Submitted by Caroline Xavier (caroline.xavier@pucrs.br) on 2017-06-30T18:05:50Z No. of bitstreams: 1 DIS_MAGALI_SANTOS_LUMERTZ_PARCIAL.pdf: 464107 bytes, checksum: 94b0248ad407b0c9f5f68f836d16f6a6 (MD5) / Approved for entry into archive by Caroline Xavier (caroline.xavier@pucrs.br) on 2017-06-30T18:06:00Z (GMT) No. of bitstreams: 1 DIS_MAGALI_SANTOS_LUMERTZ_PARCIAL.pdf: 464107 bytes, checksum: 94b0248ad407b0c9f5f68f836d16f6a6 (MD5) / Made available in DSpace on 2017-06-30T18:06:10Z (GMT). No. of bitstreams: 1 DIS_MAGALI_SANTOS_LUMERTZ_PARCIAL.pdf: 464107 bytes, checksum: 94b0248ad407b0c9f5f68f836d16f6a6 (MD5) Previous issue date: 2017-03-29 / Coordena??o de Aperfei?oamento de Pessoal de N?vel Superior - CAPES / Cystic fibrosis (CF) is a genetic disease which compromises the function of various organs. Respiratory system involvement is the main cause of morbidity and mortality in these patients. As in many chronic respiratory conditions, impairment of ventilation manifests initially during sleep. Sleep disordered breathing can lead to poorer control of the disease, by increasing local inflammation and respiratory exacerbations, reducing pulmonary function and impairing gas exchange, contributing to worse quality of life. Although relevant, there are limitations and contradictory findings about presence of sleep disordered breathing in pediatric CF population, due to small number of studies involving them and limited report of capnographic evaluation. Thus, the objectives of the study were to describe frequency of respiratory sleep disorders in pediatric patients with CF and to verify association between polysomnography findings (PSG) and other variables routinely assessed in waking period. From ninety one patients of multidisciplinary CF center at Hospital S?o Lucas Hospital (PUCRS), 54 individuals could be included in upper airway (UA) and sleep project. Of these, there was information regarding UA in 48 charts and about sleep (from PSG results recordings) in 16. All patients with PSG were included in the present study. Mean age was 11?5.6 years old, body mass index (in Z score) was 0,13?0,61 and expiratory volume in the first second (FEV1) mean was 87.95?26.21% of predicted. We found that FEV1 was correlated with mean sleep oxyhemoglobin saturation (r = 0.602, p 0.023) and with peak end-tidal carbon dioxide (EtCO2) in sleep (r = -0.645, p 0.024). In addition, presence of chronic airway colonization by Pseudomonas aeruginosa (PA) was associated with the mean EtCO2 in non-REM sleep (33?2.77 mmHg in those without colonization versus 37?1.41 mmHg in patients with chronically PA; p 0.024). Furthermore, apnea and hypopnea index per hour of sleep correlated with nadir of SaO2 (r= -0.593, p 0.015). Although our main limitation has been small sample size, we know PSG is a difficult access and execution examination, especially for pediatric population, so that previous studies involving children generally include samples between 10 to 40 CF subjects (most without sleep capnography). Finally, findings of this study corroborate previous reports suggesting sleep could serve as an early marker for respiratory disease progression in CF, although further studies are needed to assess these variables and mainly to analyze sleep carbon dioxide exchange. Thus, we conclude some groups that may benefit more from this evaluation (PSG), such as those colonized by Pseudomonas aeruginosa and those with persistent reduction in pulmonary function (especially FEV1), if not the general population of CF patients. / Fibrose c?stica (FC) ? uma doen?a gen?tica, que compromete a fun??o de diversos ?rg?os epiteliais. O acometimento do sistema respirat?rio ? a principal causa de morbidade e mortalidade nestes pacientes. Como em muitas afec??es respirat?rias cr?nicas, o comprometimento da ventila??o manifesta-se inicialmente durante o sono. A presen?a de altera??o no sono pode estar associada a um pior controle da doen?a, atrav?s de maior inflama??o local, aumento na frequ?ncia das exacerba??es respirat?rias, redu??o da fun??o pulmonar e preju?zo nas trocas gasosas, contribuindo, tamb?m, para uma piora na qualidade de vida. Embora relevantes, na literatura verificam-se achados contradit?rios e limita??es quanto ? descri??o da presen?a de dist?rbios respirat?rios do sono na popula??o pedi?trica com FC e reduzido n?mero de trabalhos com relato de avalia??o capnogr?fica. Sendo assim, os objetivos do presente estudo foram descrever a frequ?ncia de dist?rbios respirat?rios do sono em pacientes pedi?tricos portadores de FC em acompanhamento ambulatorial e verificar a associa??o entre os achados de polissonografia (PSG) e outras vari?veis avaliadas rotineiramente no per?odo de vig?lia. Dos 91 pacientes do centro multidisciplinar de FC do Hospital S?o Lucas da Pontif?cia Universidade Cat?lica do Rio Grande do Sul (PUCRS), 54 indiv?duos puderam ser inclu?dos no projeto de avalia??o da via a?rea superior (VAS) e sono. Destes, havia informa??es referentes ?s VAS em 48 prontu?rios e ao sono (atrav?s do registro do resultado de exame de PSG), em 16. Todos os pacientes com PSG foram inclu?dos neste estudo e apresentavam as seguintes caracter?sticas: idade m?dia 11?5,6 anos, ?ndice de massa corporal (em Z escore) de 0,13?0,61 e volume expirat?rio no primeiro segundo (VEF1) m?dio de 87,95?26,21% do predito. Verificamos que o VEF1 foi correlacionado ? m?dia da satura??o de oxihemoglobina no sono (r= 0,602, p 0,023) e ao pico da estimativa do g?s carb?nico (CO2) exalado (EtCO2) no sono (r= -0,645, p 0,024). Al?m disso, a presen?a de coloniza??o cr?nica das vias a?reas por Pseudomonas aeruginosa (PA) apresentou associa??o com a m?dia do EtCO2 no sono n?o REM (33?2,77mmHg nos pacientes sem coloniza??o versus 37?1,41mmHg nos com PA cronicamente; p 0,024). Ainda, o ?ndice de apneia e hipopneia por hora de sono se correlacionou com o nadir de SatO2 (r= -0,593, p 0,015). Embora nossa principal limita??o tenha sido o pequeno tamanho da amostra, sabemos que a PSG ? um exame de dif?ceis acesso e execu??o, especialmente para a popula??o pedi?trica, tanto que estudos pr?vios envolvendo crian?as incluem, em geral, amostras entre 10 e 40 indiv?duos com FC (a maior parte sem avalia??o da capnografia noturna). Por fim, os achados do presente estudo corroboram publica??es pr?vias que sugerem que a avalia??o do sono poderia servir como um precoce indicativo da progress?o da doen?a respirat?ria em FC, embora ainda sejam necess?rios mais estudos que avaliem essas vari?veis e, especialmente, que analisem a medida do CO2 noturno. Assim, conclu?mos que, se n?o a popula??o geral de fibroc?sticos, alguns grupos possam se beneficiar mais desta avalia??o (PSG) em espec?fico, como aqueles colonizados por Pseudomonas aeruginosa e os com redu??o persistente de ?ndices de fun??o pulmonar, sobretudo do VEF1.

