Perfil epidemiológico y clínico de pacientes con fibrosis quística atendidos en el servicio de neumología pediátrica del Hospital del Niño Dr. Francisco de Icaza Bustamante, periodo 2021 - 2023

Abstract

Background: Chemical fibrosis (CF) is an autosomal recessive disease most common in white people. Its main clinical manifestations are respiratory and digestive, with great repercussions on the quality of life and morbidity and mortality of the pediatric population. Objective: to describe the epidemiological and clinical profile of patients with chemical fibrosis treated in the pediatric pulmonology service of the Dr. Francisco de Icaza Bustamante Hospital from 2021 to 2023. Methodology: observational, descriptive study, where clinical records were reviewed. of patients with a confirmed diagnosis of chemical fibrosis. Descriptive statistics were applied. The presentation of results was carried out through personalized tables. Results: a total of 62 patients were found, 50% female and 50% male, 93.5% were mixed race, the average age was 9 years, 59% came from the coastal region. 43.5% of patients were diagnosed before one year of age, 62.5% of patients had repeated respiratory infections, with hospitalizations prior to diagnosis in 48% of cases, 16.5% with a family history of CF, 41.9% of participants presented exacerbations that required hospitalization in 2023. 61.3% presented pathogenic colonization for staphylococcus aureus and pseudomonas aeruginosa by 36.8% for both germ Conclusions: respiratory manifestations were the most common, the diagnosis of CF was made mainly before one year of age, pathogenic colonization by staphylococcus aureus and pseudomonas occurred with the same frequency.