo utilizzo in combinazione con la chemioterapia sui pazienti di cane può notevolmente aumentarne la sopravvivenza. Tali dati promuovono l’utilizzo dei vaccini contro TERT in combinazione con la chemioterapia in pazienti umani affetti da linfoma

SARS-Cov-2 pneumonia and concurrent myelodysplasia complicated by pseudomonas aeruginosa over-infection

The new virus called severe acute respiratory syndrome coronavirus 2 (SARSCov-2) causing Coronavirus disease 2019 (COVID-19) has spread quickly in several countries and it has become pandemic. Different types of clinical manifestations are attributed to this infection. Some mechanisms related to the infection regarding the immune response are not still elucidated. Herein we reported a case of a 66-years-old patient affected by myelodysplasia who was referred to our hospital because of clinical and radiological manifestations of viral pneumonia. The clinical course has become complicated due to bacterial secondary over-infection by Pseudomonas aeruginosa during stay in internal medicine unit whilst a persistent positive oral and naso-pharyngeal swab test was reported up to 100 days of admission. The patient had a fast clinical and radiological worsening that led her to be admitted to an intensive care unit. Despite intubation and mechanical ventilation she died in a few days

Inhibition of Cancer Cell Proliferation and Antiradical Effects of Decoction, Hydroalcoholic Extract, and Principal Constituents of Hemidesmus indicus R. Br

Indian Sarsaparilla (Hemidesmus indicus R. Br.) is widely used in Indian traditional medicine. In the present work, we explored the effects of decoction, traditional Ayurvedic preparation, and hydroalcoholic extract, a phytocomplex more traditionally studied and commercialized as food supplement in western medicine, from the roots as possible source of chemicals with new functional potential linked to their nutritional uses. The antiproliferative and antioxidant properties were assayed. To test antiproliferative affects, different cancer cell lines, growing both as monolayers (CaCo2, MCF-7, A549, K562, MDA-MB-231, Jurkat, HepG2, and LoVo) and in suspension (K562 and Jurkat) were used. The decoction showed strong activity on HepG2 cells, while the hydroalcoholic extracts were active on HepG2, LoVo, MCF-7, K562, and Jurkat cell lines. Weak inhibition of cancer cell proliferation was observed for the principal constituents of the preparations: 2-hydroxy-4-methoxybenzaldehyde, 2-hydroxy-4-methoxybenzoic acid, and 3-hydroxy-4-methoxybenzaldehyde that were tested alone. The antiradical activity was tested with 2,2-diphenyl-1-picrylhydrazyl and 2,2'-azinobis(3-ethylbenzothiazoline-6-sulfonic acid)diammonium salt tests and inhibition of nitric oxide production in lipopolysaccharide-stimulated RAW 264.7 macrophages. Interesting result has also been obtained for hydroalcoholic extract regarding genoprotective potential (58.79% of inhibition at 37.5 µg/mL). Copyright © 2015 John Wiley & Sons, Ltd

Use of Iron and Steel Slags in Concrete: State of the Art and Future Perspectives

In the two last decades, world production of pig iron and steel has undergone a significant increase. In 2018, 1252.87 and 1806.46 million tons of pig iron and steel, respectively, were produced as compared to the 575.78 and 809.94 million tons of 2000. Consequently, the amount of the different types of slags deriving from these production processes has also increased considerably. In relation to the principles of sustainability and circular economy, the available literature suggests several possible reuses for these slags (bituminous conglomerates, hydraulic engineering, metallurgy, fertilizers, etc.). This paper aims to provide an overview of the iron and steel slags production and their reuse in concrete (for example as replacement of cement, fine or coarse aggregates). The characteristics of slags are analyzed in terms of chemical, physical, and mechanical properties. Mechanical and durability tests (both from material and structures point of view) carried out in the different studies and research are shown as well. Particular attention was devoted to electric arc furnace slags (EAF) since they are the most produced in Italy. Based on this deep literature review, the gaps that still require further studies have been identified and discussed