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Copyright of Revista Foco (Interdisciplinary Studies Journal) is the property of Revista Foco and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista Nutrición Clínica y Dietética Hospitalaria is the property of Sociedad Espanola de Dietetica y Ciencias de la Alimentacion and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: The Brazilian Unified National Health System (SUS) has incorporated newborn screening for cystic fibrosis since 2001. The protocol involves two samples of immunoreactive trypsinogen (IRT1/IRT2). This study aims to analyze fixed and floating values at the first and second IRT (IRT1/IRT2) cutoff points and assess the accuracy of the IRT/IRT methodology in a population from Northeastern Brazil. Descriptive, individual-level data from the newborn screening reference service data system (2013-2017) were used in this observational population study. The sensitivity, specificity, and positive predictive values (PPV) for the protocol were calculated. The best cutoff point was determined using the Youden’s index. The previous year’s cut-off values for the IRT1 and IRT2 99.4-, 99.5-, 99.6-, and 99.7-percentiles were utilized for the floating cutoff. During the studied period, 840,832 newborns underwent screening for cystic fibrosis, obtaining 49 cystic fibrosis diagnoses: 39 by newborn screening (79.6%) and 10 (20.4%) by clinical suspicion (false negative). The sensitivity, specificity, and PPV of the protocol totaled 79.6%, 99.9%, and 6.1%, respectively. No proposed cutoff for IRT1 performed better than the current one. IRT2 performed similarly to the current protocol at a cutoff point of 90ng/mL, showing the appropriate sensitivity and specificity while reducing the frequency of false positives. The protocol to screen newborns for cystic fibrosis had low sensitivity, a predictive positive value, and a high number of false positives and negatives. A floating cut point for IRT1 or IRT2 seems to constitute no viable option. However, changing the IRT2 cut point from 70ng/mL to 90ng/mL seems to have advantages and should undergo consideration.

We present the case of a 3-year-old boy with a diagnosis of asthma, allergic rhinitis, dysmorphic craniofacial characteristics and recurrent upper and lower respiratory infections, managed as asthma from the beginning. As part of the study of comorbidities, it was decided to carry out a sweat test that came out in the intermediate range and later one mutation was found, where a positive result was obtained for a copy that is associated with cystic fibrosis. The case will be reviewed, as well as the diagnosis, symptoms and treatment of the metabolic syndrome related to the cystic fibrosis transmembrane conductance regulator (CRMS).

ABSTRACT Objective: To investigate the effect of bronchodilator on the respiratory mechanics and pulmonary function of children and adolescents with cystic fibrosis. Methods: Cross-sectional study on clinically stable children and adolescents with cystic fibrosis aged from six to 15 years. Participants underwent impulse oscillometry and spirometry evaluations before and 15 minutes after bronchodilator inhalation. The Kolmogorov-Smirnov test was applied to verify the sample distribution, and the Student's t-test and Wilcoxon test were used to compare the data before and after bronchodilator inhalation. Results: The study included 54 individuals with a mean age of 9.7±2.8 years. The analysis showed a statistically significant improvement in impulse oscillometry and spirometry parameters after bronchodilator inhalation. However, according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) recommendations (2020 and 2021), this improvement was not sufficient to classify it as a bronchodilator response. Conclusions: The use of bronchodilator medication improved respiratory mechanics and pulmonary function parameters of children and adolescents with cystic fibrosis; however, most patients did not show bronchodilator response according to ATS/ERS recommendations.

Abstract Introduction: cystic fibrosis newborn screening must enable its earlier diagnosis, which may enhance outcomes. This study was a series case of delayed-diagnosis children submitted to cystic fibrosis newborn screening. Description: fourteen children were included; eight (57.1%) were due to false-negative screening, while six (42.9%) were due to processing errors. Two samples collected after 30 days of life were incorrectly classified as negative, and four infants with a positive test could not be located due to screening processing errors. Cystic fibrosis diagnosis was confirmed at a median (IQR) age of 5.3 (4.2-7.4) months. Poor nutritional status was the most prevalent clinical sign at diagnosis, being present in 78.6% of infants. The mean (SD) weight-for-length and length-for-age Z-scores were -3.46 (0.84) and -3.99 (1.16), respectively. Half of the children had Pseudo-Bartter syndrome, and 42.9% had breathing difficulties. Twelve children (85.7%) required hospitalization, with a median (IQR) length of stay of 17.0 (11.5-26.5) days. Discussion: newborn screening had some faults, from incorrect collections to inefficient active search. Early identification of these children in which screening was unsatisfactory is essential, emphasizing the importance and efforts to not miss them. In the case of a failed test, healthcare professionals must be prepared to recognize the main symptoms and signs of the disease.

