Transcriptomic analysis of polyaromatic hydrocarbon degradation by the halophilic fungus Aspergillus sydowii at hypersaline conditions.

Polycyclic aromatic hydrocarbons (PAHs) are among the most persistent xenobiotic compounds, with high toxicity effects. Mycoremediation with halophilic Aspergillus sydowii was used for their removal from a hypersaline medium (1 M NaCl). A. sydowii metabolized PAHs as sole carbon sources, resulting in the removal of up to 90% for both PAHs [benzo [a] pyrene (BaP) and phenanthrene (Phe)] after 10 days. Elimination of Phe and BaP was almost exclusively due to biotransformation and not adsorption by dead mycelium and did not correlate with the activity of lignin modifying enzymes (LME). Transcriptomes of A. sydowii grown on PAHs, or on glucose as control, both at hypersaline conditions, revealed 170 upregulated and 76 downregulated genes. Upregulated genes were related to starvation, cell wall remodelling, degradation and metabolism of xenobiotics, DNA/RNA metabolism, energy generation, signalling and general stress responses. Changes of LME expression levels were not detected, while the chloroperoxidase gene, possibly related to detoxification processes in fungi, was strongly upregulated. We propose that two parallel metabolic pathways (mitochondrial and cytosolic) are involved in degradation and detoxification of PAHs in A. sydowii resulting in intracellular oxidation of PAHs. To the best of our knowledge, this is the most comprehensive transcriptomic analysis on fungal degradation of PAHs.

Protein complexes associated with ß-catenin differentially influence the differentiation profile of neonatal and adult CD8+ T cells.

The canonical Wnt signaling pathway is a master cell regulator involved in CD8+ T cell proliferation and differentiation. In human CD8+ T cells, this pathway induces differentiation into memory cells or a "stem cell memory like" population, which is preferentially present in cord blood. To better understand the role of canonical Wnt signals in neonatal or adult blood, we compared the proteins associated with ß-catenin, in nonstimulated and Wnt3a-stimulated human neonatal and adult naive CD8+ T cells. Differentially recruited proteins established different complexes in adult and neonatal cells. In the former, ß-catenin-associated proteins were linked to cell signaling and immunological functions, whereas those of neonates were linked to proliferation and metabolism. Wnt3a stimulation led to the recruitment and overexpression of Wnt11 in adult cells and Wnt5a in neonatal cells, suggesting a differential connexion with planar polarity and Wnt/Ca2+ noncanonical pathways, respectively. The chromatin immunoprecipitation polymerase chain reaction ß-catenin was recruited to a higher level on the promoters of cell renewal genes in neonatal cells and of differentiation genes in those of adults. We found a preferential association of ß-catenin with CBP in neonatal cells and with p300 in the adult samples, which could be involved in a higher self-renewal capacity of the neonatal cells and memory commitment in those of adults. Altogether, our results show that different proteins associated with ß-catenin during Wnt3a activation mediate a differential response of neonatal and adult human CD8+ T cells.

High-throughput genomic sequencing of cassava bacterial blight strains identifies conserved effectors to target for durable resistance.

Cassava bacterial blight (CBB), incited by Xanthomonas axonopodis pv. manihotis (Xam), is the most important bacterial disease of cassava, a staple food source for millions of people in developing countries. Here we present a widely applicable strategy for elucidating the virulence components of a pathogen population. We report Illumina-based draft genomes for 65 Xam strains and deduce the phylogenetic relatedness of Xam across the areas where cassava is grown. Using an extensive database of effector proteins from animal and plant pathogens, we identify the effector repertoire for each sequenced strain and use a comparative sequence analysis to deduce the least polymorphic of the conserved effectors. These highly conserved effectors have been maintained over 11 countries, three continents, and 70 y of evolution and as such represent ideal targets for developing resistance strategies.