Submitted by PPG Pediatria e Sa?de da Crian?a (pediatria-pg@pucrs.br) on 2017-08-23T20:19:40Z No. of bitstreams: 1 Tese MClaraversaofinal.pdf: 1140193 bytes, checksum: 36fe50dffe28e4c997ea7042cbe7267f (MD5) / Approved for entry into archive by Caroline Xavier (caroline.xavier@pucrs.br) on 2017-08-24T12:24:03Z (GMT) No. of bitstreams: 1 Tese MClaraversaofinal.pdf: 1140193 bytes, checksum: 36fe50dffe28e4c997ea7042cbe7267f (MD5) / Made available in DSpace on 2017-08-24T12:28:33Z (GMT). No. of bitstreams: 1 Tese MClaraversaofinal.pdf: 1140193 bytes, checksum: 36fe50dffe28e4c997ea7042cbe7267f (MD5) Previous issue date: 2017-03-31 / Coordena??o de Aperfei?oamento de Pessoal de N?vel Superior - CAPES / Introduction: Cystic fibrosis is an autosomal recessive genetic disorder characterized mainly by obstruction and inflammation of the airways leading to the chronic establishment of microorganisms. Acute infections (exacerbations) and/or chronic colonization lead to a progressive decline in lung function that leads to respiratory failure, the main cause of death among these individuals. Despite advances in survival, aspects related to infections still need to be reviewed. Thus, the main objective of the study was to evaluate the impact of different bacterial colonizations on nutritional status, lung function and clinical severity in patients with cystic fibrosis (CF). Methods: This is a retrospective cohort study that included patients with a confirmed diagnosis of CF, aged > 3 years and presenting complete data in a reference database of cystic fibrosis and / or available medical records. Anthropometric information (weight and height for the purpose of calculating BMI) and nutritional status, clinical severity data, colonization of airway secretions in addition to lung function (FEV1, FVC and FEV1/FVC). All the data collected were the annual evaluation of these subjects, between the period from 2009 to 2013. For statistical purposes, the Friedman test (Dunn's post-test) and the Mann Whitney U test were used. Results: We included 68 patients with CF out of a total of 80 who are regularly followed by the service, with 57.4% being male with a mean age of 10.35 years at baseline. During the five-year follow-up (mean of 3.55 years), nutritional status (p = 0.743) and clinical severity score (p = 0.235) remained stable. There was a statistically significant reduction of FEV1/FVC ratio over the follow-up (from year 2 to year 3 and year 2 to year 5; p <0.05). The most frequent colonizations were oxacillin-sensitive Staphylococcus aureus (MSSA) (29.8-46.3%), followed by non-mucoid Pseudomonas aeruginosa (PA) (8.5-19.4%), Burkholderia Cepacia Complex CBC) (4.9-21.3%), mucoid PA (2,4-6,5 %) and oxacilina/meticilin-resistent Staphylococcus aureus (MRSA) (2,4- 16,1%). Patients who were colonized throughout the study by MRSA (p = 0.040) had a reduction in FEV1 (p = 0.004) and FVC (p = 0.005) in year 5 compared to those not colonized by this bacteria. In addition, individuals with non-mucoid BP had a reduction (p = 0.007) in FVC. Conclusions: Our findings demonstrate that bacterial colonization by oxacilina/meticilin-resistent Staphylococcus aureus and non-mucoid Pseudomonas aeruginosa is related to worsening lung function (FEV1 and FVC). However, the impact of the different colonizations on BMI and on the clinical severity of Shwachman-Kulczycki score over the 5 years was not observed. Key words: Microbiology, nutritional status, respiratory function tests, prognosis, cystic fibrosis. / Introdu??o: A fibrose c?stica ? uma doen?a gen?tica autoss?mica recessiva, caracterizada