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Previous issue date: 2017-03-03 / Coordena??o de Aperfei?oamento de Pessoal de N?vel Superior - CAPES / Cystic fibrosis (CF) has long been described as a fatal genetic disease. Currently, improvement in the care of these patients contributes to longer survival and thereby allows the observation of other symptoms related to mutations of the cystic fibrosis transmembrane chloride regulator (CFTR) gene, responsible for the disease. Although pulmonary disease continues to be the leading cause of morbidity and mortality, CF also requires that attention be given to manifestations of the disease in other organs, such as liver disease. CF-associated liver disease (CFLD) can be considered an independent risk factor for mortality and lung transplantation. The major challenge is to diagnose CFLD, because the changes may remain asymptomatic until advanced stages of the disease. There is currently no single test that is sensitive and specific enough to assess liver function, and early diagnosis of CFLD requires a combination of regular clinical examination, biochemical tests, and imaging studies. There are high expectations for noninvasive tests that are harmless to patients and can be easily repeated and compared in order to detect changes as early as possible. Imaging techniques have improved significantly over the past decade and new technologies are being incorporated into clinical practice. Ultrasonography (US), computed tomography and magnetic resonance imaging (MRI) are the main imaging modalities currently used for examination, but new MRI-based techniques have acquired great importance. Thus, it would be desirable to develop a more accurate method than US, which is the most widely used routine examination, as well as the ultrasound scoring system described by Williams et al in 1995 and used so far. This study aimed to describe possible MRI abnormalities in children with CF and correlate them with US findings, particularly in relation to parameters of parenchyma, border, and liver fibrosis. Methods: Patients with a diagnosis of CF underwent clinical examination, anthropometric measurements, US, and MRI. MRI was performed without sedation or contrast. The liver was evaluated using the scoring system described by Williams et al on US and MRI images. Other MRI features were also analyzed, including degenerative nodules, atrophy of the right hepatic lobe, and posterior notch sign of the hepatic lobe. Results: The US and MRI findings of 20 patients were analyzed. Patient age ranged from 8 to 19 years. Five patients showed US abnormalities with an ultrasound score greater than 3, 4 patients with a score of 7, and 1 patient with a score of 9. When the Williams scoring criteria were applied to MRI, 5 patients were evaluated as having a score of 9. US did not assess liver fibrosis in the same way as MRI. In the US evaluation, 4 patients received a score of 1 for this parameter, while in the MRI evaluation all patients received the maximum score. MRI revealed 4 patients with atrophy of the right hepatic lobe, 3 patients with degenerative nodules, and 2 patients with esophageal varices. Four patients (80%) showed the posterior notch sign of the hepatic lobe. Conclusion: Although there was a good correlation between US and MRI, MRI performed better than US in the assessment of liver fibrosis. The Williams scoring system can also be applied to hepatic MRI. Thus, MRI stands out as a noninvasive method that can provide further information related to CFLD, contributing to the overall assessment of patients with CF. / A fibrose c?stica (FC) foi por muito tempo descrita como uma doen?a gen?tica fatal. Atualmente, a melhora nos cuidados de sa?de destes pacientes possibilitou uma maior sobrevida e assim a observa??o de outras sintomatologias relacionadas ? muta??o do gene CFTR (cystic fibrosis transmembrane chloride regulator), respons?vel pela doen?a. Apesar da doen?a pulmonar continuar sendo a principal causa de morbidade e mortalidade, a FC exige aten??o tamb?m para manifesta??es de