Orientador: Heron Fernando de Sousa Gonzaga / Tese (doutorado) - Universidade Estadual de Campinas, Faculdade de Odontologia de Piracicaba / Made available in DSpace on 2018-08-10T16:40:54Z (GMT). No. of bitstreams: 1 Rodrigues_Marcelo_D.pdf: 760160 bytes, checksum: 48a5a2282ffba53fa69fd0f990fa8ed0 (MD5) Previous issue date: 2008 / Resumo: A hialinose cutâneo-mucosa (HCM) é uma dermatose autossômica recessiva rara, congênita, de aparecimento precoce na infância. Caracteriza-se por deposição de material hialino, que se acumula na pele e mucosas. Foram descritos 258 casos na literatura mundial e no Brasil, doze casos. A HCM apresenta aumento da expressão do colágeno tipo IV e V e redução da expressão do colágeno I e III. Esta doença foi mapeada no locus do cromossomo 1q21. Mutações patogênicas foram identificadas no gene ECM1. A doença é caracterizada por voz rouca, pápulas cutâneas amareladas, cicatrizes atróficas, lesões ulceradas cutâneas, blefarose moniliforme e conjuntivite pseudo-membranosa. Manifesta multiplicidade de sintomas, afetando vários órgãos. A revisão da literatura sobre HLA e HCM não mostrou nenhum trabalho. Realizou-se o estudo clínico e das especificidades HLA na família de uma afetada por HCM. Foram submetidos a exames clínicos e complementares. A tipificação HLA foi realizada através da análise de DNA genômico pela técnica de PCR-SSP. A afetada era branca, com 14 anos. Referia choro rouco desde o nascimento e eritema na região subescapular e cervical há 13 anos, que evoluiu para vesículas e posteriormente, micropápulas, que se rompiam, evoluindo para lesões ulceradas. Nesses locais, apareciam verrucosidades nos ângulos da boca, joelhos, cotovelos e regiões palmares. Concomitantemente, evoluiu para micropápulas hipocrômicas no dorso das mãos e axilas. Referia disfagia. Negava história familiar. Ao exame dermatológico, pápulas amareladas nas pálpebras e linearmente na borda livre; cicatrizes atróficas varioliformes nas regiões antecubitais; placas verrucosas nos cotovelos, regiões palmares, joelhos; pápulas amareladas em dorso das mãos e região cervical posterior. Apresentava alopecia parieto-occipital. Ao exame bucal, abertura limitada, endurecimento labial, lesões vegetantes no ângulo da boca, placas branco-amareladas em regiões de mucosa jugal, vestibular, orofaringe, dorso e ventre da língua. Ulcerações linguais e no palato duro e mole. Xerostomia discreta foi observada. Ao exame dentário, placa bacteriana, cálculos e agenesia do dente 22. A gengiva era friável, hipertrófica com bolsas periodontais. Ao exame oftalmológico, obstrução das vias lacrimais, mucosa espessada nos pontos lacrimais e prurido periocular. Diagnóstico psiquiátrico de Distimia foi estabelecido. Na avaliação neurológica, cefaléia. O exame clínico nos outros membros da família não mostrou nenhum traço da doença. Na fibronasofaringolaringoscopia, espessamento epidérmico em epiglote e hipertrofia de falsas cordas, pregueamento mucoso em prega interaritenóidea. O EEG não mostrou anormalidades. A tomografia computadorizada apresentou calcificações parenquimatosas hipocampais bilaterais. O exame histopatológico confirmou o diagnóstico de HCM. Constatou-se que sendo a doença autossômica recessiva, a ausência de manifestações clínicas nos pais da afetada, mostrou serem os mesmos heterozigotos. As manifestações clínicas são importantes para o diagnóstico. A primeira manifestação clínica mais freqüente na literatura, apresentada pela afetada, é a rouquidão. As manifestações clínicas mais exuberantes na doença são as cutâneas e mucosas. O estudo das especificidades HLA determinou os seguintes haplótipos na afetada: A31-B39-Cw7-DR4-DQ8 e A74-B7-Cw7-DR8-DQ-. A paciente era haploidêntica a um dos seus dois meioirmãos. Este foi o primeiro trabalho em que se investigou especificidades HLA em portador de HCM / Abstract: Cutaneous-mucous hyalinosis (CMH) is a rare congenital autosomal recessive dermatose, which is presented precociously in early childhood. It is characterized by disposition of hyaline material that accumulates in the skin and cutaneous mucous. 