principalmente por obstru??o e inflama??o das vias a?reas que leva a instala??o cr?nica de microrganismos. As infec??es agudas e/ou coloniza??es cr?nicas levam a um progressivo decl?nio da fun??o pulmonar que evolui para fal?ncia respirat?ria, principal causa de morte entre estes indiv?duos. Apesar dos avan?os alcan?ados na sobrevida, aspectos relacionados a infec??es ainda necessitam ser revisados. Assim, o objetivo principal do estudo foi avaliar o impacto de diferentes coloniza??es bacterianas sobre o estado nutricional, fun??o pulmonar e gravidade cl?nica em pacientes com fibrose c?stica (FC). M?todos: Trata-se de um estudo de coorte, retrospectivo onde foram inclu?dos pacientes com diagn?stico confirmado de FC, com idade > 3anos e que apresentassem os dados completos em um banco de dados de um servi?o de refer?ncia em fibrose c?stica e/ou dispon?veis nos prontu?rios m?dicos. Foram coletadas informa??es antropom?tricas (peso e estatura para fins de c?lculo de IMC) e verifica??o do estado nutricional, dados cl?nicos de gravidade, coloniza??es presentes nas secre??es das vias a?reas al?m da fun??o pulmonar (VEF1, CVF e VEF1/ CVF). Todos os dados coletados se tratavam da avalia??o anual desses sujeitos, entre o per?odo de 2009 a 2013. Para fins estat?sticos, utilizou-se o teste de Friedman (p?s-teste de Dunn?s) e o teste U de Mann Whitney. Resultados: Foram inclu?dos 68 pacientes com FC de um total de 80 que s?o acompanhados regularmente pelo servi?o, sendo 57,4 % do sexo masculino com idade m?dia de 10,35 anos no in?cio do estudo. Ao longo do seguimento de cinco anos (m?dia de 3,55 anos) o estado nutricional (p=0,743) e o escore de gravidade cl?nica (p=0,235) permaneceram est?veis. Houve uma redu??o estatisticamente significativa da rela??o VEF1/CVF ao longo do acompanhamento (do ano 2 para o ano 3 e do ano 2 para o ano 5; p<0,05). As coloniza??es mais frequentes foram a Staphylococcus aureus sens?vel a Oxacilina (MSSA) (29,8-46,3%), seguidas da Pseudomonas aeruginosa (PA) n?o muc?ide (8,5-19,4%), Complexo Burkholderia Cepacia (CBC) (4,9-21,3%), PA mucoide (2,4-6,5 %) e Staphylococcus aureus resistente a oxacilina (2,4- 16,1%). Os pacientes que foram colonizados ao longo do estudo por MRSA (p=0,040) apresentaram uma redu??o do VEF1 (p=0,004) e da CVF (p=0,005) no ano 5, em compara??o aos n?o colonizados por essa bact?ria. Al?m disso, os indiv?duos com PA n?o mucoide apresentaram redu??o (p=0,007) da CVF. Conclus?es: Nossos achados demonstram que a coloniza??o bacteriana por Staphylococcus aureus resistente a oxacilina e por Pseudomonas aeruginosa n?o muc?ide est? relacionada a piora na fun??o pulmonar (VEF1 e CVF). Todavia, n?o foi observado impacto das diferentes coloniza??es sobre o IMC e sobre o escore de gravidade cl?nica de Shwachman-Kulczycki ao longo dos 5 anos. Palavras-chave: Microbiologia, estado nutricional, testes de fun??o respirat?ria, progn?stico, fibrose c?stica.

Submitted by PPG Pediatria e Sa?de da Crian?a (pediatria-pg@pucrs.br) on 2018-01-02T16:51:55Z No. of bitstreams: 1 Disserta??o Mestrado Marilisa.pdf: 905969 bytes, checksum: 348718dad9108301470f73e80e214e5c (MD5) / Approved for entry into archive by Caroline Xavier (caroline.xavier@pucrs.br) on 2018-01-24T12:31:32Z (GMT) No. of bitstreams: 1 Disserta??o Mestrado Marilisa.pdf: 905969 bytes, checksum: 348718dad9108301470f73e80e214e5c (MD5) / Made available in DSpace on 2018-01-24T12:33:57Z (GMT). No. of bitstreams: 1 Disserta??o Mestrado Marilisa.pdf: 905969 bytes, checksum: 348718dad9108301470f73e80e214e5c (MD5) 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.