Neonatal CD4+ T cells have a characteristic transcriptome and epigenome and respond to TCR stimulation with proliferation and yet a limited immune response.

The adaptive immune response is coordinated by CD4+ T cells, which determine the type and strength of the immune response and the effector cells involved. It has been reported that CD4+ T cells are less responsive in neonates, leading to low activation of the cellular response and poor antibody production by B cells. This low response is essential for the tolerant window that favors birth transition from the sterile environment in the womb to the outside world but leaves neonates vulnerable to infection, which is still an important health issue. Neonates have a high morbidity and mortality rate due to infections, and the molecular reasons are still understudied. We asked whether the neonatal naive CD4+ T cells have a genomic program that predisposes them to a low response. Therefore, we evaluated the transcriptome and epigenome of human neonatal and adult naive CD4+ T cells. Our results point to a gene expression profile forming a distinct regulatory network in neonatal cells, which favors proliferation and a low T-cell response. Such expression profile is supported by a characteristic epigenetic landscape of neonatal CD4+ T cells, which correlates with the characteristic transcriptome of the neonatal cells. These results were confirmed by experiments showing a low response to activation signals, higher proliferation, and lower expression of cytokines of neonatal CD4+ T cells as compared to adult cells. Understanding this network could lead to novel vaccine formulations and better deal with life-threatening diseases during this highly vulnerable period of our lives.

Metabolic alterations impair differentiation and effector functions of CD8+ T cells.

CD8+ T lymphocytes are one of the main effector cells of the immune system, they protect the organism against intracellular threats such as viruses and bacteria, as well as neoplasms. It is currently well established that CD8+ T cells have distinct immune responses, given by their phenotypes Tc1, Tc2, Tc17, and TcReg. The cellular plasticity of such phenotypes depends on the presence of different combinations of cytokines in the extracellular medium. It is known that metabolic imbalances play an important role in immune response, but the precise role of metabolic disturbances on the differentiation and function of CD8+ T cells, however, has not been explored. In this work, we used a computational model to explore the potential effect of metabolic alterations such as hyperglycemia, high alcohol consumption, dyslipidemia, and diabetes on CD8+ T cell differentiation. Our model predicts that metabolic alterations preclude the effector function of all CD8+ T cell phenotypes except for TcReg cells. It also suggests that such inhibition originates from the increase of reactive oxygen species in response to metabolic stressors. Finally, we simulated the outcome of treating metabolic-inhibited CD8+ T cells with drugs targeting key molecules such as mTORC1, mTORC2, Akt, and others. We found that overstimulation of mTORC2 may restore cell differentiation and functions of all effector phenotypes, even in diabetic patients. These findings highlight the importance of our predictive model to find potential targets to strengthen immunosuppressed patients in chronic diseases, like diabetes.

Characterization of a murine model of endothelial dysfunction induced by chronic intraperitoneal administration of angiotensin II.

Endothelial dysfunction (ED) is a key factor for the development of cardiovascular diseases. Due to its chronic, life-threatening nature, ED only can be studied experimentally in animal models. Therefore, this work was aimed to characterize a murine model of ED induced by a daily intraperitoneal administration of angiotensin II (AGII) for 10 weeks. Oxidative stress, inflammation, vascular remodeling, hypertension, and damage to various target organs were evaluated in treated animals. The results indicated that a chronic intraperitoneal administration of AGII increases the production of systemic soluble VCAM, ROS and ICAM-1 expression, and the production of TNFα, IL1ß, IL17A, IL4, TGFß, and IL10 in the kidney, as well as blood pressure levels; it also promotes vascular remodeling and induces non-alcoholic fatty liver disease, glomerulosclerosis, and proliferative retinopathy. Therefore, the model herein proposed can be a representative model for ED; additionally, it is easy to implement, safe, rapid, and inexpensive.

Contribution of ROS and metabolic status to neonatal and adult CD8+ T cell activation.