258 cases were described in the world and 12 cases in Brazil. The CMH presents an increase of the expression of the collagen types IV and V and reduction of the expression of the collagen I and II. The disorder was mapped in a locus on chromosome 1q21 and pathogenic mutations were identified in the ECM1 gene. The disease is characterized by hoarse voice, yellowed cutaneous papules, atrophic scars, ulcerated cutaneous lesions, moniliform blepharosis, and pseudomembranous conjunctivitis. It manifests multiple symptoms affecting several organs. The review of literature about HLA as well as CMH has not presented any work so far. The clinical study as well as of the HLA antigens in the family of an individual affected by CMH was performed. They were submitted to clinical and auxiliary tests. The HLA type was accomplished through the analysis of genome DNA by the technique of PCR-SSP. The proband was a 14 year old white female. It referred hoarse crying from the birth and erythema in the sub-omoplate and cervical area 13 years ago which developed for vesicles and later on, micropapules that broke up, developing for ulcerated lesions, verrucous lesions in the angles of the mouth, knees, elbows and palm areas appeared. Concomitantly it developed for hypochromic micropapules in the back of the hands and armpits. It referred dysphagia. There was no history family. During the dermatological examination she presented yellowed papules in the eyelids and lineally in the free border; atrophic varioliform scars in the ante-cubital areas; verrucous plates in the elbows, palm areas and knees: yellowed papules in the back of the hands and posterior cervical area. She showed Parietal-occipital alopecia. During the oral examination she Presented limited opening, labial hardening, vegetative lesions in the angle of the mouth, white yellowed plaques in the areas of buccal, vestibular, oro-pharynx, dorsal and ventral tongue mucous membranes. Tongue ulcerations and also in the hard and soft palate. Discreet xerostomia was observed. During the dental examination, bacterial plaque, calculous and agenesis of the tooth 22. The gengive was friable, hypertrophic with periodontal depressions. During the ophthalmic examination, obstruction of the lacrimal via, thickened mucous in the lacrimal vias and perio-ocular pruritis. Psychiatric diagnosis of Dysthymia was estableshed. In the neurological evaluation, migraine. The clinical examination in the other members of the family did not show any line of the disease. Epidermal thickening in the epiglottis and hypertrophy of false strings, mucous pleatment in interarytenoide pleat were found. EEG did not show abnormalities. Computed tomography presented bilateral parenchymatosis hippocampus calcifications. The histopathology examination confirmed the diagnosis of CMH. It was verified because of the disease was recessive autosomal, the absence of clinical manifestations in the parents of the affected individual who were heterozygote. The clinical manifestations are important for the diagnosis. The most frequent clinical manifestation in the literature, presented by the affected girl, is the hoarse voice. The most exuberant clinical manifestations in the disease are the cutaneous and the mucous ones. The study of the HLA antigens determined the following haplotypes in the affected girl: A31-B39-Cw7-DR4-DQ8 and A74-B7-Cw7DR8-DQ- . She is haplo-identical to one of two her middle-siblings. This was the first work in which HLA antigens were investigated in carrier of CMH / Doutorado / Estomatologia / Doutor em Estomatopatologia