Made available in DSpace on 2015-04-14T14:51:23Z (GMT). No. of bitstreams: 1 447380.pdf: 675913 bytes, checksum: bfb87e6d734478e3c7bb6275c3132761 (MD5) Previous issue date: 2013-02-26 / Liver fibrosis is the wound healing response to repeated injury of the liver. It is characterized by disruption of the liver architecture associated with increased expression of extracellular matrix components. Hepatic stellate cells (HSC) play a key role in liver fibrogenesis. In normal liver, HSC are quiescent and its main function is to store vitamin A. During liver injury, these cells undergo activation, become myofibroblasts and acquire fibrogenic properties. Activation of PPAR&#947; (peroxisome proliferator-activated receptor gamma) and inhibition of fibrogenic molecules are potential strategies to block HSC activation and differentiation. Aware that the process of hepatic fibrosis involves inflammatory mediators, various anti-inflammatory substances have been studied in an attempt to revert fibrosis. The purpose of this study was to investigate the in vitro effects of fructose-1,6-bisphospahte (FBP) on HSC phenotype. The results demonstrated that FBP induced quiescent phenotype in HSC via PPAR&#947; activation. Significant decrease in type I collagen mRNA expression was observed in the first 24h of treatment. These events preceded the reduction of TGF-&#946;1 (transforming growth factor-beta) and total collagen secretion. Thus, FBP promoted downregulation of HSC activation by its antifibrotic and anti-inflammatory actions. These findings demonstrate that FBP may have potential as a novel therapeutic agent for the treatment of liver fibrosis. / A fibrose hep?tica ? a resposta cicatricial do f?gado a les?es continuadas, caracterizada pelo rompimento da arquitetura hep?tica associada ao aumento da express?o dos componentes da matriz extracelular. As c?lulas estreladas hep?ticas (HSC) desempenham um papel fundamental no processo de fibrog?nese. No f?gado normal, as HSC encontram-se em sua forma quiescente de dep?sito de vitamina A. Durante a les?o hep?tica, essas c?lulas passam por uma ativa??o fenot?pica, tornam-se miofibroblastos e adquirem propriedades fibrog?nicas. O processo de fibrose hep?tica envolve v?rios mediadores inflamat?rios e, portanto, subst?ncias anti-inflamat?rias tem sido empregadas na tentativa de reverter a fibrose e bloquear a ativa??o e diferencia??o das HSC. A ativa??o de PPAR&#947; (receptor ativado por proliferador de peroxissomo Gama) e a inibi??o de mol?culas fibrog?nicas s?o poss?veis estrat?gias para estes fins. O objetivo deste estudo foi investigar os efeitos in vitro da frutose-1,6-bisfosfato (FBP) sobre o fen?tipo das HSC. Os resultados demonstraram que a FBP ? capaz de induzir o fen?tipo quiescente das HSC via ativa??o de PPAR&#947;. Foi observado nas primeiras 24h de tratamento uma diminui??o significativa da express?o de mRNA de col?geno tipo I. Posteriormente, houve uma redu??o do col?geno total e de TGF-&#946;1 (fator de transforma??o do crescimento beta). Assim, a FBP diminui o estado de ativa??o das HSC por suas a??es antifibr?ticas e anti-inflamat?rias. Estas descobertas demonstram que a FBP pode ser um potencial novo agente terap?utico para o tratamento de fibrose hep?tica.

Submitted by Setor de Tratamento da Informa??o - BC/PUCRS (tede2@pucrs.br) on 2015-10-09T20:00:24Z No. of bitstreams: 1 475666 - Texto Parcial.pdf: 421018 bytes, checksum: 08ee6322735a0efb306735f02b198a26 (MD5) / Made available in DSpace on 2015-10-09T20:00:24Z (GMT). No. of bitstreams: 1 475666 - Texto Parcial.pdf: 421018 bytes, checksum: 08ee6322735a0efb306735f02b198a26 (MD5) Previous issue date: 2015-07-31 / Introduction: recent evidence has linked vitamin D to benefits in lung health. Little is known, however, about the impact of vitamin D on clinical outcomes in children with cystic fibrosis (CF). In this study, we evaluated the association between vitamin D levels and nutritional status, pulmonary function (PF) and pulmonary exacerbations in children and adolescents with CF. Methods: 25-hydroxyvitamin D (25[OH]D) levels of 37 children and adolescents were evaluated retrospectively. Pulmonary function data, albumin, body mass index (BMI), height for age (H/A) and pulmonary exacerbations episodes were associated with vitamin D levels divided into two groups: normal (?30ng/ml) and insufficient (<30ng/ml). Results: hypovitaminosis D (25[OH]D <30ng/ml) was observed in 54% of patients. The mean of 25(OH)D was 30,53?12,14ng/ml. Pulmonary function and nutritional status were not associated with vitamin D levels. The number of pulmonary exacerbations over a period of two years (p=0,007) and post-dosing period of 25(OH)D (p=0,002) was significantly higher in patients with hypovitaminosis D. There was a trend of lower 25(OH)D levels during autumn and winter (p=0,067). Conclusion: vitamin D deficiency was associated with higher rates of pulmonary exacerbation in children and adolescents with CF. Vitamin D can be a marker of disease severity or be a significant causal factor for a higher number of pulmonary exacerbations. Prospective studies can help to clarify the causality of this association. / Introdu??o: recentes evid?ncias t?m associado a vitamina D a benef?cios na sa?de pulmonar. Pouco se sabe, entretanto, sobre o impacto da vitamina D em desfechos cl?nicos na popula??o pedi?trica com fibrose c?stica (FC). Neste estudo, avaliamos as associa??es entre os n?veis de vitamina D e o estado nutricional, a fun??o pulmonar (FP) e as exacerba??es pulmonares (EP) em crian?as e adolescentes com FC. M?todos: n?veis de 25-hidroxivitamina D (25[OH]D) de 37 crian?as e adolescentes foram avaliados retrospectivamente. Dados de fun??o pulmonar, albumina, ?ndice de massa corporal (IMC), estatura para idade (E/I) e epis?dios de exacerba??o pulmonar foram associados com n?veis de vitamina D divididos em dois grupos: normal (?30ng/ml) e insuficiente (<30ng/ml). Resultados: a hipovitaminose D (25[OH]D <30ng/ml) foi observada em 54% dos pacientes. A m?dia de 25(OH) D foi 30,53?12,14ng/ml. Fun??o pulmonar e estado nutricional n?o se associaram com os n?veis de vitamina D. O n?mero de exacerba??es pulmonares em um per?odo de dois anos (p=0,007) e no per?odo p?s-dosagem da 25(OH)D (p=0,002) foi significativamente maior em pacientes com hipovitaminose D. Houve uma tend?ncia de menores n?veis de 25(OH)D nos per?odos de outono e inverno (p=0,067). Conclus?o: a hipovitaminose D se associou com maiores taxas de exacerba??o pulmonar em crian?as e adolescentes com FC. A vitamina D pode ser um marcador de gravidade da doen?a ou ainda ser um fator causal relevante para um n?mero mais elevado de exacerba??es pulmonares. Estudos prospectivos podem contribuir para esclarecer a causalidade desta associa??o.

