Descriptive analysis of cystic fibrosis patients followed by the Pediatric Pulmonology Unit of a university hospital in Porto Alegre-RS <b>[Abstract in English]</b>

Authors

  • Valentina C. B. Gava Chakr aluna do 2º ano do curso de extensão em Pneumologia Pediátrica da PUCRS
  • Fernanda Maria Vendrusculo aluna do 9º semestre do Curso de Fisioterapia da PUCRS
  • Gabriela Tomedi Leites aluna do 9º semestre do Curso de Fisioterapia da PUCRS
  • Márcio Vinícius Fagundes Donadio Doutor em Fisiologia pela PUCRS; professor da Faculdade de Enfermagem, Nutriçã
  • Taísa Frescura Paim médica do Serviço de Pneumologia do HSL-PUCRS
  • Paulo José Cauduro Marostica Professor adjunto do Departamento de Pediatria da UFRGS; médico reponsável pel
  • Mara Rúbia Silveira

Keywords:

Mucoviscidose, fibrose cística, criança, adolescente, nutrição, espirometria, pseudomonas, diagnóstico

Abstract


Aims: To provide a descriptive analysis of patients followed in a local cystic fibrosis treatment center and compare the collected data with those found in the literature.
Methods: Relevant data for clinical follow-up of cystic fibrosis patients were used for analysis and correlation.
Results: Twenty six male and twenty two female patients were studied. From 48 patients, 89,6% presented with pancreatic insufficiency and 29,1% had at least one positive culture to Pseudomonas aeruginosa in the last 6 months. The patients colonized by P. aeruginosa and the ones with pancreatic insufficiency presented with worse values for forced expiratory volume in one second (FEV1). The pancreatic insufficiency group (84 versus 112%; p = 0,005) and the patients colonized by P. aeruginosa (75 versus 85%; p = 0,006) had lower pulmonary function parameters.
Conclusions: The data from our study show that this group of patients presents in accordance with data available from literature, considering the analysed features of the disease. However, there is a marked exception in the time of diagnosis, which is still very late in Brazil.
KEY WORDS: CYSTIC FIBROSIS/diagnosis; CYSTIC FIBROSIS/dietoterapia; CHILD; INFANT; ADOLESCENT; SPIROMETRY; PSEUDOMONAS.

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Chakr, V. C. B. G., Vendrusculo, F. M., Leites, G. T., Donadio, M. V. F., Paim, T. F., Marostica, P. J. C., & Silveira, M. R. (2007). Descriptive analysis of cystic fibrosis patients followed by the Pediatric Pulmonology Unit of a university hospital in Porto Alegre-RS <b>[Abstract in English]</b>. Scientia Medica, 16(3), 103–108. Retrieved from https://revistaseletronicas.pucrs.br/ojs/index.php/scientiamedica/article/view/1617

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