Copyright of Revista Foco (Interdisciplinary Studies Journal) is the property of Revista Foco and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

ABSTRACT Objective: To evaluate the impact of COVID-19 social distancing recommendations on nutritional status, pulmonary function, and morbidity in patients with cystic fibrosis (CF). Methods: A retrospective cohort study including patients older than six years with a diagnosis of CF was performed. Demographic and clinical data, anthropometric measurements, pulmonary function, days of antibiotic use, and length of hospital stay were recorded. Variables were recorded at three time points relative to the baseline for implementation of social distancing measures: T-1 (14 months before implementation), T0 (baseline), and T1 (14 months after implementation). Delta (Δ) was calculated for each period: Δ1 (pre-pandemic T0-T-1) and Δ2 (pandemic T1-T0). Results: The study included 25 patients, with a mean age of 11.7±4.3 years. The mean forced expiratory volume in the first second (FEV1) was 85.6±23.6%, and body mass index (BMI) was 17.5±3.0 kg/m2. When comparing the two periods (Δ1 and Δ2), there was a significant increase in the FEV1/forced vital capacity (FVC) ratio (p=0.013) and in the forced expiratory flow between 25 and 75% of vital capacity (FEF25–75%) (p=0.037) in the pandemic period. There was also a significant reduction (p=0.005) in the use of antibiotics in the pandemic period compared with the pre-pandemic period. The Δ1 and Δ2 values did not differ significantly for BMI, FEV1, or length of hospital stay. Conclusions: COVID-19 social distancing recommendations had a positive impact (decrease) on morbidity (use of antibiotics) and small airway obstruction (FEF25–75%) in patients with CF.

Copyright of Brazilian Journal of Pulmonology / Jornal Brasileiro de Pneumologia is the property of Sociedade Brasileira de Pneumologia e Tisiologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract Objective: To revise the impact of telehealth on the quality of life, reduction in pulmonary exacerbations, number of days using antibiotics, adherence to treatment, pulmonary function, emergency visits, hospitalizations, and the nutritional status of individuals with asthma and cystic fibrosis. Data source: Four databases were used, MEDLINE, LILACS, Web of Science and Cochrane, as well as manual searches in English, Portuguese and Spanish. Randomized clinical trials, published between January 2010 and December 2020, with participants aged 0 to 20 years, were included. Data synthesis: Seventy-one records were identified after the removal of duplicates; however, twelve trials were eligible for synthesis. Included trials utilized: mobile phone applications (n=5), web platforms (n= 4), mobile telemedicine unit (n=1), software with an electronic record (n=1), remote spirometer (n=1), and active video games platform (n=1). Three trials used two tools, including telephone calls. Among the different types of interventions, improvement in adherence, quality of life, and physiologic variables were observed for mobile application interventions and game platforms compared to usual care. Visits to the emergency department, unscheduled medical appointments, and hospitalizations were not reduced. There was considerable heterogeneity among studies. Conclusions: The findings suggest that better control of symptoms, quality of life, and adherence to treatment can be attributed to the technological interventions used. Nevertheless, further research is needed to compare telehealth with face-to-face care and to indicate the most effective tools in the routine care of children with chronic lung diseases.

ABSTRACT Cystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.

