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"Vasta, Giorgio"
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الزمن الحسي
تعود بنا رواية \"الزمن الحسي\" إلى عام 1978، في مدينة باليرمو الغارقة في بريتها وبدائيتها، حيث يواجه ثلاثة صبية في الحادية عشرة من أعمارهم، مليئين بالحماسة والأفكار، العالم لأول مرة، ثلاثة صبية مبكري النضج، وشهوانيين، وحوشيين، يجوبون المدينة مقتنعين بأنهم أشخاص مختارون، من روما، تهب عليهم رياح ذلك العام الفظيع-الألوية الحمر واختطاف ألدو مورو-وبسبب سخطهم من النزعة الإقليمية في إيطاليا، يبدؤون بالانفصال شيئا فشيئا عن الواقع ويؤسسون خليتهم الإرهابية الخاصة.
Book
Time on my hands
\"The year is 1978, and a chilling drama is unfolding in Rome. Members of a leftist terrorist group known as the Red Brigades have kidnapped the former Italian prime minister, Aldo Moro, and are holding him in a secret prison while broadcasting their demands to the public. Far from Rome, in Palermo, Sicily, a trio of eleven-year-old schoolboys are following Moro's abduction with intense interest. To their minds, the terrorists are warriors, striking a blow at the stifling conformity and propriety of everyday Italian life\"--P. [4] of cover.
Book
380 Ultra high-risk gestational trophoblastic neoplasia: retrospective study of a single cohort at san raffaele scientific institute, milan-italy
Introduction/BackgroundGestational trophoblastic neoplasia (GTN) with score >12 represents ultra-high-risk-GTN. Study investigates characteristics, treatment and outcome.Methodology14 ultra-high-risk-GTN patients, collected between January 1996 and October 2022, have been analyzed with descriptive statistics.ResultsAll patients were diagnosed with choriocarcinoma. Average age was 36 years, 28.6% were older than 40. All were symptomatic. Metrorrhagia was present in 57%. 78.6% had systemic symptoms, of these 55% had more than one symptom. 57% had respiratory distress, 14.3% hemorrhagic shock for rupture of arteriovenous-malformations (AVMs), 21.4% hyperthyroidism, 14.3% neurological symptoms, 21.4% hypertension, of these, one with chest pain and another with nephrotic syndrome and hypercalcemia, 14.3% gastrointestinal symptoms and two patients with kidney failure. Average serum β-sub-unit human-chorionic-gonadotropin (β-hCG) was 9773643 IU/L (477–3000000). Antecedent pregnancy was a term in 9(64.3%). Time interval from antecedent pregnancy was ≥12 months in 50%. All had lung metastases and 11(78.6%) brain and/or liver metastases. Average FIGO (International Federation Gynecology Obstetrics) score was 16 (14–18). 9(64.3%) were treated with EMA/CO (etoposide-methothrexate-dactinomicyn/cyclophosphamide-vincristine) while 1(7.1%) was treated with EP/EMA (etoposide-cisplatin/EMA and 4(28.6%) with EP/EMA with high-dose of methotrexate. From 1996 to 2004 patients did not induction with low-dose etoposide-cisplatin (EP), (7 patients). One patient died after first chemotherapy cycle. 4 received a second line for resistance development and one of them performed a third line for progression but died during treatment. All had alopecia and myelosuppression after each chemotherapy. After 2004, of 7 patients who performed EP-induction. All had alopecia and myelosuppression, 4 had oral mucositis of which one needed parenteral nutrition and had to change treatment. A patient developed corticosteroid-psychotic-disorder and another Posterior-Reversible-Encephalopathy-Syndrome-(PRES). One had disease progression, deceased from rupture of pulmonary AVMs.ConclusionUltra-high-risk-GTN is a systemic pathology such as to require medical observation both at diagnosis and during treatment. Standard regimen should be EMA/EP preceded by low-dose-EP.DisclosuresThe authors declare no conflict of interest.No financial disclosures to declare.
