96 research outputs found
Effect of moderate hydrostatic pressure on crystallization of palm kernel stearin-sunflower oil model systems
No full textCongenital Hypopigmentary Disorders with Multiorgan Impairment: A Case Report and an Overview on Gray Hair Syndromes
No full textThe term congenital hypopigmentary disorders refers to a wide group of heterogeneous hereditary diseases, clinically characterized by inborn pigmentary defects of the iris, hair, and/or skin. They include Gray Hair Syndromes (GHSs), a rare group of autosomal recessive genodermatosis hallmarked by inborn silvery gray hair. GHSs encompass Griscelli, Chediak–Higashi, Elejalde, and Cross syndromes, which are all characterized by a broad spectrum of severe multisystem disorders, including neurological, ocular, skeletal, and immune system impairment. In this manuscript, we describe in detail the clinical, trichoscopic, and genetic features of a rare case of Griscelli syndrome; moreover, we provide an overview of all the GHSs known to date. Our report highlights how an accurate clinical examination with noninvasive methods, like trichoscopy, may play a crucial rule in diagnosis of rare and potentially lethal genetic syndromes such as Griscelli syndrome, in which timely diagnosis and therapy may modify the clinical course, quality of life, and likelihood of survival
Impact of COVID-19 lockdown in children with neurological disorders in Italy
No full textBackground: The costs and benefits of full lockdown measures are debated. Neurologically impaired children are a vulnerable population with specific needs in terms of protection against infection and access to health services. Objectives: We investigated the effects of lockdown on the health of children with neurological disorders and on their access to care during lockdown. Methods: Data from 514 children (282 males e 232 females) were collected through physician administered interviews to investigate: the occurrence of viral-like physical symptoms, the correlation between the risk of developing such symptoms and several demographic and clinical variables, the occurrence of any worsening of the children's neurological conditions during lockdown, and their access to care services during this period.Results: 49.1% experienced at least one symptom during the study period, but no child developed severe complications. The prevalence of symptoms was significantly lower during lockdown than during the previous two months. The underlying neurological condition worsened in 11.5% of the patients. Children who regularly left the home during lockdown were greater risk of exhibiting symptoms. During lock down, 67.7% had a specialist appointment cancelled, 52.6% contacted their paediatrician, and 30.9% contacted their child neuropsychiatrist. Among patients who usually receive rehabilitation, 49.5% continued remotely.Conclusion: Lockdown protected children from infections. Telemedicine and telerehabilitation constituted a valid alternative for the care and treatment of these children, but they should not become a widespread and definitive model of care. COVID-19 and other emergency response plans must take into account the specific needs of children with disabilities.(c) 2020 Elsevier Inc. All rights reserved
Does early combination vs. Monotherapy improve clinical outcomes of clinically extremely vulnerable patients with COVID-19? Results from a retrospective propensity-weighted analysis
No full textAbstract Background The potential efficacy of early combination therapy, based on an antiviral plus a monoclonal antibody, for COVID-19 in severely immunocompromised patients is matter of debate. Objectives Our aim was to describe the impact on clinical outcomes of COVID-19 treatments in severely immunocompromised individuals, evaluating differences between a combination and a monotherapy. Methods We included severely immunocompromised outpatients with mild-to-moderate COVID-19 who received an early treatment (either monotherapy with nirmatrelvir/ritonavir or remdesivir or the combination of an antiviral plus sotrovimab). We then assessed differences between the two treatment strategies on three main outcomes (30-day mortality, access to emergency department, hospitalization), separately and as a composite by using a propensity score weighted (PSW) approach. Results Eighty one severely immunocompromised patients were included, 39 receiving early combination therapy and 42 receiving monotherapy. No significant difference was observed in the 30-day mortality rate and hospitalization rate between subjects in the two groups, while access to the emergency department following treatment administration was significantly higher in people who received a combination therapy. After applying the PSW, it was observed that combination therapy impacted favourably on the composite outcome, in a statistically significant fashion. In addition, PSW approach for mortality showed that age was the only significant factor influencing the death as stand-alone outcome. Conclusions Early combination therapy showed a favourable impact on a composite outcome (including mortality, hospitalizations and access to emergency department) in severely immunocompromised hosts who were all vaccinated. However, further studies are needed to support our results
Long-term results in children with head and neck rhabdomyosarcoma: A report from the Italian Soft Tissue Sarcoma Committee