doen?a em outros ?rg?os, como o caso da doen?a hep?tica. A doen?a hep?tica associada ? FC (DHFC) pode ser considerada um fator de risco independente para a mortalidade e transplante pulmonar. O grande desafio ? o seu diagn?stico, pois suas altera??es podem permanecer assintom?ticas at? est?gios avan?ados da doen?a. N?o h? dispon?vel um teste que seja sens?vel e espec?fico para avaliar a fun??o hep?tica, o diagn?stico precoce da DHFC requer o conjunto de exame cl?nico regular, exames de bioqu?mica e de imagem. ? grande a expectativa por exames n?o invasivos que sejam in?cuos para o paciente e possam ser facilmente repetidos e comparados para a busca de altera??es o mais precoce poss?vel. As t?cnicas de imagem t?m melhorado significativamente na ?ltima d?cada e as novas tecnologias est?o come?ando a entrar na pr?tica cl?nica. Ultrassonografia (US), tomografia computadorizada e resson?ncia magn?tica (RM) s?o as principais modalidades usadas atualmente, mas novas t?cnicas baseadas em RM est?o em destaque. Desta forma, seria desej?vel desenvolver uma metodologia mais acurada que a US que ? o exame rotineiramente mais difundido, assim como o escore ultrassonogr?fico descrito por Williams et al em 1995, utilizado at? ent?o. Nosso objetivo ? descrever as poss?veis altera??es encontradas na RM em crian?as com FC e correlacionar com os achados da US, principalmente em rela??o aos par?metros de par?nquima, borda e fibrose hep?tica. M?todos: Pacientes com diagn?stico de fibrose c?stica realizaram avalia??o cl?nica, antropom?trica, exames de ultrassonografia e resson?ncia magn?tica. Os exames de resson?ncia magn?tica foram realizados sem seda??o e sem contraste. O f?gado foi avaliado segundo o escore descrito por Williams et al na ultrassonografia e na resson?ncia magn?tica. Foram analisados ainda outros diferentes aspectos na RM entre eles: n?dulos degenerativos, atrofia do lobo hep?tico direito, o sinal do entalhe posterior do lobo hep?tico. Resultados: Os exames de vinte pacientes foram analisadas, os participantes do estudo tinham idade entre 8 e 19 anos. Cinco pacientes apresentaram exame de US alterado com escore ultrassonogr?fico maior que 3, quatro pacientes com escore 7 e um com escore 9. Ao aplicar os crit?rios do escore de Williams na resson?ncia magn?tica, os 5 pacientes foram avaliados com escore 9. A ultrassonografia n?o pode avaliar a fibrose hep?tica da mesma forma que a resson?ncia, na US quatro pacientes receberam pontua??o 1 para o par?metro, j? na RM todos receberam pontua??o m?xima. Na RM, foram identificados quatro pacientes com atrofia do lobo hep?tico direito, tr?s pacientes com n?dulos degenerativos e 2 pacientes com varizes esof?gicas. Quatro pacientes (80%) apresentaram o sinal do entalhe posterior do lobo hep?tico direito. Conclus?o: Existe uma boa correla??o entre US e RM, por?m a RM teve melhor desempenho na avalia??o da fibrose hep?tica. O escore de Williams tamb?m pode ser aplicado para an?lise hep?tica pela RM. Assim a RM se destaca como um m?todo n?o invasivo que nos fornecer ainda outras informa??es relacionadas com a DHFC que contribuem na avali??o global do paciente com FC.
| Identifer | oai:union.ndltd.org:IBICT/oai:tede2.pucrs.br:tede/7810 |
| Date | 03 March 2017 |
| Creators | Baldissera, Marilisa |
| Contributors | Baldisserotto, Matteo |
| Publisher | Pontif?cia Universidade Cat?lica do Rio Grande do Sul, Programa de P?s-Gradua??o em Medicina/Pediatria e Sa?de da Crian?a, PUCRS, Brasil, Escola de Medicina |
| Source Sets | IBICT Brazilian ETDs |
| Language | Portuguese |
| Detected Language | English |
| Type | info:eu-repo/semantics/publishedVersion, info:eu-repo/semantics/masterThesis |
| Format | application/pdf |
| Source | reponame:Biblioteca Digital de Teses e Dissertações da PUC_RS, instname:Pontifícia Universidade Católica do Rio Grande do Sul, instacron:PUC_RS |
| Rights | info:eu-repo/semantics/openAccess |
| Relation | 3098206005268432148, 500, 500, 500, 600, -224747486637135387, -969369452308786627, 2075167498588264571 |
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