In neonatal T cells, a low response to infection contributes to a high incidence of morbidity and mortality of neonates. Here we have evaluated the impact of the cytoplasmic and mitochondrial levels of Reactive Oxygen Species of adult and neonatal CD8+ T cells on their activation potential. We have also constructed a logical model connecting metabolism and ROS with T cell signaling. Our model indicates the interplay between antigen recognition, ROS and metabolic status in T cell responses. This model displays alternative stable states corresponding to different cell fates, i.e. quiescent, activated and anergic states, depending on ROS levels. Stochastic simulations with this model further indicate that differences in ROS status at the cell population level contribute to the lower activation rate of neonatal, compared to adult, CD8+ T cells upon TCR engagement. These results are relevant for neonatal health care. Our model can serve to analyze the impact of metabolic shift during cancer in which, similar to neonatal cells, a high glycolytic rate and low concentrations of glutamine and arginine promote tumor tolerance.

Cucumis sativus Aqueous Fraction Inhibits Angiotensin II-Induced Inflammation and Oxidative Stress In Vitro.

Inflammation and oxidative stress play major roles in endothelial dysfunction, and are key factors in the progression of cardiovascular diseases. The aim of this study was to evaluate in vitro the effect of three subfractions (SFs) from the Cucumis sativus aqueous fraction to reduce inflammatory factors and oxidative stress induced by angiotensin II (Ang II) in human microvascular endothelial cells-1 (HMEC-1) cells. The cells were cultured with different concentrations of Ang II and 0.08 or 10 µg/mL of SF1, SF2, or SF3, or 10 µmol of losartan as a control. IL-6 (Interleukin 6) concentration was quantified. To identify the most effective SF combinations, HMEC-1 cells were cultured as described above in the presence of four combinations of SF1 and SF3. Then, the effects of the most effective combination on the expression of adhesion molecules, the production of reactive oxygen species (ROS), and the bioavailability of nitric oxide (NO) were evaluated. Finally, a mass spectrometry analysis was performed. Both SF1 and SF3 subfractions decreased the induction of IL-6 by Ang II, and C4 (SF1 and SF3, 10 µg/mL each) was the most effective combination to inhibit the production of IL-6. Additionally, C4 prevented the expression of adhesion molecules, reduced the production of ROS, and increased the bioavailability of NO. Glycine, arginine, asparagine, lysine, and aspartic acid were the main components of both subfractions. These results demonstrate that C4 has anti-inflammatory and antioxidant effects.

Data of the effects of acetone fraction from Sechium edule (Jacq.) S.w. edible roots in the kidney of endothelial dysfunction induced mice.

Endothelial dysfunction induced by Angiotensin II (AG II) plays an important role in the pathogenesis of hypertension and is accompanied by a prooxidative condition, which in turn induces an inflammatory state, vascular remodeling, and tissue damage including the kidney (Schmitt and Dirsch, 2009) [1]. New drugs that can control several of these pathologies are required. Sechium edule has been reported to possess antioxidant, anti-inflammatory and antihypertensive activity (Ibarra-Alvarado et al., 2010) [2]. This paper contains data complementary to those published in Journal of Ethnopharmacology (Moreno et al., 2018) [3], evaluating the effect in kidney of hypertensive mice of the acetone fraction from S. edule to control de pro-oxidative state, reduction of the inflammatory adhesion molecule (ICAM) and recruitment of inflammatory cells.

Acetone fraction from Sechium edule (Jacq.) S.w. edible roots exhibits anti-endothelial dysfunction activity.

