Demographic, Clinical, And Laboratory Parameters Of Cystic Fibrosis During The Last Two Decades: A Comparative Analysis

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Background: In recent years, patients with cystic fibrosis (CF) have tended to experience a longer life expectancy and higher quality of life. In this context, the aim of the present study was to evaluate and compare the demographic, clinical, and laboratory markers of patients with CF during the last two decades at a CF referral center. Methods: A retrospective study of the demographic, clinical, and laboratory markers for CF treatment at a CF referral center was performed during two decades: 2000 (DI, 1990-2000, n = 104 patients) and 2010 (DII, 2000-2010, n = 181 patients). Results: The following variables were less common in DI than in DII: (i) pancreatic insufficiency, (ii) meconium ileus, (iii) diabetes mellitus, (iv) Burkholderia cepacia colonization, (v) moderate and severe Shwachman-Kulczycki score (SKS), (vi) F508del mutation screening, (vii) patients without an identified CFTR mutation (class IV, V, or VI mutation), (viii) patients above the 10th percentile for weight and height, (ix) restrictive lung disease, and (x) older patients (p < 0.01). The following variables were more common in DI than in DII: (i) excellent and good SKS, (ii) F508del heterozygous status, (iii) colonization by mucoid and nonmucoid Pseudomonas aeruginosa, iv) obstructive lung disease, and (v) minimal time for CF diagnosis (p < 0.01). Conclusion: Clinical outcomes differed between the two decades. Demographic, clinical, and laboratory markers in patients with CF are useful tools and should be encouraged in CF referral centers to determine the results of CF management and treatment, enabling a better understanding of this disease and its clinical evolution. Early diagnosis and management of CF will improve patients' quality of life and life expectancy until personalized drug therapy is possible for all patients with CF.15Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)FAEPEX- UnicampConselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)FAPESP [FALM: 2011/12939-4

Asthma: Gln27glu And Arg16gly Polymorphisms Of The Beta2-adrenergic Receptor Gene As Risk Factors.

Asthma is caused by both environmental and genetic factors. The ADRB2 gene, which encodes the beta 2-adrenergic receptor, is one of the most extensively studied genes with respect to asthma prevalence and severity. The Arg16Gly (+46A > G) and Gln27Glu (+79C > G) polymorphisms in the ADRB2 gene cause changes in the amino acids flanking the receptor ligand site, altering the response to bronchodilators and the risk of asthma through complex pathways. The ADRB2 polymorphisms affect beta-adrenergic bronchodilator action and are a tool to identify at-risk populations. To determine the frequency of these two polymorphisms in allergic asthma patients and healthy subjects and to correlate these data with the occurrence and severity of asthma. Eighty-eight allergic asthma patients and 141 healthy subjects were included in this study. The ADRB2 polymorphisms were analyzed using the amplification-refractory mutation system - polymerase chain reaction (ARMS-PCR) technique. The statistical analysis was performed with the SPSS 21.0 software using the Fisher's Exact and χ2 tests. The ADRB2 polymorphisms were associated with asthma occurrence. The Arg16Arg, Gln27Gln and Gln27Glu genotypes were risk factors; the odds ratios were 6.782 (CI = 3.07 to 16.03), 2.120 (CI = 1.22 to 3.71) and 8.096 (CI = 3.90 to 17.77), respectively. For the Gly16Gly and Glu27Glu genotypes, the odds ratios were 0.312 (CI = 0.17 to 0.56) and 0.084 (CI = 0.04 to 0.17), respectively. The haplotype analysis showed that there were associations between the following groups: Arg16Arg-Gln27Gln (OR = 5.108, CI = 1.82 to 16.37), Gly16Gly-Glu27Glu (OR = 2.816, CI = 1.25 to 6.54), Arg16Gly-Gln27Glu (OR = 0.048, CI = 0.01 to 0.14) and Gly16Gly-Gln27Glu (OR = 0.1036, CI = 0.02 to 0.39). The polymorphism Gln27Glu was associated with asthma severity, as the Gln27Gln genotype was a risk factor for severe asthma (OR = 2.798, CI = 1.099 to 6.674) and the Gln27Glu genotype was a protective factor for mild (OR = 3.063, CI = 1.037 to 9.041) and severe (OR = 0.182, CI = 0.048 to 0.691) asthma. The Arg16Gly and Gln27Glu polymorphisms in the ADRB2 gene are associated with asthma presence and severity.10

Microbiological Characteristics Of Sepsis In A University Hospital.