No full textBackground: Rhabdomyosarcoma (RMS) occurring at nonparameningeal head and neck (NPM-HN) sites carries a better prognosis than parameningeal RMS and some other sites. We analyzed the treatments administered and results obtained in patients with localized NPM-HN RMS, included in the protocols coordinated by the Italian Soft Tissue Sarcoma Committee (STSC), in an effort to identify prognostic factors that could facilitate the tailoring of treatment. Methods: Sixty-six patients up to 18 years of age with previously untreated, localized NPM-HN RMS were prospectively registered in three consecutive protocols: RMS79, RMS88, and RMS96. Primary surgery was recommended when complete tumor resection was deemed feasible without mutilations. In other cases, only a biopsy was performed, followed by chemotherapy and delayed surgery and/or radiotherapy (RT). Results: NPM-HN RMS showed favorable characteristics: 72.7% were <5 cm, 72.7% were T1, and 80.3% were N0. With a median follow-up of 16 years (range 7–27), the 10-year progression-free survival and overall survival for the whole group were 65.1% (confidence interval [CI]: 52.3–75.3) and 74.2% (CI: 61.8–83.1). Progressive improvement has been seen in the successive protocols. Age and RT emerged as independent prognostic factors. The group of young children (age <l year) not irradiated was at high risk of failure. Conclusion: The experience of the Italian STSC confirms that NPM-HN RMS has a favorable prognosis, which has improved over the years due to a better, multimodal treatment approach. RT has an important role, but different modalities such as proton therapy and brachytherapy should be explored in systematic ways in very young children
Teleriscaldamento, energie rinnovabili e recupero di FORSU: ipotesi per una gestione più efficiente e sostenibile del quartiere Crocifisso di Padova
No full textLa nuova sfida della sostenibilità è la riqualificazione di edifici già presenti nel territorio. In questo contesto si colloca il progetto della smart grid del quartiere del Crocifisso, situato nella zona Sud di Padova, che combina miglioramento impiantistico e sociale. Il quartiere presenta un’utenza mista: edifici scolastici, edifici residenziali, edifici turistico-ricettivi, uffici e luoghi di culto.
La proposta si basa sull’ottimizzazione della generazione di calore, impiegando una centrale termica esistente, integrata da una pompa di calore ad acqua di falda e due pompe di calore ad aria, più efficienti nei periodi di basso carico. Le utenze sono servite da una rete di teleriscaldamento lunga circa 650 metri, suddivisa in tre rami principali, di cui due gestiti a media temperatura e uno a bassa temperatura. Per la generazione distribuita di acqua calda sanitaria si utilizzeranno gli impianti già installati che sfruttano risorse rinnovabili e si predisporranno nuove soluzioni dove necessarie. L’elemento innovativo è il recupero di FORSU a fini energetici, che attraverso un processo di digestione potrebbe essere impiegato per il riscaldamento della serra collettiva del complesso. L’impiego di tale sottoprodotto, in abbinamento al recupero delle acque reflue, sarà l’occasione per sviluppare una cultura nuova, sostenibile e condivisa dell’abitare, realizzando un incentivo per gli utenti a gestire autonomamente le coltivazioni per uso personale e gli spazi verdi
Conversion Chemotherapy for Technically Unresectable Colorectal Liver Metastases
No full textThe response rate of patients with unresectable liver-limited metastases of colorectal cancer can be improved by converting inoperable disease to operable disease. However, the benefits of conversion chemotherapy for survival are still controversial. Patients considered to have technically inoperable disease by a multidisciplinary team were retrospectively analyzed. Patients were stratified based on the treatment they received, into the chemotherapy only (G1), chemotherapy plus bevacizumab (G2), or chemotherapy plus cetuximab (G3) groups. The primary endpoint was the resection rate. The secondary endpoint was the overall survival (OS), according to both the treatment received and liver surgery status. In total, 104 patients were included: 30 in the G1, 39 in the G2, and 35 in the G3 groups. All G3 patients had the wild-Type KRAS exon 2. The surgical resection rates for patients in the G1, G2, and G3 groups were 43.3% (13/30), 30.7% (12/39), and 51.4% (18/35), respectively. Disease-free survival did not show significant differences among the 3 groups. The median OS was 35.2 months in the G1, 28.8 months in the G2, and 42.1 months in the G3 (P=0.25) groups. The OS was significantly higher in patients who underwent surgical resection than those who did not. The median OS was 28.4 months in patients who did not undergo resection, whereas it had not been reached after a median follow-up period of 37.5 months for patients who underwent surgical resection (events: 21/43). Our data confirmed that the conversion of initially inoperable disease to operable disease conferred a survival benefit, even in patients who relapsed after surgery. The addition of cetuximab to chemotherapy improved the objective response and resection rates, conferring a potential survival benefit even in patients whose diseases were not converted to operable disease, compared to chemotherapy alone or in combination with bevacizumab
Di uomini, di terre e di acque: trame di paesaggio nel Vercellese occidentale
No full textL’Università di Pavia, all’interno di una convenzione tra il Dipartimento di Studi Umanistici, la Soprintendenza Archeologia Belle Arti e Paesaggio per le Province di Biella, Novara, Verbano Cusio Ossola e Vercelli e il Comune di Livorno Ferraris (VC), ha intrapreso dal 2017 un programma di ricerche archeologiche volto a tentare di ricostruire il paesaggio storico del Vercellese occidentale in prospettiva diacronica, dall’età del Ferro al basso Medioevo. In altre sedi abbiamo tracciato il
quadro storico-archeologico di partenza, necessaria premessa per affrontare lo studio del territorio in esame, che, avendo come centro l’areale dell’attuale Comune di Livorno F., si è esteso a includere la parte più occidentale di una regione compresa tra il fiume Sesia a E, la Dora Baltea a W, il Po a S e la linea dell’anfiteatro morenico in cui andrà a impiantarsi Eporedia a N: circa 1300 km2, scarsamente documentati e scarsamente investigati dal punto di vista archeologico. L’area è principalmente coltivata a risaia, fatto che, specialmente dall’Unità d’Italia in poi in poi, ha comportato una totale ridefinizione degli spazi e dell’andamento del piano topografico complessivo, e ha cancellato quelli che erano la sistemazione e il disegno precedenti, rimuovendo per ampie parti il deposito archeologico.