ABSTRACT Cystic Fibrosis (CF) is a genetic disease that reduces quality of life. Lung transplantation (LTx) is a strategy for end-stage lung disease treatment in CF. Pulmonary rehabilitation (PR) in LTx is effective, however, only one study has determined its effectiveness in children, and most studies have not included CF exclusively. Thus, reports showing components for PR protocols and outcomes not considered in previous studies of PR in LTx due to CF in children are still needed. To report this case, written informed assent and consent of patient and parent were obtained. Ethical Requirement was formally waived by the institution. A 12-year-old patient with CF was referred to PR due to LTx. A general and respiratory training was conducted daily for six months (pre) and two years (post) the transplantation, with the parents’ full support. General training included treadmill and cycle ergometer use and upper limbs exercises. Respiratory protocol included inspiratory training and respiratory physical therapy. We observed improvements in pulmonary function, exercise capacity, inspiratory muscle strength, and quality of life, including school functioning, with progress maintenance after 2.5 years of continuous intervention. This case presents a PR protocol pre- and post-LTx with good long-term results. These components for treatment protocols and outcomes may be useful to consider in clinical interventions or future investigations.

Abstract Objective: This study aimed to identify methodological aspects involved in determining anthropometric measurements among studies assessing the nutritional status of individuals with cystic fibrosis (CF). Methods: A search of the literature was performed on MEDLINE via Pubmed, Embase, and Web of Science databases. The population comprised children and adolescents with CF. Observational studies and clinical trials using anthropometric and body composition measures and indices determined by dual-energy X-ray absorptiometry (DXA) and bioelectrical impedance assessment (BIA) were included. Use of a standardized procedure for data collection was defined when details on the instruments and their calibration were given, the measuring procedures were described, and when it was clear measures had been determined by a trained team, or the use of an anthropometric reference manual was cited. Data extracted were expressed as absolute and relative frequencies. Results: A total of 32 articles were included, and a total of 233 measures or indices were observed. The most frequently used measures were body mass index (kg/m2; 35%), weight (kg; 33%), and height (cm; 33%). Among the 28 studies that used anthropometric measures, 21 (75%) provided a complete or partial description of the measurement instruments used, 3 (11%) reported information on equipment calibration, 10 (36%) indicated the measurement procedures employed by assessors, and 2 (7%) stated a trained team had carried out the measurements. Conclusions: The poor description of measuring procedures precluded a meaningful evaluation of data quality. Scientific debate on this theme can help raise awareness of the need to ensure quality in collecting and fully presenting data.

ABSTRACT Objective: To evaluate the effectiveness of treatment with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) and to characterize its safety profile in cystic fibrosis (CF) patients in a real-world clinical setting. Methods: This was a prospective observational study carried out in a CF referral center in Portugal involving adult CF patients who started treatment with ELX/TEZ/IVA. Clinical characteristics of the patients were collected, and effectiveness and safety data were evaluated. Results: Of the 56 patients followed in the center at the time of the study, 28 were eligible for ELX/TEZ/IVA treatment in accordance with the Portuguese National Authority for Medicines and Health Products at the time of the study. Of these, 24 met the follow-up time requirement to be included in the clinical effectiveness analysis. The mean follow-up time was 167.3 ± 96.4 days. Adverse events were generally mild and self-limited. Significant improvements in lung function, BMI, sweat chloride concentration, and number of pulmonary exacerbations were observed. No significant differences in outcomes between F508del homozygous and heterozygous patients were found. The effectiveness of this new CFTR modulator combination also applied to patients with advanced lung disease. Conclusions: Treatment with ELX/TEZ/IVA showed effective improvement in real-world clinical practice, namely in lung function, BMI, sweat chloride concentration, and number of pulmonary exacerbations, with no safety concerns.