Microbiological characteristics of sepsis and antimicrobial resistance are well studied, although in State University of Campinas, no data has been published yet. The main agents related to sepsis and antimicrobial resistance were analyzed. The blood culture records requested from 4,793 hospitalized patients were analyzed. The samples were processed using the Bact/Alert system for agent identification and antimicrobial susceptibility. A total of 1,017 patients met the inclusion criteria for a sepsis diagnosis, with 2,309 samples tested (2.27 samples/patient). There were 489 positive samples (21% positive) isolated from 337 patients (33.13%), but more rigorous criteria excluding potential contaminants resulted in analysis being restricted to 266 patients (315 agents). The prevalent microorganisms were coagulase negative Staphylococcus (CNS) (15.87%), Escherichia coli (13.0%), Staphylococcus aureus (11.7%), Klebsiella pneumoniae (9.8%), Enterobacter sp (9.5%), Acinetobacter baumannii (9.2%), Pseudomonas aeruginosa (5.7%) and Candida sp (5.1%). Examining antimicrobial resistance in the agents revealed that 51% of the S. aureus isolates were methicillin-resistant S. aureus (MRSA) and 80% of the CNS isolates were oxacillin-resistant. For A. baumannii, the ideal profile drugs were ampicillin sulbactam and piperacillin/tazobactam, and for P. aeruginosa, they were piperacillin/tazobactam and ceftazidime. Enterobacteria showed on average 32.5% and 35.7% resistance to beta-lactams and ciprofloxacin, respectively. When all Gram-negative bacteria were considered, the resistance to beta-lactams rose to 40.5%, and the resistance to ciprofloxacin rose to 42.3%. Eighty percent of the agents identified in blood cultures from patients with sepsis belonged to a group of eight different agents. For empirical treatment, carbapenems and vancomycin unfortunately still remain the best therapeutic choice, except for A. baumannii and P. aeruginosa, for which piperacillin/tazobactan is the best option.155

Microbiological characteristics of sepsis in a university hospital

Microbiological characteristics of sepsis and antimicrobial resistance are well studied, although in State University of Campinas, no data has been published yet. The main agents related to sepsis and antimicrobial resistance were analyzed. The blood culture records requested from 4,793 hospitalized patients were analyzed. The samples were processed using the Bact/Alert (R) system for agent identification and antimicrobial susceptibility. A total of 1,017 patients met the inclusion criteria for a sepsis diagnosis, with 2,309 samples tested (2.27 samples/patient). There were 489 positive samples (21% positive) isolated from 337 patients (33.13%), but more rigorous criteria excluding potential contaminants resulted in analysis being restricted to 266 patients (315 agents). The prevalent microorganisms were coagulase negative Staphylococcus (CNS) (15.87%), Escherichia coli (13.0%), Staphylococcus aureus (11.7%), Klebsiella pneumoniae (9.8%), Enterobacter sp (9.5%), Acinetobacter baumannii (9.2%), Pseudomonas aeruginosa (5.7%) and Candida sp (5.1%). Examining antimicrobial resistance in the agents revealed that 51% of the S. aureus isolates were methicillin-resistant S. aureus (MRSA) and 80% of the CNS isolates were oxacillin-resistant. For A. baumannii, the ideal profile drugs were ampicillin sulbactam and piperacillin/tazobactam, and for P. aeruginosa, they were piperacillin/tazobactam and ceftazidime. Enterobacteria showed on average 32.5% and 35.7% resistance to beta-lactams and ciprofloxacin, respectively. When all Gram-negative bacteria were considered, the resistance to beta-lactams rose to 40.5%, and the resistance to ciprofloxacin rose to 42.3%. Eighty percent of the agents identified in blood cultures from patients with sepsis belonged to a group of eight different agents. For empirical treatment, carbapenems and vancomycin unfortunately still remain the best therapeutic choice, except for A. baumannii and P. aeruginosa, for which piperacillin/tazobactan is the best option15FAEPEX: Fundo de apoio ao ensino, a pesquisa e a extensã

Demographic, Clinical, And Laboratory Parameters Of Cystic Fibrosis During The Last Two Decades: A Comparative Analysis.

