Cystic fibrosis (CF) is a genetic disease with defective chloride transport across cell
membranes, causing exocrine glands present viscous secretions. It mainly affects the
lungs, pancreas and sweat glands. The life expectancy was 2 years in the past, today
increased to 37 years. The objective is to report cases of CF in two adult brothers in
the Amazon. Patient 01: 33, since adolescence had dyspnea on exertion, cough and
pneumonia. In adulthood, it evolved with worsening of lung function and
hypersecretion, being diagnosed with cystic fibrosis by two positive sweat tests.
Presented infectious complications, was admitted to perform antibiotic therapy during
hospitalization had respiratory failure and shock. Was referred to the ICU (Intensive
Care Unit), where hemodynamic stabilization was attempted, however, he presented
clinical deterioration and evolved to death. Patient 02: 32, had in childhood cough
secretive and dyspnea on exertion. As an adult, the symptoms worsened. In
consultation was evaluated and diagnosed with CF through positive test sweat.
Follow stable with occasional antibiotics for clinical exacerbations. The family had
positive history for the disease, with a brother and paternal aunt deceased with
diagnostic and clinical history suggestive, respectively. Discussion: Affirming the
genetic character of the CF, described the report of two adult patients and siblings.
The disease is directly related to the severity of lung disease. One patient had
malnutrition, confirming the severe pancreatic insufficiency described in the literature.
Studies say that infertility is common in most CF patients, unlike patients in this
study, who already have formed offspring. One patient died during the study,
confirming the poor prognosis of the disease. Conclusion: The study is configured as
important local registry on the subject presented in view of the scarcity of scientific
literature on the CF. The biggest incentive for research in this area of knowledge is
necessary for patients with the disease are diagnosed early and more grounded way,
thus improving the quality and life expectancy.
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