ABSTRACT Objective: To analyze the characteristics of cystic fibrosis (CF) care centers (CFCCs) in Brazil. Methods: A questionnaire was sent to the coordinators of all 51 registered CFCCs between May and September of 2021. Results: The response rate was 100%. Southeastern Brazil is the region where most of the CFCCs in the country are located (21 centers; 41%), followed by the southern and northeastern regions (11 centers each; 21.5%), the central-western region (6; 12%), and the northern region (2; 4%). A total of 4,371 patients with CF were cared for in Brazil during the study period, ranging from 7 to 240 patients per center (mean, 86 patients/center; median, 75 patients/center); 2,197 patients (50%) were cared for in centers in the southeastern region of the country, particularly in the state of São Paulo (33%), the remaining patients being treated in southern Brazil (1,014 patients, 23%), northeastern Brazil (665 patients, 15%), central-western Brazil (354 patients, 8%), and northern Brazil (141 patients, 4%). Overall, 47 centers (92%) reported having an incomplete multidisciplinary team; 4 (8%) lacked essential team members; 6 (12%) lacked a physical therapist; 5 (10%) lacked a dietitian; 17 (33%) lacked outpatient nursing care; 13 (25%) lacked outpatient social work services; 14 (27%) lacked a psychologist; and 32 (63%) lacked a clinical pharmacist. Seven CFCCs (14%) in the northern and northeastern regions of Brazil reported that the quality of newborn screening for CF was poor. All centers reported having difficulties in accessing CF medications. Conclusions: Brazilian CFCCs experience multiple problems, including inadequate staffing, infrastructure, testing, and medication supply. There is an urgent need to regulate the implementation of CF referral centers and an appropriate network structure for the diagnosis and follow-up of CF patients using optimal treatment recommendations.

Copyright of Interface - Comunicação, Saúde, Educação is the property of Fundacao UNI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Purpose: To evaluate the nutritional, food and biochemical profile of children and adolescents with cystic fibrosis (CF) treated at a reference center in southern Brazil. Methods: Crosssectional study including patients with CF at a pediatric center in Florianópolis, from 2019 to 2020. Questionnaires with questions on sociodemographic, clinical, biochemical, 24- hour dietary recall, and anthropometric measurements were applied. Participants were stratified into age groups, infants (under 2 years); preschoolers (2-5 years), schoolchildren (5-10 years), and adolescents (≥ 10 years). Discussion: Periodic monitoring of nutritional status should be part of the routine of patients with CF, being essential to prevent the decline of the clinical picture. Results: Of the 102 patients included in the study, 52.9% were male, mostly white (87.3%), with a mean age of 6.11±4.63 years. Most patients (84.3%) were classified as eutrophic. When comparing the age groups, 81% of the infants had an excellent clinical score, the nutritional supplement was more used in the school group (90.9%); adolescents were diagnosed later (11.55±26.40 months), and 19.4% of them had CF-related diabetes. n infants, the serum albumin level had a lower mean and the serum phosphorus level showed a slight increase in relation to the reference value. Conclusion: In the present study, a decline in clinical score scores, the prevalence of CF-related diabetes, and higher food intake among adolescents were observed. [ABSTRACT FROM AUTHOR]

ABSTRACT Objective: There is still limited information on the clinical characteristics and outcomes of cystic fibrosis (CF) patients with COVID-19 in Brazil. The objective of this study was to describe the cumulative incidence of COVID-19 in CF patients, as well as their clinical characteristics and outcomes. Methods: This was a prospective cohort study involving unvaccinated adult CF patients and conducted during the first year of the SARS-CoV-2 pandemic in the city of Porto Alegre, in southern Brazil. The clinical course of the disease was rated on the WHO Ordinal Scale for Clinical Improvement. The primary outcome was the number of incident cases of COVID-19. Results: Between April 30, 2020 and April 29, 2021, 98 CF patients were included in the study. Seventeen patients were diagnosed with COVID-19. For the CF patients, the annual cumulative incidence of COVID-19 was 17.3%, similar to that for the general population, adjusted for age (18.5%). The most common symptoms at diagnosis of COVID-19 were cough (in 59%), dyspnea (in 53%), fatigue (in 53%), and fever (in 47%). Only 6 (35%) of the patients required hospitalization, and 3 (17.6%) required oxygen support. Only 1 patient required mechanical ventilation, having subsequently died. Conclusions: During the first year of the SARS-CoV-2 pandemic in southern Brazil, the cumulative incidence rate of COVID-19 was similar between CF patients and the general population. More than 50% of the CF patients with SARS-CoV-2 infection had a mild clinical presentation, without the need for hospital admission, and almost the entire sample recovered completely from the infection, the exception being 1 patient who had advanced lung disease and who died.