ETHNOPHARMACOLOGICAL RELEVANCE: A recent ethnomedical survey on medicinal plants grown in Mexico revealed that Sechium edule (Jacq.) Sw. (Cucurbitaceae) is one of the most valued plant species to treat cardiovascular diseases, including hypertension. Fruits, young leaves, buds, stems, and tuberous roots of the plant are edible. Considering that endothelial dysfunction induced by Angiotensin II plays an important role in the pathogenesis of hypertension and is accompanied by a prooxidative condition, which in turn induces an inflammatory state, vascular remodeling, and tissue damage, and that S. edule has been reported to possess antioxidant, anti-inflammatory and antihypertensive activity, its capability to control endothelial dysfunction was also assessed. AIM OF THE STUDY: To assess in vivo the anti-endothelial dysfunction activity of the acetone fraction (rSe-ACE) of the hydroalcoholic extract from S. edule roots. MATERIALS AND METHODS: Endothelial dysfunction was induced in female C57BL/6 J mice by a daily intraperitoneal injection of angiotensin II for 10 weeks. Either rSe-ACE or losartan (as a control) were co-administered with angiotensin II for the same period. Blood pressure was measured at weeks 0, 5, and 10. Kidney extracts were prepared to determine IL1ß, IL4, IL6, IL10, IL17, IFNγ, TNFα, and TGFß levels by ELISA, along with the prooxidative status as assessed by the activity of antioxidant enzymes. The expression of ICAM-1 was evaluated by immunohistochemistry in kidney histological sections. Kidney and hepatic damage, as well as vascular tissue remodeling, were studied. RESULTS: The rSe-ACE fraction administered at a dose of 10 mg/kg was able to control hypertension, as well as the prooxidative and proinflammatory status in kidney as efficiently as losartan, returning mice to normotensive levels. Additionally, the fraction was more efficient than losartan to prevent liver and kidney damage. Phytochemical characterization identified cinnamic acid as a major compound, and linoleic, palmitic, and myristic acids as the most abundant non-polar components in the mixture, previously reported to aid in the control of hypertension, inflammation, and oxidative stress, three important components of endothelial dysfunction. IN CONCLUSION: this study demonstrated that rSe-ACE has anti-endothelial dysfunction activity in an experimental model and highlights the role of cinnamic acid and fatty acids in the observed effects.

Cell biology of T cell activation and differentiation.

T cells are major components of the adaptive immune system. They can differentiate into two different populations of effector cells-type one and type two-and may also become tolerant. T cells respond to immune challenges by interacting with antigen-presenting cells of the innate immune system. These latter cells can identify the nature of any immune challenge and initiate adaptive immune responses. Dendritic cells are the most important antigen-presenting cells in the body. The T cell recognizes both peptides associated with MHC molecules on the antigen-presenting cells and also other molecules in a complex structure known as an immunological synapse. The nature of the antigen, the cytokine environment, and other molecules on the dendritic cell surface instruct the T cells as to the response required. A better understanding of the biology of T cell responses offers the prospect of more effective therapeutic interventions.

Low-level resistance and clonal diversity of Pseudomonas aeruginosa among chronically colonized cystic fibrosis patients.

A prospective study was conducted in Brazil to evaluate antimicrobial resistance patterns and molecular epidemiology of Pseudomonas aeruginosa isolates from cystic fibrosis (CF) patients with chronic lung infection. All isolates were obtained between May 2009 and June 2010 from 75 patients seen in four reference centers in Brazil: HCPA (20 patients) and HEOM (15 patients), located in southern and northeastern Brazil, respectively; IFF (20 patients) and HUPE (20 patients), both in southwestern Brazil. Antimicrobial susceptibility testing, PCR for detection of carpapenemases, and pulsed-field gel electrophoresis (PFGE) were performed in 274 isolates. A total of 224 PFGE types were identified and no clones were found circulating among the centers or within the same center. Despite the chronic infection, most patients were colonized by intermittent clones. Only three patients (4%) maintained the same clone during the study. The resistance rates were lower than 30% for the majority of antimicrobials tested in all centers and only 17% of isolates were multiresistant. Isolates (n = 54) with reduced susceptibility to imipenem and/or meropenem presented negative results for blaSPM-1, blaIMP-1, blaVIM , and blaKPC genes. Our results indicate an unexpected low level of antimicrobial resistance and a high genotypic diversity among P. aeruginosa from Brazilian chronic CF patients.

