3 resultados para Síndrome de Christ-Siemens-Touraine
em AMS Tesi di Dottorato - Alm@DL - Università di Bologna
Resumo:
Introduction. Ectodermal Dysplasias are a heterogeneous group of inherited disorders characterized by dysplasia of tissues of ectodermal origin (hair, nails, teeth, skins and glands). Clinically, it may be divided into two broad categories: the X-linked hypoidrotic form and the hidrotic form. Hypohidrotic Ectodermal Dysplasia (H.E.D) is characterized by the triad oligo-anodontia, hypotricosis, hypo-anhydrosis (Christ-Siemens-Tourane syndrome). The incidence of HED is about 1/100,000. Mutation in the actodysplasin-A (EDA) and ectodysplasin-A receptor (EDAR) genes are responsible for X-linked and autosomal HED. The clinical features include sparse, fine hair, missing or conical-shaped teeth, decreased sweat and mucous glands, hypoplastic skin, and heat intolerance with exercise or increased ambient temperature. Complete or partial anodontia and malformation of teeth are the most frequent dental findings. Incisors and canines are often conical-shaped while primarily second molars, if present, are mostly affected by taurodontism. Treatment is supportive and includes protection from heat exposure, early prosthetic rehabilitation, skin, hair ear, nose and nail care, and genetic counseling for family planning. The diagnosis of HED in the neonatal and early infancy period may be difficult since sparse hair and absent teeth are normal finding at this age. In childhood the diagnosis is more easily made on the basis of history and clinical examination. Dental abnormalities are the most common complaint. Prosthetic rehabilitation has been recommended as an essential part of the management of HED because is important from functional, esthetic, and psychological standpoint. A team approach that includes input from a pediatric dentist, an orthodontist, a prosthodontist, and an oral and maxillofacial surgeon is necessary for a successful outcome. Conventional prosthodontic rehabilitation in young patient is often difficult because of the anatomical abnormalities of existing teeth and alveolar ridges. The conical shaped teeth and “knife-edge” alveolar ridges result in poor retention and instability of dentures. Moreover, denture must permit jaws expansion and a correct pattern of growth. Materials and Methods. Complete removable dentures were provided to allow for normal physiological development and a corrected masticatory function. Initial maxillary and mandibular impressions were made with smallest stock trays and irreversible hydrocolloid and then final impressions ware made with light-bodied polysulfide rubber base impression material. A base of autopolymerizing resin was constructed and a wax rim was added to the base. The patient’s vertical dimension of occlusion was established by assessing phonetic and esthetic criteria. Preliminary occlusal relations were recorded, and the mandibular cast was mounted on the articulator. Acrylic resin teeth specific for children dentures were selected and mounted. The dentures were tried in and, after proper adjustments, were inserted. The patients were monitored clinically every month to fit prostheses. Cephalometric radiographs were taken every 6 month with the prostheses in place in order to evaluate correct pattern of growth. Cephalometric measurements were realized and used to evaluate the effect of rehabilitation on craniofacial growth. Cephalometric measurements of sound patients were compared with ED patients. After two month expander screws (three-way screw in the upper denture and two-way the lower one)were inserted in each denture in order to permit the expansion of the denture and the jaws growth. Where conical teeth were present, composite crown were realized and luted to improve the esthetic and phonesis. In order to improve retention the placement of endosseous implants was carried out. TC 3D Accuitomo was performed and a resin model of mandibular bone of the patient was realized. At the age of 11 years two implants were inserted into anterior mandible in a child with anodontia. Despite a remarkable multi-dimensional atrophy of the mandibular alveolar process, the insertion of two tapered screw implants (SAMO Smiler, diameter 3.8, length 10 mm). After a submerged healing period of two-three month, the implants were exposed. Implants were connected with an expansion guide that permits mandibular growth and prosthetic retention. The amount of mandibular growth was also evaluate dusing the expansion guide. Results. Early oral rehabilitation improve oral function, phonesis and esthetic, reducing social impairment. Treated patients showed normal cephalometric measurement. Early rehabilitation is able to prevent the prognatissm of the mandibula . The number of teeth was significantly related to several changes in craniofacial morphology. Discussion. In the present study the 5,3% of ED patients showed hypodontia, the l’89,4% di oligodontia, and the 5,3% di anodontia. The cephalometric analysis supports that ED patients showed midface hypoplasia. ED groups showed an increased pogonion to nasion measurement than sound patients, indicative of class III tendency. The present study demonstrated that number of teeth was significantly correlated with deviation of cephalometric measurements from normality. Oligoanodontia is responsible for changing of cephalometric measuraments also on sagittal plane with a class III tendency. Maxillary jaw showed a retrused position related to the presence of hypodontia.