In recent years, patients with cystic fibrosis (CF) have tended to experience a longer life expectancy and higher quality of life. In this context, the aim of the present study was to evaluate and compare the demographic, clinical, and laboratory markers of patients with CF during the last two decades at a CF referral center. A retrospective study of the demographic, clinical, and laboratory markers for CF treatment at a CF referral center was performed during two decades: 2000 (DI, 1990-2000, n = 104 patients) and 2010 (DII, 2000-2010, n = 181 patients). The following variables were less common in DI than in DII: (i) pancreatic insufficiency, (ii) meconium ileus, (iii) diabetes mellitus, (iv) Burkholderia cepacia colonization, (v) moderate and severe Shwachman-Kulczycki score (SKS), (vi) F508del mutation screening, (vii) patients without an identified CFTR mutation (class IV, V, or VI mutation), (viii) patients above the 10th percentile for weight and height, (ix) restrictive lung disease, and (x) older patients (p < 0.01). The following variables were more common in DI than in DII: (i) excellent and good SKS, (ii) F508del heterozygous status, (iii) colonization by mucoid and nonmucoid Pseudomonas aeruginosa, (iv) obstructive lung disease, and (v) minimal time for CF diagnosis (p < 0.01). Clinical outcomes differed between the two decades. Demographic, clinical, and laboratory markers in patients with CF are useful tools and should be encouraged in CF referral centers to determine the results of CF management and treatment, enabling a better understanding of this disease and its clinical evolution. Early diagnosis and management of CF will improve patients' quality of life and life expectancy until personalized drug therapy is possible for all patients with CF.15

Epidemiological And Genetic Characteristics Associated With The Severity Of Acute Viral Bronchiolitis By Respiratory Syncytial Virus.

to assess the epidemiological and genetic factors associated with severity of acute viral bronchiolitis (AVB) by respiratory syncytial virus (RSV). the key words bronchiolitis, risk factor, genetics and respiratory syncytial virus, and all combinations among them were used to perform a search in the PubMed, SciELO, and Lilacs databases, of articles published after the year 2000 that included individuals younger than 2 years of age. a total of 1,259 articles were found, and their respective summaries were read. Of these, 81 were selected, which assessed risk factors for the severity of AVB, and were read in full; the 60 most relevant studies were included. The epidemiologic factors associated with AVB severity by RSV were prematurity, passive smoking, young age, lack of breastfeeding, chronic lung disease, congenital heart disease, male gender, ethnicity, viral coinfection, low weight at admission, maternal smoking during pregnancy, atopic dermatitis, mechanical ventilation in the neonatal period, maternal history of atopy and/or asthma during pregnancy, season of birth, low socioeconomic status, Down syndrome, environmental pollution, living at an altitude > 2,500 meters above sea level, and cesarean section birth. Conversely, some children with severe AVB did not present any of these risk factors. In this regard, recent studies have verified the influence of genetic factors on the severity of AVB by RSV. Polymorphisms of the TLRs, RANTES, JUN, IFNA5, NOS2, CX3CR1, ILs, and VDR genes have been shown to be associated with more severe evolution of AVB by RSV. the severity of AVB by RSV is a phenomenon that depends on the varying degrees of interaction among epidemiological, environmental, and genetic variables.89531-4

Epidemiological aspects of and risk factors for wheezing in the first year of life