Clinical and radiological analysis of children and adolescents with tuberculosis in Bahia, Brazil.

We reviewed the clinical and radiological characteristics of tuberculosis (TB) in children and adolescents at the Hospital Especializado Octávio Mangabeira, (HEOM) in Salvador, Bahia. This study included 275 TB patients aged 1 to 15 years seen between January 1990 and November 2001. Standardized forms were filled out on the basis of a review of patient records and x-rays. Through a retrospective and descriptive analysis, it was found that 51.6% were male, 35.3% were aged 1 to 5 years, 28% were aged 6 to 10 and 36.7% were aged 11 to 15. Among all patients, 79.6% lived in the city of Salvador. A history of contact with TB was found in 63.9%, most frequently among children under 5 years old; 77.2% were vaccinated with Bacillus Calmette-Guerin (BCG). The most frequently observed symptoms were coughing (76%), fever (73.1%) weight loss (53.1%), and 4.7% were asymptomatic. Pulmonary TB was most frequent (57.8%) and extra-pulmonary TB occurred in 24.4%, with a predominance of hilar adenopathy. Both forms occurred simultaneously in 17.8%. In 53.1% of the cases the diagnosis was not determined by bacteriology or pathological anatomy; in these cases diagnosis was reached through clinical and radiological criteria, contact history, a tuberculin test > or = 10mm and a positive response to tuberculostatic drugs.

Pseudotumoral form of primary progressive tuberculosis: a diagnosis to be considered.

The diversity of clinical presentations of primary progressive tuberculosis (TB) and the difficulty in establishing the diagnosis of paucibacillary forms is the subject of painstaking research, as well as a cause of delay in therapy. We report the case of a 10-year-old black child who presented with chest pain and progressive widening of the upper mediastinum. Computerized tomography of the chest revealed multiple calcifications that were not identified with X-rays. Biopsy through mediastinoscopy was compatible with a diagnosis of tuberculosis. Despite exhaustive investigation that included direct examination, culture for mycobacteria and PCR (Polymerase Chain Reaction) of tissue samples, the etiologic agent was not revealed. Tuberculin conversion was observed during the follow-up and resolution period of the lesion, after administration of isoniazid, rifampicin and pyrazinamide. The nodal pseudotumoral form of tuberculosis is rare in immunocompetent children and it may simulate neoplastic disease; therefore, it should be included in the list of differential diagnoses of masses located in the anterosuperior mediastinum.

Prevalence of pathogens in cystic fibrosis patients in Bahia, Brazil.

INTRODUCTION: Recurrent respiratory infections account for most of the morbidity and mortality of cystic fibrosis patients. MATERIALS AND METHODS: The objective was to determine the prevalence of pathogens isolated from lower respiratory tract secretions in cystic fibrosis patients. In this descriptive observational study, data from 69 patients was collected from medical records. RESULTS: The microorganisms that were identified included 36.2 P. aeruginosa, 28.9% S. Aureus, 4.3% K. pneumoniae, 1.5% H. influenzae, 1.5% E. coli, 1.5% S. maltoophilia, and in 27.5% the flora was normal. The prevalence of P. aeruginosa was 83% in patients under two years of age, demonstrating early colonization. CONCLUSION: P. aeruginosa and S. aureus were the most prevalent pathogens; there was also early infection/colonization by P. aeruginosa. This information will contribute to improved therapeutic measures for patients of the Bahia Cystic Fibrosis Reference Center.

Nontuberculosis mycobacteria at a multiresistant tuberculosis reference center in Bahia: clinical epidemiological aspects.

