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https://repositorio.unichristus.edu.br/jspui/handle/123456789/1830Full metadata record
| DC Field | Value | Language |
|---|---|---|
| dc.contributor.advisor | Marinho, Rebeca Bastos Vasconcelos | - |
| dc.contributor.author | Dutra, Eduarda Vitória Viana | - |
| dc.date.accessioned | 2024-12-26T13:19:53Z | - |
| dc.date.available | 2024-12-26T13:19:53Z | - |
| dc.date.issued | 2024-11-26 | - |
| dc.identifier.uri | https://repositorio.unichristus.edu.br/jspui/handle/123456789/1830 | - |
| dc.description | ANDERSON, J. Transtornos do espectro de Cholbam® e Zellweger: implementação e gerenciamento do tratamento. Orphanet Journal of Rare Diseases, v. 16, p. 1–13, 2021. BRAVERMAN, N. E. et al. Peroxisome biogenesis disorders in the Zellweger spectrum: An overview of current diagnosis, clinical manifestations, and treatment guidelines. Molecular genetics and metabolism, v. 117, n. 3, p. 313– 321, 2016. CARDOSO, P. Síndrome de Zellweger com desnutrição grave, estado imunocomprometido e infecções oportunistas. Case Reports , v. [s.l: s.n.]. CRANE, D. I. Revisitando a neuropatogênese da síndrome de Zellweger. Neurochemistry international, p. 1–8, 2014. Elumalai V, Pasrija D. Zellweger Spectrum Disorder. In: StatPearls. Treasure Island (FL): StatPearls Publishing; August 2, 2020. ENNS, G. M. et al. Diagnostic challenges and disease management in patients with a mild Zellweger spectrum disorder phenotype. Molecular genetics and metabolism, v. 134, n. 3, p. 217–222, 2021. FARRÉ, J.-C. et al. Peroxisome biogenesis, membrane contact sites, and quality control. EMBO reports, v. 20, n. 1, p. e46864, 2019. FEDICK, A.; JALAS, C.; TREFF, N. R. A deleterious mutation in the PEX2 gene causes Zellweger syndrome in individuals of Ashkenazi Jewish descent: A deleterious mutation in thePEX2gene. Clinical genetics, v. 85, n. 4, p. 343–346, 2014. FRANSEN, M.; LISMONT, C.; WALTON, P. The peroxisome-mitochondria connection: How and why? International journal of molecular sciences, v. 18, n. 6, 2017. FUJIKI, Y. et al. Transtornos do espectro de Zellweger: visão geral clínica e abordagem de tratamento. Biochimica et Biophysica Acta. Molecular Cell Research, v. 10, n. 11, 2015. KLOUWER, F. C. et al. Poll-The BT. Transtornos do espectro de Zellweger: visão geral clínica e abordagem de tratamento. Orphanet J Rare Dis, v. 10, 2015. KUMAR, S. et al. Zellweger syndrome: prenatal and postnatal growth failure with epiphyseal stippling. Journal of pediatric endocrinology & metabolism, v. 27, n. 1–2, p. 185–188, 2014. LEE, P. R.; GERALD, V. Neurologia infantil: síndrome de Zellweger. Neurology, v. 20, p. e207–e210, 2013. 32 LERTSIRIVORAKUL, J.; WONGSWADIWAT, M.; TREESUWAN, P. Oral manifestations and dental management of a child with Zellweger syndrome. Special care in dentistry: official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry, v. 34, n. 1, p. 46–50, 2014. MOHAN, V. A case study through an audiological perspective on a pediatric patient diagnosed with zellweger Syndrome. Indian journal of otolaryngology and head and neck surgery: official publication of the Association of Otolaryngologists of India, v. 75, n. Suppl 1, p. 429–432, 2023. OLIVEIRA, S. et al. Uso de canabidiol como terapia adjuvante em paciente com síndrome de Zellweger: relato de caso. Medicina (Ribeirao Preto Online), v. 53, n. 3, p. 321–326, 2020. RAFIQUE M, Z. S.; MN, R.; OA, M. Síndrome de Zellweger - um distúrbio letal da biogênese do peroxissomo. J Pediatr Endocrinol Metab, v. 26, n. 3–4, p. 377–379, 2013. RAVINDRAN, R. et al. Peroxisome biogenesis initiated by protein phase separation. Nature, v. 617, n. 7961, p. 608–615, 2023. SALPIETRO, V. et al. Zellweger syndrome and secondary mitochondrial myopathy. European journal of pediatrics, v. 174, n. 4, p. 557–563, 2015. SCHIEFERDECKER, A.; WENDLER, P. Structural mapping of missense mutations in the Pex1/Pex6 complex. International journal of molecular sciences, v. 20, n. 15, p. 3756, 2019. STEINBERG, Steven J. et al. Zellweger spectrum disorder. 2020. YOGI, P. Síndrome de Zellweger: Relato de Caso. JNMA: Journal of the Nepal Medical Association, v. 270, n. 62, 2024 | pt_BR |