ABSTRACT Objective: Pulmonary disease in cystic fibrosis (CF) is characterised by recurrent episodes of pulmonary exacerbations (PExs), with acute and long-term declines in lung function (FEV1). The study sought to determine whether routine spirometry increases the frequency of PEx diagnosis, resulting in benefits to long-term pulmonary function. Methods: CF patients in the 5- to 18-year age bracket were followed for 1 year, during which they underwent spirometry before every medical visit. The main variables were the frequency of PEx diagnosis and use of antibiotics; the use of spirometry as a criterion for PEx diagnosis (a decline ≥ 10% in baseline FEV1); and median percent predicted FEV1 over time. The data were compared with those for the previous 24-month period, when spirometry was performed electively every 6 months. Results: The study included 80 CF patients. PExs were diagnosed in 27.5% of the visits, with a mean frequency of 1.44 PExs per patient/year in 2014 vs. 0.88 PExs per patient/year in 2012 (p = 0.0001) and 1.15 PExs per patient/year in 2013 (p = 0.05). FEV1 was used as a diagnostic feature in 83.5% of PExs. In 21.9% of PExs, the decision to initiate antibiotics was solely based on an acute decline in FEV1. The median percent predicted FEV1 during the follow-up year was 85.7%, being 78.5% in 2013 and 76.8% in 2012 (p > 0.05). The median percent predicted FEV1 remained above 80% during the two years after the study. Conclusions: Routine spirometry is associated with higher rates of diagnosis and treatment of PExs, possibly impacting long-term pulmonary function.

ABSTRACT Objective To develop a scientific consensus on nutrition in cystic fibrosis. Methods Sixteen coordinators elaborated relevant questions on nutritional therapy in cystic fibrosis, which were divided into six sections: nutritional assessment, nutritional recommendations, nutritional intervention, dietary counseling, special situations and enzyme replacement, and gastrointestinal manifestations. Two to three specialists in the field were responsible for each section and obtaining answers formulated based on standardized bibliographic searches. The available literature was searched in the PubMed®/MEDLINE database, after training and standardization of search strategies, to write the best level of evidence for the questions elaborated. Issues related to disagreement were discussed until a consensus was reached among specialists, based on the current scientific literature. Results Forty-two questions were prepared and objectively answered, resulting in a consensus of nutritional therapy in cystic fibrosis. Conclusion This work enabled establishing a scientific consensus for nutritional treatment of cystic fibrosis patients.

La justicia de la provincia de Córdoba hizo lugar al pedido de una medida cautelar autorizando el 100% de la cobertura del medicamento TRIKAFTA para el tratamiento de fibrosis quística. Se trata de una joven, Sabrina Monteverde, quien padece esta enfermedad poco frecuente y que dado al estado grave de salud que atraviesa, debió iniciar acción de amparo contra Apross. Si bien, no es el primer caso de un paciente que requiere la medicación mencionada, la historia de Sabrina resonó en los medios de comunicación, alcanzando rápidamente una gran adhesión y apoyo social. Poco tiempo después de esta resolución, el Congreso de la Nación Argentina sancionó la Ley 27.552 de lucha contra la enfermedad de fibrosis quística de páncreas o mucoviscidosis. Ley que permitió al Estado Nacional dar un paso firme en la protección de los derechos humanos fundamentales de un grupo vulnerable de nuestra sociedad. Adicionalmente, para ese tiempo, Argentina estaba comenzando a sentir el peso de las primeras medidas del aislamiento social preventivo y obligatorio en torno a la pandemia mundial, ocasionada por el COVID-19.

Cystic fibrosis (CF) is an autosomal recessive genetic disease characterized by the dysfunction of a protein called cystic fibrosis transmembrane conductance regulator (CFRT), affecting multiple systems and thickening airway secretions. Respiratory physiotherapy has as main role to promote the removal of accumulated secretions in the lungs due to the dysfunctions that cystic fibrosis entails, being one of the therapeutic resources indispensable for the maintenance, prevention and promotion of the patient's health. Low adherence to treatment is one of the main causes of worsening of the clinical situation becoming a difficulty for physiotherapists. The aim of this research is to analyze the importance and benefits for the pediatric patient with cystic fibrosis in joining respiratory physiotherapy. An integrative bibliographical review was carried out, which identified 817 publications of the subject, articles were excluded that the public studied or method did not correspond to the one proposed in the research; after the refinement of the search, 21 original articles were included. Respiratory physiotherapy enters as one of the traditional methods of treatment, aiding in the removal of secretions with bronchial hygiene techniques. In the studied population, the low adherence to the therapy is related to the dependency of those responsible and the lack of knowledge of the future implications of the disease. It is up to the professional who works in this area to explain and clarify to parents and patients that respiratory therapy is equally important for drug therapy and that both act together in the treatment of cystic fibrosis. It is concluded that low adherence is directly related to the lack of information about the disease and that parents and professionals have to promote daily the education of this patient in relation to their pathology. [ABSTRACT FROM AUTHOR]