Journal Article
Seven mutations in the human insulin gene linked to permanent neonatal/infancy-onset diabetes mellitus
Permanent neonatal diabetes mellitus (PNDM) is a rare disorder usually presenting within 6 months of birth. Although several genes have been linked to this disorder, in almost half the cases documented in Italy, the genetic cause remains unknown. Because the Akita mouse bearing a mutation in the Ins2 gene exhibits PNDM associated with pancreatic beta cell apoptosis, we sequenced the human insulin gene in PNDM subjects with unidentified mutations. We discovered 7 heterozygous mutations in 10 unrelated probands. In 8 of these patients, insulin secretion was detectable at diabetes onset, but rapidly declined over time. When these mutant proinsulins were expressed in HEK293 cells, we observed defects in insulin protein folding and secretion. In these experiments, expression of the mutant proinsulins was also associated with increased Grp78 protein expression and XBP1 mRNA splicing, 2 markers of endoplasmic reticulum stress, and with increased apoptosis. Similarly transfected INS-1E insulinoma cells had diminished viability compared with those expressing WT proinsulin. In conclusion, we find that mutations in the insulin gene that promote proinsulin misfolding may cause PNDM.
Journal Article
Disentangling the relationship between social cognition, executive functions and behaviour changes in amyotrophic lateral sclerosis
BackgroundSocial cognition (SC) deficits are included in the amyotrophic lateral sclerosis-frontotemporal spectrum disorder (ALS-FTDS) revised diagnostic criteria. However, the impact of SC assessment on cognitive classification and the cognitive–behavioural correlates of SC remain unclear. This cross-sectional study aimed to assess the impact of SC assessment on ALS-FTDS categorisation and explore the relationship of SC with executive functions (EF) and behaviour changes in a cohort of ALS patients.Methods121 patients and 56 healthy controls from the Turin ALS Centre underwent cognitive/behavioural testing, including the SC subdomains of facial emotion recognition, and cognitive and affective theory of mind (ToM).ResultsPatients performed significantly worse than controls in all SC explored domains, and 45% of patients exhibited a deficit in at least one SC test, dissociated from the presence of EF deficits. In 13% of cases, the SC deficit was isolated and subclinical. SC assessment contributed to the attribution of cognitive impairment in 10% of patients. Through a statistical clustering approach, we found that ToM only partially overlaps with EF while behaviour changes are associated with emotional disorders (anxiety and depression).ConclusionsSC is overall independent of EF in ALS, with ToM only partially associated with specific EF measures, and behaviour changes associated with emotional disorders. The influence of SC on cognitive categorisation and the frequent identification of a subclinical SC impairment have implications in a clinical setting, considering the substantial impact of cognitive impairment on disease burden and therapeutic choices.
Journal Article
Surveillance alone in stage I malignant ovarian germ cell tumors: a MITO (Multicenter Italian Trials in Ovarian cancer) prospective observational study
ObjectiveThe aim of this study was to analyze the oncological outcome of stage I malignant ovarian germ cell tumors patients included in the MITO-9 study to identify those who might be recommended routine surveillance alone after complete surgical staging.MethodsMITO-9 was a prospective observational study analyzing data collected between January 2013 and December 2019. Three groups were identified: group A included 13 patients stage IA dysgerminoma and IAG1 immature teratoma; group B included 29 patients with stage IB–C dysgerminomas, IA–C G2–G3 immature teratomas and stage IA mixed malignant ovarian germ cell tumors and yolk sac tumors; and group C included five patients (two patients with stage IC1 and one patient with stage IC2 yolk sac tumors and two patients with mixed-stage IC2 malignant ovarian germ cell tumors).ResultsA total of 47 patients with stage I conservatively treated malignant ovarian germ cell tumors were analyzed. Two patients in group B were excluded from the routine surveillance alone group due to positive surgical restaging. Therefore, a total of 45 patients were included in the study. Median follow-up was 46.2 months (range; 6–83). In total, 14 of 45 patients (31.1%) received chemotherapy, while 31 (68.9%%) underwent surveillance alone. One patient in group A, with stage IA dysgerminoma had a relapse, successfully managed with conservative surgery and chemotherapy. None of the patients in group B and C relapsed. All patients were alive at completion of the study. Overall, among 31 patients (68.9%) who underwent surveillance alone, only one patient relapsed but was treated successfully.ConclusionsOur data showed that close surveillance alone could be an alternative option to avoid adjuvant chemotherapy in properly staged IB–C dysgerminomas, IA–IC G2–G3 immature teratomas, and IA mixed malignant ovarian germ cell tumors with yolk sac tumor component.
Journal Article