No description available.

Wide bandgap perovskites are emerging as suitable candidates for the technology of tandem solar cells. Understanding their optical properties is a prerequisite for improving the corresponding solar cells’ efficiencies. In this thesis, we employ various steady-state spectroscopies to reveal the optical properties of two wide bandgap perovskites: FA0.83Cs0.17Pb(I0.7Br0.3)3 or PVK1 and FA0.83Cs0.17Pb(I0.5Br0.5)3 or PVK2. The optical properties of interest are the semiconductors’ absorption spectra, the sub-bandgap absorption features, the bandgap energy, the Urbach energy, and the excitonic binding energy. We find that the sub-bandgap absorption can be characterized by a single exponential function. We also find that the Urbach energies and the excitonic binding energies are below the thermal energy at room temperature, which signals that PVK1 and PVK2 are excellent nominees for photovoltaic absorbers. Finally, the bandgap energy is red shifted due to excitonic effects as revealed by the Elliot model.

No description available.

Urbach-Wiethe Sindroom (beter bekend as lipo"ied prote"inose of hyalinosis cutis et mucosae) is 'n seldsame, outosomaal-oorerflike siekte. Die kenmerkendste simptome van die siekte is vel- en slymvliesveranderinge wat deur 'n neerslag van ekstrasellulere hialienagtige materiaal van onbekende oorsprong veroorsaak word. Die vel word maklik beseer, genees stadig en lelike, pokagtige letsels ontstaan. 'n Fyn, korrelagtige neerslag op die ooglede, die sogenaamde "kralestringvoorkoms" kenmerk die siekte, sowel as heesheid, die prominentste en lastigste simptoom, wat sedert geboorte teenwoordig kan wees. Radiografie en tomografie toon bilaterale,boontjievormige verkalking op die temporale lobbe van die brein, wat tot epileptiese aanvalle en ander neuropsigiese simptome soos geremde geheue en aggressie lei. Heesheid veroorsaak kommunikatiewe beperkinge vir die lyer,terwyldie opsigtelike velletsels aversiewe- en die verkalkings onsigbare beperkinge meebring. Die lyer aan Urbach-Wiethe Sindroom kan volgens die beginsels en kriteria van die medies-kliniese, die persoonsgeorienteerde en die sosio-omgewingsperspektiewe gestremdheid ondervind. Teoreties kan die lyer se belewinge van sy andersheid en die nie-aanvaardingdeurdie gemeenskap, soos by gestremdes,die handhawing van sy selfagting rem, sodat 'n negatiewe selfkonsep tot skuldgevoelens, angsbelewinge en depressie kan lei. 'n Betekenisvolle verband is in die empiriese ondersoek tussen die graad van aantasting en wanaanpassing in die lyer se leefwereld gevind. Die lyers wat ernstig aangetas is, identifiseer moeilik met hulle fisieke voorkoms. ldentiteitsvorming word gerem en die selfagting is negatief. Hulle openbaar 'n negatiewe selfkonsep en depressiewe gevoelens met selfmoordgedagtes. Hulle relasies en sosialisering is problematies en hulle voel hulle word nie deur die gemeenskap aanvaar nie. Die lyers ondervind 'n algemene wanaanpassing in hulle leefwereld. 'n Geval van paranoia is ook gevind. Alhoewel die ouers vrae oor die toestand het en sekere emosies beleef, kan die meeste van hulle die situasie hanteer. Riglyne vir 'n terapeutiese begeleidingsprogram vir lyers aan Urbach-Wiethe Sindroom, wat op die verbetering van die selfkonsep; die hantering van depressie, aggressie en woedebuie, asook die verbetering van relasies en sosialisering gerig is, is saamgestel. 'n Ondersteuningsgroep waarby lyers en hulle ouers kan inskakel, is gestig. / Urbach-Wiethe Syndrome, also known as lipoid proteinosis or hyalinosis cutis et mucosae, is a rare, recessively inherited, autosomal disorder characterized by lesions of the skin and mucosae, caused by widespread deposition of hyaline material of unknown etiology. The skin injures easily and heals slowly with "pock­ like" lesions. Bead-like deposits on the eyelids, called "string of pearls",are often found. Present since early infancy, hoarseness is the first, and most striking, irritating symptom. Bean-shaped intracranial calcification within the temporallobes of the brain in the area of the hippocampus, shown up by radiography and tomography, may cause epileptic seizures and other neuropsychological complications like impaired memory and aggression. The sufferer experiences communicative impairment through hoarseness, aversive impairment because of the conspicuous lesions, and concealed impairment as a result of the calcifications. According to the medical-clinical,person-orientated and socio-environmental perspectives on disability, a sufferer of Urbach-Wiethe Syndrome may experience disability. Like disabled persons, the sufferer finds it difficult to identify with his physical appearance. Impaired identity formation and low self-esteem cause a negative self­ concept. Feelings of guilt,anxiety and depression result from perceived dissimilarity, social rejection and low self-esteem. Socialising is adversely affected. A significant relationship was found in the empirical study between the extent to which the sufferer is affected and the degree of maladjustment in his life-world. Seriously affected sufferers display a negative self-concept and feelings of depression with suicidal thoughts. Socialisation and relationships are problematic and sufferers feel unaccepted by the community. General maladjustment in the life-world is experienced. A case of paranoia was reported. Except for some questions and unresolved feelings about the disease,most parents are able to cope with the situation. Guidelines have been set for a therapeutic programme for sufferers from Urbach­ Wiethe Syndrome aimed at enhancing self-concept, coping with depression and aggression, and improving relationships and socialising. A support group has been ounded for sufferers and their parents. / Psychology of Education / D. Ed. (Sielkundige Opvoedkunde)