Introduction: Nutrition plays an essential role in the survival and quality of life (QOL) of patients with cystic fibrosis (CF). Objective: This study aims to assess the relationship between nutritional status and QOL in children and adolescents with CF. Materials and methods: It is a cross-sectional study that analyzed patients aged 2 to 14 years with CF between May 2019 and March 2020. We analyzed sociodemographic data, clinical characteristics, nutritional status, and QOL by using the Cystic Fibrosis Questionnaire and Pediatric Quality of Life Inventory. Patients were divided into two groups by body mass index (BMI < or = 50th percentile). Discussion: Periodic monitoring of nutritional status should be part of the routine of CF patients, being essential to prevent the decline in clinical status and QOL. Results: 81 patients participated in the study, 56.8% male, 87.7% white; 41.3% were in the BMI = 50th percentile. When comparing the QOL domains concerning the BMI percentile, we observed a statistical difference in the domains of eating and weight for children aged 6 to 11 years. As for the domains of body image and eating, the participants were 12/13-year-old adolescents who's those in the BMI <50th percentile, obtained the lowest score. There was an association between QOL with age, sex, nutritional status, and clinical variables of CF patients. Conclusion: Patients who were at BMI <50th percentile obtained the lowest scores in the domains of eating, weight, and body image, supporting the hypothesis that nutritional status negatively affects QOL, especially in these domains. [ABSTRACT FROM AUTHOR]

ABSTRACT Objective: To assess the association of sleep disorders with the findings of heart rate variability (HRV) in children and adolescents with cystic fibrosis (CF). Methods: Cross-sectional study including children and adolescents aged six to 18 years with a clinical diagnosis of CF. Sociodemographic and clinical data were collected. Sleep disorders were evaluated using baseline nocturnal polysomnography. The autonomic nervous system (ANS) was evaluated through resting HRV. Results: A total of 30 individuals (11.2 years) with a mean forced expiratory volume in the first second (FEV1) of 62.7% were included. The respiratory disturbance index presented a median of 2.6 and obstructive sleep apnea syndrome (OSAS) was identified in 30%. In the HRV analysis, a mean standard deviation of all inter-beat (RR) intervals (SDNN) of 60.8±45.9ms was found. There was a significant correlation between the HRV low-frequency/high-frequency (LF/HF) global modulation index and the minimum SpO2 during sleep in patients with FEV1

ABSTRACT Objective: Massive hemoptysis is one of the most serious complications in patients with cystic fibrosis (CF). This study aimed to evaluate the hemoptysis-free period following bronchial and non-bronchial artery embolization (BAE/non-BAE) in CF patients and to investigate predictors of recurrent bleeding and mortality by any cause. Methods: This was a retrospective cohort study of CF patients ≥ 16 years of age undergoing BAE/non-BAE for hemoptysis between 2000 and 2017. Results: We analyzed 39 hemoptysis episodes treated with BAE/non-BAE in 17 CF patients. Hemoptysis recurrence rate was 56.4%. Of the sample as a whole, 3 (17.6%) were hemoptysis-free during the study period, 2 (11.8%) underwent lung transplantation, and 3 (17.6%) died. The median hemoptysis-free period was 17 months. The median hemoptysis-free period was longer in patients with chronic infection with Pseudomonas aeruginosa (31 months; 95% CI: 0.00-68.5) than in those without that type of infection (4 months; 95% CI: 1.8-6.2; p = 0.017). However, this association was considered weak, and its clinical significance was uncertain due to the small number of patients without that infection. Conclusions: BAE appears to be effective in the treatment of hemoptysis in patients with CF.

