Escore de Williams aplicado na ressonância magnética para avaliação hepática de crianças com fibrose cística

• Ano de defesa: 2017
• Autor(a) principal: Baldissera, Marilisa
• Orientador(a): Baldisserotto, Matteo
• Tipo de documento: Dissertação
• Tipo de acesso: Acesso aberto
• Idioma: por
• Instituição de defesa: Pontifícia Universidade Católica do Rio Grande do Sul
• Programa de Pós-Graduação: Programa de Pós-Graduação em Medicina/Pediatria e Saúde da Criança
• Departamento: Escola de Medicina
• País: Brasil
• Palavras-chave em Português: Doença Hepática Associada à Fibrose Cística; Ultrassonografia; Ressonância Magnética
• Área do conhecimento CNPq: CIENCIAS DA SAUDE::MEDICINA
• Link de acesso: http://tede2.pucrs.br/tede2/handle/tede/7810

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.