Abstract

FUENTES FERNANDEZ, Gladys  and  PORTUONDO LEYVA, Reyna. Characterization of cystic fibrosis patients who died in the course of disease. Rev Cubana Pediatr [online]. 2014, vol.86, n.3, pp. 344-353. ISSN 0034-7531.

Introduction: cystic fibrosis is one of the most deadly genetic diseases with survival rates over 30 years. Objectives: to describe the complications and causes of death in cystic fibrosis patients and the association of mortality with factors at the onset of disease and with clinical variables. Methods: retrospective, longitudinal and descriptive study of all dead patients in the period of 1993 through December 2013, who had been treated at the follow-up service of pediatric hospital of Centro Habana, according to the following variables: sex, age of onset of disease and death, classification, nutritional status, Pseudomona aeruginosa colonization, complications and necropsy results, all of them obtained from the clinical records. Results: fifteen patients aged 9.4 years as average died, which accounted for 18 % of patients seen in the analyzed period. Males (10/66,67 %) and the 5 to 9 y age group (33.3 %) predominated, although 4 out of 5 nurslings diagnosed with the disease, died before their first birthday. The first manifestation in 60 % was typical respiratory disease and pancreatic failure, plus associated malnutrition in 80 %. At the time of death, all were undernourished. Pseudomona aeruginosa had colonized most of the dead children after one year of diagnosis (67 %). Acute respiratory failure-related bronchial pneumonia was the main cause of death (66 %) and severe pulmonary sepsis was the most common necropsy result (60 %). Conclusions: malnutrition as associated cause and acute chronic respiratory failure were present in most of the dead cases, being the mortality rates higher in very early diagnosed cases.

Keywords : cystic fibrosis; pulmonary disease; malnutrition.

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