Resumo:
Il lavoro che presento propone un’analisi di una chiesa africana indipendente in Italia, la Celestial Church Of Christ Worldwide (CCCW), cercando di mettere in luce il nesso tra religione, migrazione e il processo di ‘plunting churches’ (Kooning 2009) nel contesto italiano. Attraverso una ricerca sul campo, sono stati indagati i percorsi personali, familiari e comunitari dei membri di una ‘Celestial Parish’ presente nel comune di Brescia, ‘Ileri Oluwa Parish’, al fine di comprendere la natura dei processi identitari coinvolti nell’organizzazione della CCC in Italia. ‘Ileri Oluwa Parish’, in quanto luogo che denota una ‘chiesa individuale collegata ad una Diocesi’ (CCC Constitution (CCC Constitution, 107 (d) si rivela, nella materialità delle sue forme e dei ‘Devotional Services’ che in essa si svolgono, a ‘field of action’ (Lefebvre, 1991). La storia della chiesa, i fondamenti della sua dottrina e i significati comunicati attraverso le forme rituali e religiose che la stessa promuove, sono stati contestualizzati alla luce delle tensioni e delle strategie di potere che strutturano il campo. Le storie dei membri della parrocchia, percorsi di migrazione e mobilità in itinere, rappresentano la lente attraverso cui si è guardato alle relazioni vissute nel nome dello ‘Spirito’, e alla percezione stessa di ciò che gli stessi Celestians definiscono sacro, santo, puro e impuro. Lo sguardo fisso alla vita ordinaria di una Celestial parish in Italia, esteso nell’ultima parte dell’elaborato alla Celestial parish londinese, è stato fondamentale per capire l’intreccio di relazioni spirituali, reti familiari e mobilità degli individui sul territorio italiano ed europeo, processo che ribalta la condizione diasporica della CCC, trasformando una condizione di dispersione in un valore aggiunto, nella possibilità di nuove traiettorie territoriali e spazi di presenza religiosa e socioeconomica.
Resumo:
« Dieu est mort » proclame à l’envi le fou nietzschéen. C’est sous l’égide inquiète de cette assertion paroxystique, traduisant ce «malaise de la culture» qu’évoquait Freud, que la pensée, la littérature et l’art du XXe siècle européen évoluent. Cependant, le christianisme dont ce cri signe l’extrême décadence, n’est pas seul à imprégner les productions artistiques de ce siècle, même les plus prétendument athées, mais avant tout la figure du Christ - autour de laquelle sont structurés tant cette religion que son système de croyance – semble, littéralement et paradoxalement, infester l’imaginaire du XXe siècle, sous des formes plus ou moins fantasmatiques. Ce travail se propose ainsi précisément d’étudier, dans une optique interdisciplinaire entre littérature, art et cinéma, cette dynamique controversée, ses causes, les processus qui la sous-tendent ainsi que ses effets, à partir des œuvres de trois auteurs : Artaud, Beckett et Pasolini. L’objectif est de fournir une clé de lecture de cette problématique qui mette en exergue comment « la conversion de la croyance », comme la définit Deleuze, à laquelle ces auteurs participent, n’engendre pas un rejet purement profanatoire du christianisme mais, à l’inverse, la mise en œuvre d’un mouvement aussi violent que libératoire qualifié par Nancy de « déconstruction du christianisme ». Ce travail entend donc étudier tout d’abord à la lumière de l’expérience intérieure de Bataille, l’imaginaire christique qui sous-tend leurs productions ; puis, d’en analyser les mouvements et les effets en les questionnant sur la base de cette dynamique ambivalente que Grossman nomme la « défiguration de la forme christique ». Les excès délirants d’Artaud, l’ironie tranchante de Beckett et la passion ambiguë de Pasolini s’avèrent ainsi participer à un mouvement commun qui, oscillant entre reprise et rejet, débouche sur une attitude tout aussi destructive que revitalisante des fondements du christianisme.