Copyright of Estilos da Clínica is the property of FEUSP - Faculdade de Educacao da USP - Universidade de Sao Paulo and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract Objectives To assess the perinatal and maternal outcomes of pregnant women with cystic fibrosis (CF) and severe lung impairment. Methods This was a series of cases aiming to review the maternal and fetal outcomes in cases of singleton pregnant women with a diagnosis of CF. We have included all of the cases of singleton pregnancy in patients with CF who were followed-up at the obstetrics department of the Medical School of the Universidade de São Paulo, between 2003 and 2016. The exclusion criteria were the unattainability of the medical records of the patient, and delivery at other institutions. A forced expiratory volume in 1 second < 50% was considered as severe lung impairment. We have also analyzed data regarding maternal hospitalization and respiratory exacerbations (REs). Results Pregnant women with CF and severe lung impairment did not present an association with spontaneous prematurity, fetal growth restriction or fetal demise. All of the cases involved multiple RE episodes requiring antibiotic therapy. The median (range) of events per patient was of 4 (2-4) events. Conclusion Cystic fibrosis patients with severe lung impairment may achieve successful term pregnancies. However, pregnancies of women with CF are frequently complicated by REs, and this population may require hospital admission during the course of the pregnancy. Cystic fibrosis patients should be followed by a specialized team with experience in treating respiratory diseases.

Introduction: Inhalation therapy is a crucial part of the cystic fibrosis (CF) treatment regimen. Drugs that assist in mucociliary clearance and inhaled antibiotics are used by most patients. Methods: This is a cross-sectional study where patients with CF and their caregivers answered questionnaires regarding their adherence to inhalation therapy and QoL. Demographic, spirometric, and bacteriological data, as well as S-K scores and hospitalization frequencies were also collected. Results: We included 66 patients in this study; participants had a mean age of 12.3 years and Z-scores of -1.4 for forced expiratory volume in 1 second and 48.6 for body mass index. Patients were divided into 2 groups according to their self-reported adherence to inhalation therapy: high adherence (n = 46) and moderate/low adherence (n = 20). When comparing both groups, there was no statistically significant differences in age, sex, family income, and S-K score (p > 0.05). The high-adherence group had had shorter hospitalization periods in the previous year (p = 0.016) and presented better scores in the following domains of the QoL questionnaire: emotion (p = 0.006), eating (p = 0.041), treatment burden (p = 0.001), health perception (p = 0.001), and social (p = 0.046). Conclusions: A low self-reported adherence to inhalation therapy recommendations was associated with longer hospitalizations in the previous year and with a decrease in QoL in pediatric patients with CF.

ABSTRACT Background: Meconium ileus is a common cause of intestinal obstruction in neonates that different surgical methods have been described for its management such as Santulli and loop ileostomy. Aim: To evaluate and compare clinical efficacy of Santulli and loop ileostomy in neonates with meconium ileus. Methods: In this retrospective study, 58 patients with meconium ileus were evaluated. After analyses of hospital records, 53 patients with completed hospital records were included. Demographic information, surgery parameters and postoperative complications were extracted from the hospital records or calling parents. Results: Skin excoriation (21.4% vs. 84%, p

Objective: to analyze the prevalence of diseases diagnosed by neonatal screening in a region of Mato Grosso, Brazil. Method: descriptive and quantitative study, with analysis of neonatal screening of 4057 newborns in municipalities of Mato Grosso, using secondary data from the state neonatal screening service in the state. Results: among diagnosed patients, there was a prevalence of congenital hypothyroidism (1:1014), cystic fibrosis (1:2029), phenylketonuria (1:4057) and sickle cell anemia (1:4057). Conclusion: the percentage of neonatal screening coverage in the studied region was considered below the average for this state and country. The most common diseases were congenital hypothyroidism and cystic fibrosis. [ABSTRACT FROM AUTHOR]

