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Prenatal diagnosis of bicuspid aortic valve is challenging. Bicuspid aortic valve is often associated with aortic dilation.
Fetuses with postnatally confirmed bicuspid aortic valve were gestational age-matched with normal controls. Complex lesions were excluded. Aortic valve and arch measurements by two blinded investigators were compared.
We identified 27 cases and 27 controls. Estimated fetal weight percentile was lower in cases than controls. Seven cases had one or more significant lesions including perimembranous ventricular septal defects (n = 2), isolated annular hypoplasia (n = 2), and/or arch hypoplasia/coarctation (n = 4). Fetuses with bicuspid aortic valves had significantly smaller median z-scores of the aortic annulus (–1.60 versus –0.53, p < 0.001) and root (–1.10 versus –0.53, p = 0.040), and larger ratios of root to annulus (1.32 versus 1.21, p < 0.001), sinotubular junction to annulus (1.07 versus 0.99, p < 0.001), ascending aorta to annulus (1.29 versus 1.18, p < 0.001), and transverse aorta to annulus (1.04 versus 0.96, p = 0.023). Leaflets were “doming” in 11 cases (41%) and 0 controls (p = 0.010), “thickened” in 10 cases (37%) and 0 controls (p = 0.002). We noted similar findings in the subgroup without significant additional cardiac defects.
The appearance of doming or thickened aortic valve leaflets on fetal echocardiogram is associated with bicuspid aortic valve. Compared to controls, fetuses with bicuspid aortic valve had smaller aortic annulus sizes (possibly related to smaller fetal size) without proportionally smaller aortic measurements, resulting in larger aortic dimension to annulus ratios. Despite inherent challenges of diagnosing bicuspid aortic valve prenatally, these findings may increase suspicion and prompt appropriate postnatal follow-up.
Behavioral and psychological symptoms of dementia (BPSD), constitute a major clinical component of Alzheimer’s disease (AD). There is a growing interest in BPSD as they are responsible for a large share of the suffering of patients and caregivers, and they strongly determine the patient’s lifestyle and management. Better detection and understanding of these symptoms is essential to provide appropriate management. This article is a consensus produced by the behavioral group of the European Alzheimer’s Disease Consortium (EADC). The aim of this article is to present clinical description and biological correlates of the major behavioral and psychological symptomatology in AD. BPSD is not a unitary concept. Instead, it should be divided into several symptoms or more likely: groups of symptoms, each possibly reflecting a different prevalence, course over time, biological correlate and psychosocial determinants. There is some clinical evidence for clusters within groups of BPSD. Biological studies indicate that patients with AD and BPSD are associated with variations in the pathological features (atrophy, brain perfusion/metabolism, histopathology) when compared to people with AD without BPSD. An individually tailored approach taking all these aspects into account is warranted as it may offer more, and better, pharmacological and non-pharmacological treatment opportunities.
The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion.
Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7 days).
The median Fontan age (14 centres, 320 patients) was 3.1 years (range from 1.7 to 3.9), and the weight-for-age z-score was −0.56 (−1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4–100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3–100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7–33%). The length of stay was 9.5 days (9–12); 15% (6–33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98).
Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.
Chrétien de Troyes's twelfth-century romance Perceval ou le Conte du Graal stages a telling encounter between the naïve and bumbling Perceval and his newfound chivalric mentor, Gornemont de Gort, in which the mentor asks, “Et de vos armes, biax amis, / Me redites que savez faire?” (1391–2), [Tell me again, my friend, what can you do with your arms/armor?]. Perceval responds curiously:
Jes sai bien vestir et retraire, Si com li vallés m'en arma Qui devant moi en desarma Le chevalier qu'avoie mort. (1392–5)
[I can put them on and remove them, just like the squire who armed me after disarming the knight I had slain.]
Perceval's seemingly silly reply appears at first to miss the point of Gornemont's inquiry. Instead of attesting to his skills as a knight, the newly dubbed Perceval can speak only of his skill at dressing and undressing as a knight.
And yet, Perceval's remarks aptly convey the importance of material culture, especially clothing, in the creation of chivalric masculinity within the Arthurian world. As Laurie A. Finke and Martin B. Shichtman have shown in Cinematic Illuminations (in which they analyze both Chrétien's text and the film version of it, “Perceval Le Gallois,” directed by Eric Rohmer), knights in King Arthur's realm have to perform chivalry constantly in order to maintain it.