L’analisi del territorio, di conseguenza, è stata affrontata avvalendosi di diversi approcci metodologici:
– Raccolta dei dati archeologici noti;
– Studio geomorfologico e paleoidrografico;
– Esame della cartografia e dei catasti storici;
– Analisi della toponomastica;
– Censimento delle chiese e delle cascine;
– Esame delle immagini satellitari e delle fotografie aeree;
– Attività di ricognizione.
Tutti i dati sono stati poi integrati e analizzati con l’ausilio di una piattaforma GIS.
In questa sede, si proverà a evidenziare la correlazione tra geomorfologia, paleoidrografia e scelte insediative, focalizzando l’attenzione sulla presenza di edifici rustici e di impianti produttivi nell’are
Le proprietà del decanato di Santa Maria in Via Lata in Carignano e la loro gestione: il "Liber B" dell'Archivio Fieschi - Thellung de Courtelary (secc. XIV-XV)
No full textTraiettorie di sviluppo neuropsicologico in bambini di età prescolare nati moderatamente pretermine
No full textINTRODUZIONE
Nonostante la presenza di ricerche sulle difficoltà della nascita prematura, i risultati sugli esiti
evolutivi sono eterogenei (Aarnoudse-Moens et al., 2012; Kerstjens et al., 2012). Ciò potrebbe
essere spiegato da aspetti biologici e ambientali, ma anche metodologici. Tra questi l’uso dell’età
cronologica (UCA) o corretta (CA) (Romeo et al., 2012). Gli obiettivi di questo lavoro sono:
confrontare lo sviluppo neuropsicologico di bambini pretermine a basso rischio con quello di nati a
termine a 24 e a 36 mesi e indagare l’utilità della CA e della UCA nel follow-up dei pretermine.
METODO
Il campione è composto da 104 bambini (55 pretermine; EG media=32.01; peso medio=1610.20).
Gli strumenti utilizzati (previo consenso) sono le Bayley-III (Bayley, 2006) e la CBCL 11⁄2-5
(Achenbach & Rescorla, 2000).
RISULTATI
Le analisi preliminari (t-test, ANOVA e correlazioni) sembrano evidenziare: un minor livello di
performance dei pretermine nelle scale cognitiva e del linguaggio a 24 (t = 5.79, p < .001; t = 3.32,
p < .001) e a 36 (t = 1.98, p < .05; t = 3.09, p < .01) mesi; la presenza di un ritardo nei pretermine
con UCA nello sviluppo di competenze cognitive tra 24 e 36 mesi (F = 3,930, p < .05); correlazioni
significative tra punteggi alle Bayley, sia per CA sia per UCA, e: a) età gestazionale (24 mesi) (ρ =
.468, p < .001); b) giorni di degenza (36 mesi) (ρ = -.449, p < .01); c) presenza di tratti
ansiosi/depressivi e aggressivi (36 mesi) (ρ = .479, p < .05; ρ = .514, p < .01).
CONCLUSIONI
I dati indicano che i bambini pretermine hanno maggiori difficoltà in particolare nello sviluppo
cognitivo, anche se il gap evolutivo sembra ridursi negli anni. L’utilizzo della CA e della UCA ha
evidenziato come l’uso della CA sia utile fino ai 2 anni. I risultati evidenziano come le variabili
biologiche alla nascita influenzino lo sviluppo dei bambini pretermine fino ai 24 mesi, mentre le
loro performance a 36 mesi appaiono influenzate da più fattori quali i tratti comportamentali del
bambino e i giorni di degenza
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