Fatores de risco da hepatopatia da fibrose cística

dc.TypeArticlept_BR
dc.contributor.authorFagundes, Eleonora Druve Tavares
dc.contributor.authorRoquete, Mariza Leitão Valadares
dc.contributor.authorPenna, Francisco José
dc.contributor.authorReis, Francisco José Caldeira
dc.contributor.authorGoulart, Eugênio Marcos Andrade
dc.contributor.authorDuque, Cristiano Guedes
dc.date.accessioned2022-08-17T19:27:23Z
dc.date.available2022-08-17T19:27:23Z
dc.date.issued2005
dc.descriptionp. 478-484.: tab. p&b.
dc.description.abstractIdentificar os fatores de risco associados à hepatopatia da fibrose cística. Métodos: Dos 106 pacientes acompanhados regularmente no ano de 1999, 10 preencheram os critérios clínicos, bioquímicos e/ou ultra sonográficos para hepatopatia (9,4%). Foram obtidos dos pacientes, no protocolo do serviço à admissão, fatores relacionados aos dados demográficos, genótipo, idade e manifestações ao diagnóstico da fibrose cística, estado nutricional e dados laboratoriais. As variáveis associadas à hepatopatia foram inicialmente identificadas pelo método de Kaplan-Meier. Os fatores significativos na análise univariada foram incluídos na análise multivariada, utilizando o modelo de regressão de Cox. Resultados: Os fatores associados à hepatopatia na análise univariada foram: sexo masculino, idade ao diagnóstico da fibrose cística, insuficiência pancreática, escore z de peso à admissão, escore de Shwachman e bioquímica à admissão. Após o ajustamento pelo modelo de Cox, duas variáveis demonstraram estar independentemen te associadas ao desenvolvimento de hepatopatia: escore de Shwach man (p = 0,0057) e idade ao diagnóstico da fibrose cística (p = 0,014). Conclusões: O risco de hepatopatia é maior entre os pacientes que apresentam diagnóstico mais precoce e entre aqueles com pior estado clínico avaliado pelo escore de Shwachman, evidenciando que a hepatopatia parece fazer parte de uma forma mais grave da doença. Esses pacientes merecem mais atenção quanto ao screening para hepatopatia e instituição mais precoce do ácido ursodesoxicólico na ocorrência de alterações.
dc.description.abstractenTo identify the risk factors for cystic fibrosis related liver disease. Methods: Ten patients out of a total of 106 patients regularly followed-up during 1999 met the clinical, biochemical and/or ultrasound criteria for liver disease (9.4%). Using information from the admissions records at the service, we collected data on demography, genotype, age and manifestations at diagnosis of cystic fibrosis, nutritional status and laboratory findings. Variables associated with liver disease were initially identified by the Kaplan-Meier method. Those factors that were significant in the univariate analysis were included in the multivariate analysis by means of a Cox regression model. Results: Under univariate analysis the following factors were associated with liver disease: male sex, age at diagnosis of cystic fibrosis, pancreatic insufficiency, z score for weight at admission, Shwachman score and biochemistry at admission. After adjustment by Cox model, two variables were independently associated with liver disease: Shwachman score (p = 0.0057) and age at diagnosis of cystic fibrosis (p = 0.014). Conclusions: The risk of developing liver disease is higher among patients diagnosed at an early age and those with worse clinical status as assessed by the Shwachman score, indicating that liver involvement might be part of a more severe form of the condition. These patients merit greater attention in terms of screening for liver disease and should be given treatment with ursodeoxycholic acid earlier in the event of abnormal findings.
dc.identifier.citationFAGUNDES, Eleonora Druve Tavares et al. Fatores de risco da hepatopatia da fibrose cística. Jornal de Pediatria, v. 81, n. 6, p. 478-484, 2005.
dc.identifier.issn1678-4782
dc.identifier.urihttp://sr-vmlxaph03:8080/jspui/handle/123456789/10213
dc.publisherJornal de Pediatriapt_BR
dc.subjectFibrose Císticapt_BR
dc.subjectCystic Fibrosispt_BR
dc.subjectHepatopatiaspt_BR
dc.subjectLiver Diseasespt_BR
dc.subjectAnálise Multivariadapt_BR
dc.subjectMultivariate Analysispt_BR
dc.titleFatores de risco da hepatopatia da fibrose císticapt_BR
dc.title.alternativeRisk factors for cystic fibrosis related liver diseasept_BR

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