Made available in DSpace on 2015-04-14T13:33:09Z (GMT). No. of bitstreams: 1 459023.pdf: 766453 bytes, checksum: df09225ab12d18f640f368a0a5436a50 (MD5) Previous issue date: 2014-03-31 / Introduction : Cystic Fibrosis (CF) is a disease of autosomal recessive character, chronic and progressive, with involvement in various organs and systems. Respiratory and gastrointestinal disorders are more prevalent, which usually results in nutritional deficit. However, when there is a worsening of the clinical condition, requiring hospitalization, the risk of nutritional inbalance increases. The aim of this study was to evaluate variation in weight and body composition during hospitalization of children and adolescents with cystic fibrosis who received hypercaloric and hyperlipidic diet during hospitalization. Methods : This is a longitudinal study involving children and adolescents with CF hospitalized at S?o Lucas's Hospital/ PUCRS. Bioimpedance analysis was used to assess body composition. The evaluation took place within 48 hours after admission and 24 hours prior to hospital discharge. The energy and nutrient intake was investigated through a food record during three consecutive days. Comparisons between weight and body composition during hospitalization were performed through the corresponding statistical tests (t test and the Mann - Whitney test). Differences were considered significant at p < 0.05. Results : The sample consisted of 13 patients, 8 (62 %) male. The mean age was 10.8 ? 3.61 years. The mean hospital stay was 14.5 ? 3.75 days. At the beginning of hospitalization, most patients were eutrophic (n = 9). Regarding categorization by pulmonary function, patients had moderate pulmonary disease according to FEV1 (52.08 ? 22.2). Patients received hypercaloric and hyperlipidic diet during hospitalization , and the findings showed significant changes in the variables, weight and fat mass (weight = 1.47 kg and fat mass 1.06 kg , p < 0.0001). Conclusion : The hypercaloric and hyperlipidic diet recommended during hospitalization was associated with significant weight gain and fat mass, with improvement of the nutritional status of most patients. / Introdu??o : A Fibrose C?stica (FC) ? uma doen?a gen?tica de car?ter autoss?mico recessivo, cr?nica e progressiva, com acometimento em diversos ?rg?os e sistemas. As altera??es respirat?rias e gastrointestinais s?o mais prevalentes, o que resulta geralmente em um comprometimento nutricional. Todavia, quando existe uma piora do estado cl?nico com necessidade de interna??o hospitalar, o risco ou fal?ncia nutricional aumenta. O objetivo deste estudo foi avaliar a varia??o de peso e da composi??o corporal durante a interna??o hospitalar de crian?as e adolescentes portadores de Fibrose C?stica que receberam dieta hipercal?rica e hiperlip?dica durante a interna??o hospitalar. M?todos : Estudo longitudinal, envolvendo crian?as e adolescentes com Fibrose C?stica internados no Hospital S?o Lucas/PUCRS. A an?lise de bioimped?ncia foi utilizada para avalia??o da composi??o corporal. A avalia??o ocorreu em at? 48 horas ap?s a interna??o e 24 horas antes da alta hospitalar. A ingest?o energ?tica e de nutrientes foi investigada por meio do registro alimentar de tr?s dias consecutivos. As compara??es entre o peso e a composi??o corporal durante a interna??o foram realizadas mediante os testes estat?sticos correspondentes (teste t e teste de Mann-Whitney). As diferen?as foram consideradas significativas com p < 0,05. Resultados : A amostra estudada constituiu-se de 13 pacientes, sendo 8 (62%) do sexo masculino. A m?dia de idade foi de 10,8? 3,61 anos. O tempo m?dio de interna??o foi 14,5?3,75 dias. No in?cio da interna??o, a maioria dos pacientes encontravam-se eutr?ficos (n=9). Quanto ? categoriza??o pela fun??o pulmonar, os pacientes apresentaram doen?a pulmonar moderada conforme os valores de VEF1 (52,08?22,2). Os pacientes receberam dieta hipercal?rica e hiperlip?dica durante a interna??o, e os achados demonstraram altera??o significativa nas m?dias das vari?veis, peso e massa gorda (peso= 1,47 kg e massa gorda 1,06 kg; p<0,0001). Conclus?o : A dieta hipercal?rica e hiperlip?dica preconizada durante a interna??o esteve associada a um ganho significativo de peso e de massa gorda, com melhoria do estado nutricional de grande parte dos pacientes.