OBJECTIVE: Examine the prevalence and clinical/epidemiological aspects of patients with nontuberculous mycobacteria (NTM) isolated from sputum provided by an outpatient clinic specializing in the treatment of multiresistant tuberculosis (MRTB) in Bahia, Brazil. METHODS: All patients followed at the MRTB outpatient clinic of the Octávio Mangabeira Specialized Hospital (HEOM) were evaluated retrospectively from July 1998 to July 2003. All patients underwent direct examinations and cultures to identify the mycobacteria species found during initial and subsequent evaluations. The following variables were recorded: age, gender, clinical symptoms and signs, pre-existing lung disease, prior TB treatment, HIV serology, and NTM species. Categorical and quantitative variables were respectively characterized using proportions and measures +/- SD. RESULTS: NTM were isolated in 19 of 231 patients (8.2%; 95%CI: 5.2%-12.3%), with the following species distribution: 58% (11/19) M. chelonae/abscessus; 16% (3/19) M. avium-intracellular complex; 16% (3/19) M. kansasii; and 11% (2/19) M. fortuitum. HIV serology was positive for just one patient (5%), from whom M. chelonae/abscessus was isolated. Productive coughing was observed in all cases. American Thoracic Society (ATS) diagnostic criteria for NTM lung disease were observed in 14 patients (74%). CONCLUSION: The prevalence of NTM isolated from patients referred to the MRTB outpatient clinic in Bahia was 8.2% (CI 95%: 5.2%-12.3%); rapid-growth mycobacteria (M. chelonae/M. fortuitum) were the most frequently isolated (68%).

Microbiology of the middle meatus compared to sputum in young patients with cystic fibrosis from Bahia - Brazil.

Lower airway infection is a major cause of morbidity and mortality in patients with cystic fibrosis. It is currently unknown if the infection of the upper airway can cause exacerbation of lower respiratory tract infection. This study aimed to determine the microbiological profile of the anterior paranasal sinuses outflow tract (middle meatus) of cystic fibrosis outpatients. The microbiological profile was defined using endoscopically directed middle meatal cultures. Paired middle meatal and sputum specimens were collected from 56 outpatients for aerobic cultures. A semi-quantitative leukocyte count of the middle meatal samples was performed. The median age of patients was nine years (3-20 years). Staphylococcus aureus (37%), Staphylococcus coagulase-negative (25%), Neisseriac (14%), Pseudomonas aeruginosa (11%), and Streptococcus pneumoniae (7%) were the most prevalent microorganisms in the middle meatal cultures. Using the middle meatal leukocyte count, 16 out of 54 patients (29.6%) presented sinus infection. The most frequently identified pathogens in patients with sinus infections were Staphylococcus aureus (10 patients), Pseudomonas aeruginosa (4 patients), and Streptococcus pneumoniae (3 patients). Agreement of paired middle meatal and sputum cultures was significantly higher among patients with infection in middle meatal (69%). The most common middle meatal pathogens were the typical cystic fibrosis spectrum. This suggests the potential for participating in post-nasal lower airway seeding.

A first-year dornase alfa treatment impact on clinical parameters of patients with cystic fibrosis: the Brazilian cystic fibrosis multicenter study.