Urbach-Wiethe Sindroom (beter bekend as lipo"ied prote"inose of hyalinosis cutis et mucosae) is 'n seldsame, outosomaal-oorerflike siekte. Die kenmerkendste simptome van die siekte is vel- en slymvliesveranderinge wat deur 'n neerslag van ekstrasellulere hialienagtige materiaal van onbekende oorsprong veroorsaak word. Die vel word maklik beseer, genees stadig en lelike, pokagtige letsels ontstaan. 'n Fyn, korrelagtige neerslag op die ooglede, die sogenaamde "kralestringvoorkoms" kenmerk die siekte, sowel as heesheid, die prominentste en lastigste simptoom, wat sedert geboorte teenwoordig kan wees. Radiografie en tomografie toon bilaterale,boontjievormige verkalking op die temporale lobbe van die brein, wat tot epileptiese aanvalle en ander neuropsigiese simptome soos geremde geheue en aggressie lei. Heesheid veroorsaak kommunikatiewe beperkinge vir die lyer,terwyldie opsigtelike velletsels aversiewe- en die verkalkings onsigbare beperkinge meebring. Die lyer aan Urbach-Wiethe Sindroom kan volgens die beginsels en kriteria van die medies-kliniese, die persoonsgeorienteerde en die sosio-omgewingsperspektiewe gestremdheid ondervind. Teoreties kan die lyer se belewinge van sy andersheid en die nie-aanvaardingdeurdie gemeenskap, soos by gestremdes,die handhawing van sy selfagting rem, sodat 'n negatiewe selfkonsep tot skuldgevoelens, angsbelewinge en depressie kan lei. 'n Betekenisvolle verband is in die empiriese ondersoek tussen die graad van aantasting en wanaanpassing in die lyer se leefwereld gevind. Die lyers wat ernstig aangetas is, identifiseer moeilik met hulle fisieke voorkoms. ldentiteitsvorming word gerem en die selfagting is negatief. Hulle openbaar 'n negatiewe selfkonsep en depressiewe gevoelens met selfmoordgedagtes. Hulle relasies en sosialisering is problematies en hulle voel hulle word nie deur die gemeenskap aanvaar nie. Die lyers ondervind 'n algemene wanaanpassing in hulle leefwereld. 'n Geval van paranoia is ook gevind. Alhoewel die ouers vrae oor die toestand het en sekere emosies beleef, kan die meeste van hulle die situasie hanteer. Riglyne vir 'n terapeutiese begeleidingsprogram vir lyers aan Urbach-Wiethe Sindroom, wat op die verbetering van die selfkonsep; die hantering van depressie, aggressie en woedebuie, asook die verbetering van relasies en sosialisering gerig is, is saamgestel. 'n Ondersteuningsgroep waarby lyers en hulle ouers kan inskakel, is gestig. / Urbach-Wiethe Syndrome, also known as lipoid proteinosis or hyalinosis cutis et mucosae, is a rare, recessively inherited, autosomal disorder characterized by lesions of the skin and mucosae, caused by widespread deposition of hyaline material of unknown etiology. The skin injures easily and heals slowly with "pock­ like" lesions. Bead-like deposits on the eyelids, called "string of pearls",are often found. Present since early infancy, hoarseness is the first, and most striking, irritating symptom. Bean-shaped intracranial calcification within the temporallobes of the brain in the area of the hippocampus, shown up by radiography and tomography, may cause epileptic seizures and other neuropsychological complications like impaired memory and aggression. The sufferer experiences communicative impairment through hoarseness, aversive impairment because of the conspicuous lesions, and concealed impairment as a result of the calcifications. According to the medical-clinical,person-orientated and socio-environmental perspectives on disability, a sufferer of Urbach-Wiethe Syndrome may experience disability. Like disabled persons, the sufferer finds it difficult to identify with his physical appearance. Impaired identity formation and low self-esteem cause a negative self­ concept. Feelings of guilt,anxiety and depression result from perceived dissimilarity, social rejection and low self-esteem. Socialising is adversely affected. A significant relationship was found in the empirical study between the extent to which the sufferer is affected and the degree of maladjustment in his life-world. Seriously affected sufferers display a negative self-concept and feelings of depression with suicidal thoughts. Socialisation and relationships are problematic and sufferers feel unaccepted by the community. General maladjustment in the life-world is experienced. A case of paranoia was reported. Except for some questions and unresolved feelings about the disease,most parents are able to cope with the situation. Guidelines have been set for a therapeutic programme for sufferers from Urbach­ Wiethe Syndrome aimed at enhancing self-concept, coping with depression and aggression, and improving relationships and socialising. A support group has been ounded for sufferers and their parents. / Psychology of Education / D. Ed. (Sielkundige Opvoedkunde)