Submitted by PPG Pediatria e Sa?de da Crian?a (pediatria-pg@pucrs.br) on 2017-11-28T18:15:11Z No. of bitstreams: 1 Tese-MC.pdf: 2529108 bytes, checksum: d7557f03bd9245db7376252ca33303d0 (MD5) / Approved for entry into archive by Caroline Xavier (caroline.xavier@pucrs.br) on 2017-12-04T12:45:28Z (GMT) No. of bitstreams: 1 Tese-MC.pdf: 2529108 bytes, checksum: d7557f03bd9245db7376252ca33303d0 (MD5) / Made available in DSpace on 2017-12-04T12:47:32Z (GMT). No. of bitstreams: 1 Tese-MC.pdf: 2529108 bytes, checksum: d7557f03bd9245db7376252ca33303d0 (MD5) Previous issue date: 2017-07-31 / Coordena??o de Aperfei?oamento de Pessoal de N?vel Superior - CAPES / Introduction-objective: Cystic fibrosis is the most common genetic disease in the Caucasian population. The reduction in life expectancy is due to progressive lung disease, characterized by severe changes in the pulmonary structure, more precisely, bronchiectasis and air catabolization. Computed tomography of the chest is considered the most sensitive method to monitor lung disease in cystic fibrosis. The main disadvantage is the patient's exposure to radiation. Magnetic resonance imaging of the chest, a radiation-free technique, has been introduced as an alternative to computed tomography. Magnetic resonance has been compared to computed tomography in several studies using various sequences, but none have used the combined sensing, parallel imaging, and golden-angle radial sampling technique. The aim of this study was to evaluate the combined golden-angle radial technique of magnetic resonance imaging in patients with cystic fibrosis in relation to conventional magnetic resonance imaging and computed tomography of the chest. Cystic fibrosis has been compared to computed tomography in several studies using various sequences, but none have used the combined sensing, parallel imaging, and golden-angle radial sampling technique. The aim of this study was to evaluate the combined radial-angle golden-angle technique of magnetic resonance imaging in patients with cystic fibrosis in relation to conventional magnetic resonance imaging and computed tomography of the chest. Methods: Computed tomography and magnetic resonance of the chest were performed in 29 patients with cystic fibrosis who were followed at the pediatric pulmonology outpatient clinic of the S?o Lucas Hospital of the Pontifical Catholic University of Rio Grande do Sul. The Cartesian K-space sampling and, after the free-breathing magnetic resonance techniques, using the Golden_Angle Radial Sparse Parallel technique. Magnetic resonance imaging and computed tomography of the chest were evaluated by two independent observers using the Helbich-Bhalla score. Intraclass correlation coefficient and Bland-Altman analysis were used to assess agreement and reproducibility in the Helbich-Bhalla severity score. No patient was sedated or used contrast medium. Results: Intraclass correlation coefficients and the Bland-Altman graphical model between the Helbich-Bhalla scores and the evaluated methods, computed tomography, magnetic resonance and free breating magnetic resonance , showed very strong correlations in all comparisons of the total scores (ICCCT -RM = 97%, ICCCT-FB-RM = 96% and ICCCT-FB-RM = 99%). The association of the Helbich-Bhalla score of both computed tomography and magnetic resonance with pulmonary function tests were significant, mainly in relation to the severity of bronchiectasis and thickening of the bronchial walls. Conclusion: This study demonstrated that the new technique of free-breathing magnetic resonance and combination of compressed sensing, parallel imaging and radial golden-angle sampling, showed a good concordance with computed tomography in the assessment of pulmonary alterations in cystic fibrosis. The combined golden-angle radial technique can be used in clinical practice to monitor fibrocystic lung disease, and demonstrates particular value for patients who can not hold their breath during the examination. / Introdu??o-objetivo: Fibrose c?stica ? a doen?a gen?tica mais comum na popula??o caucasiana. A redu??o da expectativa de vida ? devido ? doen?a pulmonar progressiva, caracterizada por mudan?as severas na estrutura pulmonar, mais precisamente, bronquiectasias e alcaponamento de ar. A tomografia computadorizada de t?rax ? considerada o m?todo mais sens?vel para monitorar a doen?a pulmonar na fibrose c?stica. A principal desvantagem ? a exposi??o do paciente ? radia??o. A resson?ncia magn?tica de t?rax, t?cnica livre de radia??o, tem sido introduzida como uma alternativa ? tomografia computadorizada. A resson?ncia magn?tica tem sido comparada ? tomografia computadorizada em v?rios estudos usando v?rias sequ?ncias, mas, nenhuma utilizou a t?cnica de sensoriamento comprimido, imagem paralela e amostragem golden-angle radial combinada. O objetivo deste estudo foi avaliar a t?cnica de golden-angle radial combinada de resson?ncia magn?tica de t?rax em pacientes com fibrose c?stica em rela??o ? tomografia computadorizada e a resson?ncia magn?tica convencional de t?rax. M?todos: Foram realizados exames de tomografia computadorizada e resson?ncia magn?tica de t?rax em 29 pacientes com fibrose c?stica que eram acompanhados no ambulat?rio de pneumologia pedi?trica do Hospital S?o Lucas da Pontif?cia Universidade Cat?lica do Rio Grande do Sul. Os exames, foram realizados utilizando, primeiro, a amostragem de K-espa?o cartesiano e, ap?s, as t?cnicas de resson?ncia magn?tica com respira??o livre, utilizando a t?cnica Golden_Angle Radial Sparse Parallel. As imagens de resson?ncia magn?tica e tomografia computadorizada de t?rax foram avaliadas por dois observadores independentes utilizando o escore de Helbich-Bhalla. Coeficiente de correla??o intraclasse e an?lise de Bland-Altman foram usados para avaliar a concord?ncia e a reprodutibilidade no escore de severidade de Helbich-Bhalla. Nenhum paciente foi sedado ou usado meio de contraste. Resultados: Os coeficientes de correla??o intraclasse e o modelo gr?fico de Bland-Altman entre os escores de Helbich-Bhalla e os m?todos avaliados, tomografia computadorizada, resson?ncia magn?tica e resson?ncia magn?tica de respira??o livre, evidenciaram, em todas as compara??es dos escores totais, correla??es muito fortes (ICCCT-RM = 97%; ICCCT-FB-RM = 96% e ICCCT- FB-RM=99%). A associa??o do escore de Helbich-Bhalla, tanto da tomografia computadorizada como das imagens de resson?ncia magn?tica, com os testes de fun??o pulmonar foram significativas, principalmente, em rela??o ? severidade das bronquiectasias e espessamento das paredes br?nquicas. Conclus?o: Este estudo demonstrou que a nova t?cnica de resson?ncia magn?tica com respira??o livre e combina??o de sensoriamento comprimido, imagem paralela e amostragem golden-angle radial, mostrou uma boa concord?ncia com a tomografia computadorizada na avalia??o das altera??es pulmonares na fibrose c?stica. A t?cnica de golden-angle radial combinada pode ser utilizada na pr?tica cl?nica para acompanhamento de doen?a pulmonar fibroc?stica, e demonstra particular valor para os pacientes que n?o conseguem suspender a respira??o durante o exame.

