Cystic fibrosis in adults. 2007

Ilma Aparecida Paschoal, and Wander de Oliveira Villalba, and Carmen Sílvia Bertuzzo, and Elza Maria F P Cerqueira, and Mônica Corso Pereira
Pulmonary Diseases Service, Department of Internal Medicine, Faculty of Medical Sciences, State University of Campinas (UNICAMP), Campinas, São Paulo, Brazil.

The aim of this cross-sectional retrospective study was to describe clinical and functional characteristics and the microbiological profile of an adult population with cystic fibrosis. The study was performed at the Pulmonary Diseases Service of the Hospital of the Medical School of the State University of Campinas. The charts of 54 adults (27 males, 27 females) with cystic fibrosis were reviewed. Demographic, clinical, and microbiological data were collected. Clinical and functional characteristics of patients with chronic Pseudomonas infection were compared with those without it. The mean age of the patients was 41.8 years and the mean sweat chloride concentration was 106.4 mEq/L. Forty-nine percent had chronic respiratory symptoms only after 18 years of age, and 85% reported chronic production of purulent sputum. Six percent had diarrhea or fat in stools, and no patient suffered from diabetes mellitus. Pseudomonas was present in 48%, and 73% of them had the mucoid strain. Others agents found were Staphylococcus aureus (28%), Hemophylus sp. (34%), Aspergillus sp. (22%), Stenotrophomonas maltophilia (9%), Acinetobacter sp. (7%), and Burkholderia cepacia (2%). Nontuberculous mycobacteria were found in sputum from 9 patients (11%); 34 patients (67%) had obstructive ventilatory defect; and 47% had obstruction and restriction. Concerning the groups with and without Pseudomonas colonization, only the values of sweat chloride were significantly higher in chronic colonized patients. Less severe phenotypes of cystic fibrosis may be found among patients previously diagnosed as having bronchiectasis. Chronic colonization with Pseudomonas aeruginosa in this study was not associated with worse clinical status but colonized patients did show significantly higher levels of chloride in sweat.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010641 Phenotype The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment. Phenotypes
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D011549 Pseudomonas A genus of gram-negative, aerobic, rod-shaped bacteria widely distributed in nature. Some species are pathogenic for humans, animals, and plants. Chryseomonas,Pseudomona,Flavimonas
D011552 Pseudomonas Infections Infections with bacteria of the genus PSEUDOMONAS. Infections, Pseudomonas,Pseudomonas aeruginosa Infection,Infection, Pseudomonas,Pseudomonas Infection,Pseudomonas aeruginosa Infections
D002712 Chlorides Inorganic compounds derived from hydrochloric acid that contain the Cl- ion. Chloride,Chloride Ion Level,Ion Level, Chloride,Level, Chloride Ion
D003430 Cross-Sectional Studies Studies in which the presence or absence of disease or other health-related variables are determined in each member of the study population or in a representative sample at one particular time. This contrasts with LONGITUDINAL STUDIES which are followed over a period of time. Disease Frequency Surveys,Prevalence Studies,Analysis, Cross-Sectional,Cross Sectional Analysis,Cross-Sectional Survey,Surveys, Disease Frequency,Analyses, Cross Sectional,Analyses, Cross-Sectional,Analysis, Cross Sectional,Cross Sectional Analyses,Cross Sectional Studies,Cross Sectional Survey,Cross-Sectional Analyses,Cross-Sectional Analysis,Cross-Sectional Study,Cross-Sectional Surveys,Disease Frequency Survey,Prevalence Study,Studies, Cross-Sectional,Studies, Prevalence,Study, Cross-Sectional,Study, Prevalence,Survey, Cross-Sectional,Survey, Disease Frequency,Surveys, Cross-Sectional
D003550 Cystic Fibrosis An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION. Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D005260 Female Females

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