Hexagonal boron nitride (h-BN) thin films are deposited by plasma enhanced chemical vapor deposition (PECVD). Effects of heat treatment and source gases on the structure and physical properties are investigated. Chemical bonding is analyzed in comparison with the better understood isoelectronic carbon compound, graphite. It seems that the basic difference between h-BN and graphite arises from the different electronegativities of boron and nitrogen atoms. Optical absorptions in UV-visible range for crystalline and amorphous structures are outlined. The expressions used for the evaluation of mechanical stress induced in thin films are derived. The deposited films are considered to be turbostratic as they do not exhibit the characteristic optical absorption spectra of a crystal. A new system, stylus profilometer, is implemented and installed for thin film thickness and mechanical stress measurements. Hydrogen atom density within the films, estimated from FTIR spectroscopy, is found to be a major factor affecting the order and mechanical stress of the films. Heat treatment of the films reduces the hydrogen content, does not affect the optical gap and slightly increases the Urbach energy probably due to an increased disorder. Increasing the nitrogen gas flow rate in the source gas results in more ordered films. The virtual crystal of these films is detected to be unique. Relative bond concentrations of the constituent elements indicate a ternary boron-oxygen-nitrogen structure. The physical properties of h-BN such as high resistivity and wide band gap seem suitable for optoelectronic applications such as gate dielectrics in thin film transistors and light emitting devices in the blue region.

No description available.

No description available.

Notre travail s'intègre dans les recherches actuelles sur la continuité optique du traitement et du transport de l'information. Une des techniques visant à assurer cette continuité optique consiste à réaliser une commutation de voies par déflexion holographique. Nous avons étudié théoriquement et expérimentalement la déflexion d'impulsions laser d'une durée voisine de 20 picosecondes par des réseaux photoinduits dans les semiconducteurs. Après avoir décrit et caractérisé les dispositifs expérimentaux, nous présentons les performances de deux matériaux massifs II-VI, CdZnTe (à la longueur d'onde 0,8micron) et HgCdTe (à la longueur d'onde 1,5micron). Dans le domaine spectral de la queue d'Urbach, l'efficacité de diffraction au premier ordre atteint sept pour cent pour les deux matériaux. L'ensemble des résultats expérimentaux est expliqué par un modèle basé sur l'écrantage statique des microchamps électriques locaux dus aux phonons LO. Dans un second temps, nous étudions les réseaux plasma photogénérés dans des puits quantiques InGaAs / InGaAsP inclus dans une cavité de Fabry-Pérot. Les résultats sont comparés à une modélisation prenant en compte les effets de cavité. Ceux-ci induisent une amplification du signal diffracté dont les limites sont discutées. Nous présentons enfin des résultats de modulation de transmission optique dans des puits quantiques CdTe / CdZnTe sous polarisation externe. Nous montrons que les porteurs photogénérés écrantent le champ électrique appliqué produisant ainsi une variation d'absorption par disparition de l'effet Stark dans les puits. Après avoir introduit des coefficients de mérite, nous comparons les performances des différentes structures.