Submitted by Setor de Tratamento da Informa??o - BC/PUCRS (tede2@pucrs.br) on 2015-10-29T10:21:57Z No. of bitstreams: 1 475918- Texto Parcial.pdf: 356208 bytes, checksum: a918d2df47ed4b4e5d722fc922cb0fca (MD5) / Made available in DSpace on 2015-10-29T10:21:57Z (GMT). No. of bitstreams: 1 475918- Texto Parcial.pdf: 356208 bytes, checksum: a918d2df47ed4b4e5d722fc922cb0fca (MD5) Previous issue date: 2015-08-18 / Coordena??o de Aperfei?oamento de Pessoal de N?vel Superior - CAPES / Introduction : Cystic fibrosis is a chronic disease that requires regular monitoring and testing. Chest CT is the gold standard to the evaluation of the pulmonary parenchyma, but because of the risk caused by radiation, technical alternatives should be evaluated. MRI is a test that does not use radiation. The aim of this study is to evaluate the performance of MRI chest and correlated with clinical parameters such as spirometry, Shawchman clinical score and the 6-minute walk test. Methods : 19 patients with cystic fibrosis Clinic underwent magnetic resonance of the chest, 6-minute walk test, FEV1 and clinical Shwachman score. MR score was evaluated and correlated with other parameters. Results : MRI correlated with the 6-minute walk test and the clinical score of Shwachman, but not with FEV1. Conclusion : MRI is a good alternative for airway and lung parenchyma assessment in patients with CF, showing a significant correlation with the6-minute walk test and the clinical score of Shwachman. / Introdu??o : a fibrose c?stica ? uma doen?a cr?nica, que necessita de acompanhamento e exames regulares. A TC de t?rax ? o padr?o-ouro para avalia??o das altera??es do par?nquima pulmonar, mas pelo risco causado pela radia??o, t?cnicas alternativas devem ser avaliadas. A RM ? um exame que n?o utiliza radia??o. O objetivo desse estudo ? avaliar o desempenho da RM de t?rax, correlacionando com par?metros cl?nicos, como espirometria, escala de Shawchman e o teste de caminhada de 6 minutos. M?todos : 19 pacientes do ambulat?rio de fibrose c?stica realizaram resson?ncia magn?tica de t?rax, teste de caminhada de 6 minutos, VEF1 e escore clinico de Shwachman. Foi avaliado o escore de RM e correlacionado com os outros par?metros. Resultados : a RM apresentou correla??o com o teste de caminhada de 6 minutos e com o escore cl?nico de Shwachman, mas n?o apresentou com o VEF1. Conclus?o : A RM ? uma boa alternativa para avalia??o de via a?rea e par?nquima pulmonar em pacientes com FC, mostrando correla??o com o teste de caminhada de 6 minutos e com o escore cl?nico de Shwachman.

Made available in DSpace on 2015-04-14T13:32:49Z (GMT). No. of bitstreams: 1 432332.pdf: 2538626 bytes, checksum: ed0a976ccffcbec71b37addcb58469cc (MD5) Previous issue date: 2011-03-30 / Objetivo: O objetivo deste estudo ? determinar se a administra??o de ciproeptadina (CH) ? capaz de induzir o ganho de peso, melhorar o consumo alimentar em pacientes com Fibrose C?stica (FC). M?todos: Realizamos um estudo duplo-cego, controlado por placebo em dois centros de Porto Alegre. Vinte e um pacientes com FC entre 5 e 18 anos completaram o estudo. Os pacientes foram divididos aleatoriamente em dois grupos, para receber 4 mg de CH, tr?s vezes ao dia durante 12 semanas, ou placebo. Todos os dados foram coletados no in?cio e no final do per?odo de estudo inclu?ram peso, altura, circunfer?ncia muscular do bra?o, prega cut?nea tricipital, espirometria e um question?rio validado sobre calorias ingeridas diariamente. Este ? um estudo em curso. Os dados apresentados s?o preliminares. Resultado: O ganho de peso m?dio foi de 1,72 kg no grupo CH e 0,740 kg no grupo placebo, p = 0,072. A mudan?a no ?ndice de massa corporal (IMC) para a idade (escore z) foi de 0,16 no CH e -0,10 no grupo placebo, p = 0,062. O P/I apresentou uma m?dia de 0,13 no grupo CH e 0,04 no grupo placebo, p= 0,074 . A mudan?a no ?ndice de Massa Corporal (IMC) para a idade (em escore Z) foi de 0,16 no grupo CH e -0,10 no grupo placebo, p= 0,062. O IMC variou para 0,49 kg/m? e -0,02 kg/m?, respectivamente, no grupo interven??o e placebo, p= 0,048. Altera??es na fun??o pulmonar, circunfer?ncia muscular do bra?o, altura, dobra cut?nea triciptal e ingesta de calorias di?rias n?o foram estatisticamente diferentes. Conclus?o: CH ajuda a melhorar o ganho de peso em pacientes com FC. A espirometria n?o melhorou. CH poderia ser extremamente ?til no manejo nutricional dos pacientes com FC.