Genetic epidemiology explores the interrelationship of genetic and environmental risk factors in which genes are measured indirectly in ways that reflect aggregate effects "averaged" across the entire genome. This chapter describes the principles and methodology of psychiatric genetics using four-paradigm framework: basic genetic epidemiology, advanced genetic epidemiology, gene finding, and molecular genetics. Each of these paradigms has strengths and limitations, and they are in a process of dynamic interaction with each other. Genetic epidemiology has proved a reliable method to answer basic questions about the overall importance of genetic risk factors for psychiatric illness. The advanced genetic epidemiology paradigm has been used to study the relationships between neuroticism and depression. Molecular genetics is an entirely laboratory-based discipline applying a range of modern methods from genomics to neuroscience to try to identify and then trace pathophysiological pathways.
This collection of essays pays tribute to Nancy Freeman Regalado, a ground-breaking scholar in the field of medieval French literature whose research has always pushed beyond disciplinary boundaries. The articles in the volume reflect the depth and diversity of her scholarship, as well as her collaborations with literary critics, philologists, historians, art historians, musicologists, and vocalists - in France, England, and the United States. Inspired by her most recent work, these twenty-four essays are tied together by a single question, rich in ramifications: how does performance shape our understanding of medieval and pre-modern literature and culture, whether the nature of that performance is visual, linguistic, theatrical, musical, religious, didactic, socio-political, or editorial? The studies presented here invite us to look afresh at the interrelationship of audience, author, text, and artifact, to imagine new ways of conceptualizing the creation, transmission, and reception of medieval literature, music, and art.
EGLAL DOSS-QUINBY is Professor of French at Smith College; ROBERTA L. KRUEGER is Professor of French at Hamilton College; E. JANE BURNS is Professor of Women's Studies and Adjunct Professor of Comparative Literature at the University of North Carolina, Chapel Hill.
Contributors: ANNE AZÉMA, RENATE BLUMENFELD-KOSINSKI, CYNTHIA J. BROWN, ELIZABETH A. R. BROWN, MATILDA TOMARYN BRUCKNER, E. JANE BURNS, ARDIS BUTTERFIELD, KIMBERLEE CAMPBELL, ROBERT L. A. CLARK, MARK CRUSE, KATHRYN A. DUYS, ELIZABETH EMERY, SYLVIA HUOT, MARILYN LAWRENCE, KATHLEEN A. LOYSEN, LAURIE POSTLEWATE, EDWARD H. ROESNER, SAMUEL N. ROSENBERG, LUCY FREEMAN SANDLER, PAMELA SHEINGORN, HELEN SOLTERER, JANE H. M. TAYLOR, EVELYN BIRGE VITZ, LORI J. WALTERS, AND MICHEL ZINK.
If all of Proust's world comes out of a teacup, the world of Sebelinne, a littleknown heroine in the Old French Dit de l'Empereur Constant, comes out of a silk purse. Indeed this thirteenth-century Byzantine romance about religious conversion and male dynastic succession actually turns on a small object fashioned from cloth: a richly decorated, heavily embroidered aumousniere. Whether damask or velvet, decorated with silk or gold embroidery, Old French aumousnieres described in romance texts and trade accounts of the thirteenth century are fashioned typically from costly silk and hung from a belt, itself often made of rich silk fabric. Although the name aumousniere suggests a pouch that might hold alms for the poor or money to be donated at pilgrimage shrines, many literary texts feature silk purses as practical receptacles for small change, herbs, unguents, and medicines, or as decorative items of attire holding anything from rings, keys, and jewels to relics and even sewing supplies. Used at times to carry holy bread, aumousnieres could also become highly symbolic gifts given as love tokens. Most interestingly, however, we know from guild accounts that silk aumonières were produced in thirteenth-century Paris and that they included a category termed “aumosnieres sarrasinoises,” purses fashioned in the manner of highly decorated Saracen work on imported silk cloth or copies thereof. The very combination of the terms “aumosnieres” and “sarrasinoises” generates a network of rich cultural resonances that are staged in the Dit de l'Empereur Constant through a dynamic and interactive performance between an unprepossessing heroine and a small piece of silk. Atypically, the aumousniere in this text contains not a relic, ring or coin but a sequence of hand-written messages. Indeed, this ornate purse is perhaps most significant as a material object from which the heroine scripts and fashions a brief plot of monumental proportions. Deftly manipulating a culturally charged item of silk, she executes a remarkable cultural performance. I would like to analyze that performance here in honor of Nancy Regalado's extended scholarly commitment to broadening our understanding of medieval performances in many guises.
A Conversion Story?
The Empereur Constant recounts no less than the legendary transformation of Byzantium into Constantinople, ostensibly by a lowly youth, Constant, who is himself changed unpredictably in the course of the tale from a “fils de vilain” into a worthy nobleman.