Objective: To determine, in a sample of infants, the prevalence of and risk factors for occasional wheezing (OW) and recurrent wheezing wheezy baby syndrome (WBS). Methods: Parents of infants (12-15 months of age) completed the International Study of Wheezing in Infants questionnaire. Results: We included 1,269 infants residing in the city of Blumenau, Brazil. of those, 715 (56.34%) had a history of wheezing, which was more common among boys. the prevalences of OW and WBS were 27.03% (n = 343) and 29.31% (n = 372), respectively. On average, the first wheezing episode occurred at 5.55 +/- 2.87 months of age. Among the 715 infants with a history of wheezing, the first episode occurred within the first six months of life in 479 (66.99%), and 372 (52.03%) had had three or more episodes. Factors associated with wheezing in general were pneumonia; oral corticosteroid use; a cold; attending daycare; having a parent with asthma or allergies; mother working outside the home; male gender; no breastfeeding; and mold. Factors associated with WBS were a cold; physician-diagnosed asthma; ER visits; corticosteroid use; pneumonia; bronchitis; dyspnea; attending daycare; bronchodilator use; having a parent with asthma; no breastfeeding; mother working outside the home; and a dog in the household. Conclusions: the prevalence of wheezing in the studied population was high (56.34%). the etiology was multifactorial, and the risk factors were intrinsic and extrinsic (respiratory tract infections, allergies, attending daycare, and early wheezing). the high prevalence and the intrinsic risk factors indicate the need and the opportunity for epidemiological and genetic studies in this population. in addition, mothers should be encouraged to prolong breastfeeding and to keep infants under six months of age out of daycare.Univ Estadual Campinas, Fac Ciencias Med, Dept Pediat, Campinas, SP, BrazilWeb of Scienc

Saliva as a potential tool for cystic fibrosis diagnosis

BACKGROUND: Saliva and sweat are modified by cystic fibrosis (CF). In both cases the chloride and sodium ion concentrations for healthy subjects and CF patients differ, this representing a possible alternative tool for CF diagnosis. In this context, the aim of this study was to compare the concentrations of these ions in saliva samples taken from CF patients and healthy subjects. METHODS: A case–control study was carried out at a university CF center, in which the saliva samples were analyzed on an ABL 835 Radiometer(®) to determine the ion concentration. RESULTS: For the CF patients (n = 80) the values for the biochemical parameters of chloride, potassium and sodium ion concentration were higher (p < 0.009) and the volume and pH of the saliva were lower than in the case of healthy subjects (p < 0.009). For the healthy subjects group (n = 84) versus CF patients, according to the ROC curve, the values for sodium were: cutoff: 13.5 mmol/L, sensitivity: 73.4%, specificity: 70.6%; and for chloride: cutoff: 20 mmol/L, sensitivity: 68.1%, specificity: 72.9%. CONCLUSIONS: The chloride and sodium concentrations in the saliva samples were higher for CF patients in comparison with healthy subjects. Thus, saliva as a tool for CF diagnosis can be considered a new challenge, and a population study including patients in all age classes needs to be performed, in different countries over the world, to extend the database to include a broad spectrum of information in order to identify normal ion concentration ranges for CF patients according to age, genotype and environment. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/261423314875014

Lung ultrasound assessment of response to antibiotic therapy in cystic fibrosis exacerbations: a study of two cases

Sem informação456CONSELHO NACIONAL DE DESENVOLVIMENTO CIENTÍFICO E TECNOLÓGICO - CNPQ407364/2016-12011/12939-4; 2011/18845-1; 2015/12183-8; 2015/12858-5Sem informaçã

Walk Test And School Performance In Mouth-breathing Children.

In recent decades, many studies on mouth breathing (MB) have been published; however, little is known about many aspects of this syndrome, including severity, impact on physical and academic performances. Compare the physical performance in a six minutes walk test (6MWT) and the academic performance of MB and nasal-breathing (NB) children and adolescents. This is a descriptive, cross-sectional, and prospective study with MB and NB children submitted to the 6MWT and scholar performance assessment. We included 156 children, 87 girls (60 NB and 27 MB) and 69 boys (44 NB and 25 MB). Variables were analyzed during the 6MWT: heart rate (HR), respiratory rate, oxygen saturation, distance walked in six minutes and modified Borg scale. All the variables studied were statistically different between groups NB and MB, with the exception of school performance and HR in 6MWT. MB affects physical performance and not the academic performance, we noticed a changed pattern in the 6MWT in the MB group. Since the MBs in our study were classified as non-severe, other studies comparing the academic performance variables and 6MWT are needed to better understand the process of physical and academic performances in MB children.79212-