Made available in DSpace on 2015-04-14T13:33:06Z (GMT). No. of bitstreams: 1 449068.pdf: 853739 bytes, checksum: 3cf4fe7841c2c460526d0ee48c6d4191 (MD5) Previous issue date: 2013-03-25 / Objectives : To evaluate possible postural changes and the distribution of plantar pressures in patients with CF. Furthermore, were sought to evaluate the effects of an educational guideline for physical activity in children and adolescents with CF. Method : This study was divided into two phases, being held in a CF center with children and adolescents between seven and 20 years. The first phase is a cross-sectional study in which healthy subjects were selected in order to obtain a normality pattern for postural assessment and baropodometry, aiming to later comparison with patients CF. In the second phase, we performed a randomized controlled clinical trial to access the influence of the exercise guideline on the postural alterations seen in the results of phase I. Patients were assigned to two groups, 17 in the control group (G1) and 17 in the intervention group (G2). The intervention evaluated consisted of a manual with instructions for aerobic exercise and stretching. Data was analyzed using the Kolmogorov-Smirnov and the student t test. The effect size was calculated using the Effect Size Calculator tool, considering a significance level of 5%. Results : In phase I, thirty four patients with CF were included in the study with a mean age of 12.6?2.9 years and twenty of them were boys (58.8%). Thirty four healthy children with a mean age of 12.8?3.3 years were selected and paired to the CF patients. No significant baseline differences between groups were identified, as expected. CF children presented more postural deviations compared to healthy subjects. Significant differences were observed as to alignment of the head (p=0.001), shoulder girdle (p=0.015) and pelvis (p=0.001), as well as increased cervical lordosis (p=0.001) and lateral chest distance (p=0.002). Children with CF also showed higher degrees of thoracic kyphosis, although this difference was not significant (p=0.068). No significant differences were demonstrated in the baropodometry. In phase II (n=34), there were no baseline differences between both intervention and control groups. On the other hand, results demonstrate that the intervention caused a decrease in cervical lordosis (p=0.0003; ES=1.41), thoracic kyphosis (p=0.01; ES=0.89), lumbar lordosis (p=0.05; ES=0.71), lateral chest distance (p=0.01; ES=0.91) and abdominal protrusion (p=0.04; ES=0.75). In the baropodometric evaluation, there were significant differences in the mean pressure (p=0.001; ES=1.25) and in the contact area (p=0.01; ES=1.02). Conclusions : CF children and adolescents present postural changes when compared to healthy individuals. Furthermore, the study demonstrated that the educational guideline for exercise practice helped to improve posture in children and adolescents with CF, preventing the progression of some postural disorders. / Objetivos : Avaliar as poss?veis altera??es posturais e a distribui??o das press?es plantares em pacientes com FC. Al?m disso, buscou-se avaliar os efeitos de um programa de orienta??es para a pr?tica de exerc?cio f?sico em crian?as e adolescentes com FC. M?todo : Este estudo foi dividido em duas fases, sendo realizado em um centro de FC com crian?a e adolescentes entre sete e 20 anos. A primeira fase constitui um estudo de corte transversal, em que indiv?duos saud?veis foram selecionados com objetivo de obter um padr?o de normalidade para avalia??o postural e baropodometria visando ? compara??o com pacientes com FC. Na segunda fase, foi realizado um ensaio clinico controlado e randomizado, de orienta??es para o exerc?cio f?sico a partir dos resultados obtidos na fase I. Os pacientes foram alocados em dois grupos, sendo 17 no grupo controle (G1) e 17 no grupo interven??o (G2). A interven??o utilizada foi um manual de orienta??es com exerc?cios f?sicos aer?bicos e alongamentos. Para an?lise de dados utilizou-se o teste de Kolmogorov-Smirnov e o teste t de student . O tamanho de efeito (TE) foi calculado utilizando-se a ferramenta Effect Size Calculator, considerando-se n?vel de signific?ncia de 5%. Resultados : Na fase I foram inclu?das 34 crian?as e adolescentes com FC, m?dia de idade de 12,6?2,9 anos, sendo 20 pacientes (58,8%) do sexo masculino. Para o pareamento, foram inclu?das 34 crian?as saud?veis, com m?dia de idade de 12,8?3,3. Como esperado, n?o houve diferen?a significativa entre os grupos quanto ? caracteriza??o da amostra. Crian?as com FC apresentaram maiores desvios posturais em compara??o com crian?as saud?veis. Foram observadas diferen?as significativas quanto ao alinhamento da cabe?a (p=0,001), cintura escapular (p=0,015) e pelve (p=0,001), assim como aumento da lordose cervical (p=0,001) e dist?ncia latero-lateral do t?rax ( p=0,002). Crian?as com FC tamb?m apresentaram maiores graus de cifose tor?cica, embora essa diferen?a n?o tenha sido significativa (p=0,068). Os resultados da baropodometria n?o demonstraram diferen?as estatisticamente significativas. Na fase II (n= 34), n?o houve diferen?as na avalia??o inicial entre os grupos interven??o e controle. Por outro lado, os resultados demonstram que a interven??o provocou uma diminui??o na lordose cervical (p=0,0003; TE=1,41), na cifose tor?cica (p=0,01; TE=0,89), na lordose lombar (p=0,05; TE=0,71), na dist?ncia lateral do t?rax (p=0,01; TE=0,91) e na protus?o abdominal (p=0,04; TE=0,75). Na avalia??o baropodom?trica, houve diferen?a significativa na press?o m?dia (p=0,001; TE=1,25) e na ?rea de contato (p=0,01; TE=1,02). Conclus?o : Crian?as e adolescentes com FC apresentam altera??es posturais quando comparados com indiv?duos saud?veis. Al?m disso, o estudo demonstrou que a orienta??o para a pr?tica de exerc?cios contribui para a melhora da postura em crian?as e adolescentes com FC, evitando a progress?o de algumas desordens posturais.