Diplomatici e libri greci a Venezia: la biblioteca di Guillaume Pellicier

La biblioteca di Guillaume Pellicier (ca. 1490-1568), vescovo di Montpellier e ambasciatore francese a Venezia negli anni 1539-1542, è una collezione libraria di grande interesse storico-culturale, che non ha riscosso l’attenzione che merita. Durante gli anni veneziani, approfittando dell’incarico ricevuto dal re di procurare libri per la biblioteca di Fontainebleau, Pellicier al contempo acquistò libri e commissionò copie per la propria biblioteca privata, mettendo insieme una collezione notevole. Il presente contributo si propone di presentare questa biblioteca, di ripercorrerne la storia e di delineare future linee di ricerca.The library of Guillaume Pellicier (ca. 1490-1568), bishop of Montpellier and French ambassador to Venice in the years 1539-1542, is a book collection of high historical and cultural interest, which so far has not received adequate consideration. Taking advantage of the King’s assignment to acquire books in Venice for the library of Fontainebleau, at the same time Pellicier acquired and commissioned books for his own library, putting together a huge book collection. The paper aims at presenting the book collection, its history and some paths of further investigation

Dyke-Davidoff-Masson syndrome: case report of fetal unilateral ventriculomegaly and hypoplastic left middle cerebral artery

Prenatal ultrasonographic detection of unilateral cerebral ventriculomegaly arises suspicion of pathological condition related to cerebrospinal fluid flow obstruction or cerebral parenchimal pathology. Dyke-Davidoff-Masson syndrome is a rare condition characterized by cerebral hemiatrophy, calvarial thickening, skull and facial asymmetry, contralateral hemiparesis, cognitive impairment and seizures. Congenital and acquired types are recognized and have been described, mainly in late childhood, adolescence and adult ages. We describe a female infant with prenatal diagnosis of unilateral left ventriculomegaly in which early brain MRI and contrast enhanced-MRI angiography, showed cerebral left hemiatrophy associated with reduced caliber of the left middle cerebral artery revealing the characteristic findings of the Dyke-Davidoff-Masson syndrome. Prenatal imaging, cerebral vascular anomaly responsible for the cerebral hemiatrophy and the early clinical evolution have never been described before in such a young child and complete the acquired clinical descriptions in older children. Differential diagnosis, genetic investigations, neurophysiologic assessments, short term clinical and developmental follow up are described. Dyke-Davidoff-Masson syndrome must be ruled out in differential diagnosis of fetal unilateral ventriculomegaly. Early clinical assessment, differential diagnosis and cerebral imaging including cerebral MRI angiography allow the clinicians to diagnose also in early infancy this rare condition

Invasive pneumococcal diseases in children aged 1-59 months in sicily, Italy: Importance of active family paediatrician surveillance and vaccination coverage

Purpose: Aim of this study was to analyze pediatric invasive pneumococcal disease rates several years after the implementation of infant pneumococcal vaccination. Methods: The study was carried out in Sicily and involved about 30,000 children, aged 1-59 months, actively monitored by 100 family pediatricians during 2010 and 2011. All children who met the inclusion criteria were considered eligible, recorded using a stan-dardized case report form and investigated for the presence of S. pneumoniae in speci-mens from sterile sites. Results: None of the 40 eligible children was confirmed as a case of invasive pneumococ-cal disease. The incidence rate of invasive pneumococcal disease cases was 0.0/100,000 in both years. Regional childhood pneumococcal vaccination coverage rates were 90.7% in 2010 and 92.0% in 2011. Conclusions: Our results show that during the study period invasive pneumococcal dis-ease cases were rare in Sicilian children, suggesting a very effective control of the disease in a region with very high vaccination coverage against S. pneumoniae

PTEN hamartoma tumor syndromes in childhood: description of two cases and a proposal for follow-up protocol

PTEN hamartoma tumor syndromes (PHTS) are a spectrum of hamartomatous overgrowth syndromes associated with germline mutations in the tumor suppressor PTEN gene located on 10q23.3. It is widely accepted that two of these disorders,Cowden syndrome and Bannayan–Riley–Ruvalcaba syndrome, are allelic conditions. BecausePTENmutations are not identifiable in every case of the PHTS phenotype, the inability to detect a mutation within thePTENgene does not invalidate the clinical diagnosis of Cowden syndrome, or Bannayan–Riley–Ruvalcaba syndrome, in patients who meet diagnostic criteria for these disorders. PTEN mutations are associated with an increased risk for developing breast, thyroid, endometrial, and sometimes renal cancers. Thus, cancer surveillance is the cornerstone of PHTS patient management. Although a consensus cancer surveillance protocol has not been formally instituted, all PTEN mutation carriers should adopt the cancer surveillance strategies proposed for patients with Cowden syndrome. In addition, because gastrointestinal and vascular complications can be more severe in Bannayan– Riley–Ruvalcaba syndrome than in Cowden syndrome, patients with Bannayan–Riley–Ruvalcaba syndrome should be monitored from this point of view too. In this study, we report on two cases with Bannayan–Riley–Ruvalcaba phenotype that showed two different PTEN mutations.Wealso propose practice recommendations for management of PHTS patient

Intrauterine growth pattern and birthweight discordance in twin pregnancies: a retrospective study

Background: Twins, compared to singletons, have an increased risk of perinatal mortality and morbidity, due mainly to a higher prevalence of preterm birth and low birthweight. Intrauterine growth restriction (IUGR) is also common and can affect one or both fetuses. In some cases, however, one twin is much smaller than the other (growth discordance). Usually, high birthweight discordance is associated with increased perinatal morbidity. The aim of this study is to describe the epidemiological features of a population of twins at birth, with particular reference to the interpretation and clinical effects of birthweight discordance. Methods: We evaluated retrospectively the clinical features of 70 infants born from twin pregnancies and assessed birthweight discordance in 31 pregnancies where both twins were followed at our institution. Discordance was treated both as a continuous and a categorical variable, using a cutoff of 18%. Possible relationships between birthweight discordance and other variables, such as maternal age, gestational age, birthweight percentile, number of SGA newborns in the pair, Hematocrit (Ht) discordance and neonatal anemia, prevalence of malformations, neonatal morbidity and death, were analyzed. Results: In our cohort birthweight percentile decreased slightly with increasing gestational age. Birthweight discordance, on the contrary, increased slightly with the increase of gestational age. A high discordance is associated to the presence of one SGA twin, with the other AGA or LGA. In our population, all 6 pregnancies in which discordance exceeded 18% belonged to this category (one SGA twin). Ht discordance at birth is associated to the presence of neonatal anemia in a twin, but it is not significantly related to weight discordance. Finally, in our case history, weight discordance is not associated in any way with the prevalence of malformations, morbidity and mortality. Conclusions: Birthweight discordance is an important indicator of complications that act asymmetrically on the two fetuses, affecting intrauterine growth in one of them, and usually determining the birth of a SGA infant. Our case history shows a significant statistical association between pair discordance and IUGR in one of the twins, but we could not demonstrate any relationship between discordance and the prevalence of malformations, morbidity and mortality

CONGENITAL DIAPHRAGMATIC HERNIA AND ESOPHAGEAL ATRESIA: THE IMPORTANCE OF RESPIRATORY FOLLOW-UP IN CONGENITAL THORACIC MALFORMATION

Esophageal atresia, congenital diaphragmatic hernia, pulmonary function test, respiratory morbidity, Long-term follow-u