OBJECTIVE: To describe the clinical impact of the first year treatment with dornase alfa, according to age groups, in a cohort of Brazilian Cystic Fibrosis (CF) patients. METHODS: The data on 152 eligible patients, from 16 CF reference centers, that answered the medical questionnaires and performed laboratory tests at baseline (T0), and at six (T2) and 12 (T4) months after dornase alfa initiation, were analyzed. Three age groups were assessed: six to 11, 12 to 13, and >14 years. Pulmonary tests, airway microbiology, emergency room visits, hospitalizations, emergency and routine treatments were evaluated. Student's t-test, chi-square test and analysis of variance were used when appropriated. RESULTS: Routine treatments were based on respiratory physical therapy, regular exercises, pancreatic enzymes, vitamins, bronchodilators, corticosteroids, and antibiotics. In the six months prior the study (T0 phase), hospitalizations for pulmonary exacerbations occurred in 38.0, 10.0 and 61.4% in the three age groups, respectively. After one year of intervention, there was a significant reduction in the number of emergency room visits in the six to 11 years group. There were no significant changes in forced expiratory volume in one second (VEF(1)), in forced vital capacity (FVC), in oxygen saturation (SpO(2)), and in Tiffenau index for all age groups. A significant improvement in Shwachman-Kulczychi score was observed in the older group. In the last six months of therapy, chronic or intermittent colonization by P. aeruginosa was detected in 75.0, 71.4 and 62.5% of the studied groups, respectively, while S. aureus colonization was identified in 68.6, 66.6 and 41.9% of the cases. CONCLUSIONS: The treatment with dornase alfa promoted the maintenance of pulmonary function parameters and was associated with a significant reduction of emergency room visits due to pulmonary exacerbations in the six to 11 years age group, with better clinical scores in the >14 age group, one year after the intervention.

Nocturnal hypoxemia in children and adolescents with cystic fibrosis.

OBJECTIVE: To determine the prevalence of nocturnal hypoxemia and its association with pulmonary function, nutritional status, sleep macrostructure, and obstructive respiratory events during sleep in a population of clinically stable children and adolescents with cystic fibrosis (CF). METHODS: This was a cross-sectional study involving 67 children and adolescents with CF between 2 and 14 years of age. All of the participants underwent polysomnography, and SpO2 was measured by pulse oximetry. We also evaluated the Shwachman-Kulczycki (S-K) scores, spirometry findings, and nutritional status of the patients. RESULTS: The study involved 67 patients. The mean age of the patients was 8 years. The S-K scores differed significantly between the patients with and without nocturnal hypoxemia, which was defined as an SpO2 < 90% for more than 5% of the total sleep time (73.75 ± 6.29 vs. 86.38 ± 8.70; p < 0.01). Nocturnal hypoxemia correlated with the severity of lung disease, FEV1 (rs = -0.42; p = 0.01), FVC (rs = -0.46; p = 0.01), microarousal index (rs = 0.32; p = 0.01), and apnea-hypopnea index (rs = 0.56; p = 0.01). CONCLUSIONS: In this sample of patients with CF and mild-to-moderate lung disease, nocturnal oxygenation correlated with the S-K score, spirometry variables, sleep macrostructure variables, and the apnea-hypopnea index.

Sleep architecture and polysomnographic respiratory profile of children and adolescents with cystic fibrosis.

OBJECTIVES: To evaluate sleep architecture in children and adolescents with both cystic fibrosis (CF) and a clinical suspicion of sleep-disordered breathing (SDB), and to identify the respiratory polysomnographic profile of these patients. METHODS: Parents or guardians of children with CF filled out a questionnaire designed to assess their clinical and sleep conditions. Children who were identified as having behaviors associated with SDB underwent polysomnography. After polysomnography, patients were grouped according to the obstructive apnea index (AI) obtained (either < 1 or ≥ 1), and a multiple correspondence factor analysis was used to analyze and identify the polysomnographic profile of patients. RESULTS: Of the 74 patients who met inclusion criteria for this study, 67 underwent polysomnography, and 38 (56.7%) of the 67 patients showed an AI ≥ 1. Median age was 8 years. The group of patients with an AI ≥ 1 was characterized by total sleep time (TST) during stage 4 and rapid eye movement (REM) stage of sleep < 21 and 13%, respectively, REM sleep latency > 144 minutes, percentage of TST with pulse oxyhemoglobin saturation (SpO2) < 90% higher than 0.28 seconds, and an oxygen desaturation index higher than 0.92. CONCLUSION: Results suggest that clinically stable pediatric patients with CF have a high prevalence of SDB and present frequent sleep complaints, significant changes in sleep architecture, and episodes of oxygen desaturation during sleep.