| dc.description.abstract | Este estudo teve como objetivo realizar uma revisão de literatura acerca dos achados clínicos gerais e a visão odontológica no contexto do tratamento multidisciplinar em pacientes com Síndrome de Zellweger. Foi feito uma busca por artigos nas línguas portuguesa e inglesa, nos anos de 2009 a 2024 através da base de dados PuBMED, utilizando os descritores: Síndrome de Zellweger, Odontologia, Medicina, Nutrição e Fonoaudiologia. Foram achados 167 artigos, dentre os quais 22 foram selecionados seguindo os critérios de inclusão. A Síndrome de Zellweger é uma doença rara causada pelos distúrbios nos peroxissomos, tornando-se possível observar suas alterações no período neonatal e na primeira infância que requerem tratamento multidisciplinar precoce. Os pacientes são caracterizados principalmente com dismorfismo craniofacial típico e anormalidades neurológicas. Por apresentarem um prognóstico ruim, geralmente acabam não resistindo e vindo a óbito no primeiro ano de vida. Além disso as manifestações orais são escassas e apenas o palato arqueado alto, possível presença de fissura palatina e a micrognatia são frequentemente mencionados. Com base nos estudos avaliados, concluiu-se que embora haja variados relatos na literatura relacionados as áreas de saúde como: medicina, nutrição, as informações sobre as manifestações orais e a abordagem odontológica são pouco relatadas. Ademais, atualmente não há terapia curativa para tal síndrome, os cuidados são voltados essencialmente ao controle dos sintomas. Contudo, os pacientes apresentam baixa expectativa de vida e consequentemente não conseguem passar por uma avaliação e seguir em tratamento odontológico, inviabilizando seguir o plano de cuidado em conjunto com a assistência multidisciplinar. | pt_BR |
| dc.language.iso | pt_BR | pt_BR |
| dc.subject | Síndrome de Zellweger | pt_BR |
| dc.subject | Oodntologia | pt_BR |
| dc.subject | Medicina | pt_BR |
| dc.subject | Nutrição | pt_BR |
| dc.subject | Fonaudiologia | pt_BR |
| dc.title | SÍNDROME DE ZELLWEGER: VISÃO ODONTOLÓGICA NO CONTEXTO DO TRATAMENTO MULTIDISCIPLINAR – REVISÃO DE LITERATURA | pt_BR |
| dc.type | TCC | pt_BR |
| dc.title.ingles | ZELLWEGER SYNDROME: DENTAL VIEW IN CONTEXT OF MULTIDISCIPLINARY TREATMENT – REVIEW OF LITERATURE | pt_BR |
| dc.description.resumo_abstract | This study aimed to carry out a literature review about general clinical findings and dental vision in the context of multidisciplinary treatment in patients with Zellweger Syndrome. A search was carried out for articles in Portuguese and English, from 2009 to 2024 through the PuBMED database, using the descriptors: Zellweger Syndrome, Dentistry, Medicine, Nutrition and Speech Therapy. 167 articles were found, of which 22 were selected following the inclusion criteria. Zellweger Syndrome is a rare disease caused by disorders in peroxisomes, making it possible to observe changes in the neonatal period and early childhood that require early multidisciplinary treatment. Patients are mainly characterized with typical craniofacial dysmorphism and neurological abnormalities. Because they have a poor prognosis, they generally end up not resisting and dying within the first year of life. Furthermore, oral manifestations are scarce and only the high arched palate, possible presence of cleft palate and micrognathia are frequently mentioned. Based on the studies evaluated, it was concluded that although there are varied reports in the literature related to health areas such as: medicine, nutrition, information on oral manifestations and dental approach is little reported. Furthermore, there is currently no curative therapy for this syndrome, care is essentially aimed at controlling symptoms. However, patients have a low life expectancy and are consequently unable to undergo an assessment and continue dental treatment, making it impossible to follow the care plan in conjunction with multidisciplinary care. | pt_BR |
| Appears in Collections: | Odontologia - Trabalhos de Conclusão de Curso - Campus PARQUE ECOLÓGICO | |
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| File | Description | Size | Format | |
|---|---|---|---|---|
| DutraEduarda.pdf | 555.69 kB | Adobe PDF |  View/Open |
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