Objectives: This study primarily aimed to investigate the clinical determinants of the Modified Incremental Step Test (MIST) in adults with non-cystic fibrosis bronchiectasis (NCFB). A secondary objective was to compare the cardiopulmonary responses after the MIST and Incremental Shuttle Walk Test (ISWT), two commonly adopted symptom-limited maximum field tests in chronic respiratory diseases., Methods: Forty-six patients with clinically stable bronchiectasis participated in this cross-sectional study. MIST and ISWT were performed to determine exercise capacity, while disease severity, fatigue, and quality of life were assessed using the Bronchiectasis Severity Index (BSI), the Fatigue Severity Scale (FSS), and St. George's Respiratory Questionnaire (SGRQ), respectively. Quadriceps muscle strength was evaluated using a hand-held dynamometer, walking speed with a wireless inertial sensing device, and the level of physical activity (steps/day) with a pedometer., Results: The BSI score, quadriceps muscle strength, daily step count, and the SGRQ total score explained 61.9% of the variance in the MIST (p < 0.001, R2 = 0.67, AR2 = 0.619). The BSI score (r = -0.412, p = 0.004), quadriceps muscle strength (r = 0.574, p = 0.001), daily step count (r = 0.523, p < 0.001), walking speed (r = 0.402, p = 0.006), FSS score (r = -0.551, p < 0.001), and SGRQ total score (r = -0.570, p < 0.001) correlated with the MIST. The patients achieved higher heart rates (HR), HR%, desaturation, dyspnea, and leg fatigue in the MIST compared to the ISWT (p < 0.05)., Conclusions: Disease severity, quadriceps muscle strength, physical activity level, and quality of life were determinants of MIST. The advantages of the MIST, including higher cardiopulmonary response than ISWT and greater portability, which facilitates its use in various settings, make MIST the preferred choice for investigating symptom-limited exercise capacity in patients with NCFB.

The Hospital of the Ribeirão Preto Medical School, University of São Paulo is one of the three screening centers in São Paulo State, Brazil, and has included a test for cystic fibrosis (CF) since February 6, 2010, by a court order. We evaluated the first five years of this CF-newborn screening program. The original immunoreactive trypsinogen (IRT)/IRT screening protocol was adopted in Brazil. A total of 173,571 newborns were screened, 1,922 (1.1%) of whom showed IRT1 ≥ 70ng/mL. Of these, 1,795 (93.4%) collected IRT2, with elevated results (IRT2 ≥ 70ng/mL) in 102 of them (5.2%). We identified a total of 26 CF cases during this period, including three CF cases that were not detected by the CF-newborn screening. The incidence of the disease among the screened babies was 1:6,675 newborns screened. Median age at the initial evaluation was 42 days, comparable to that of neonates screened with the IRT/DNA protocol. Almost all infants with CF already exhibited some manifestations of the disease during the neonatal period. The mutation most frequently detected in the CF cases was F508del. These findings suggest the early age at the beginning of treatment at our center was due to the effort of the persons involved in the program regarding an effective active search. Considering the false negative results of CF-newborn screening and the early onset of clinical manifestations of the disease in this study, pediatricians should be aware of the diagnosis of CF even in children with negative test.

ABSTRACT Objective: To evaluate factors associated with the performance of children and adolescents with cystic fibrosis (CF) in the Modified Shuttle Test (MST) and compare it with healthy children and adolescents. Methods: This is a cross-sectional study, with children and adolescents divided into two groups: cystic fibrosis (CFG) and control (CG). Variables evaluated in the MST: walking distance, test level, heart rate variation (∆Hr), post-test mean arterial pressure (MAP Pt) and peripheral oxygen saturation variation (∆SPO2). Statistical analysis included Mann Whitney and Spearman coefficient tests, being significant p

ABSTRACT Objective: To verify the association between quality of life, functional capacity and clinical and nutritional status in children and adolescents with cystic fibrosis (CF). Methods: Cross-sectional study, including patients from eight to 18 years old with CF. Quality of life, functional capacity, nutritional status and clinical status were evaluated with the Cystic Fibrosis Questionnaire; the 6-minute walk test (6MWT) and manual gripping force (MGF); the height percentiles for age and body mass index for age and respiratory function test, respectively. Pearson and Spearman correlation tests and logistic regression were used to analyze the data. Results: A total of 45 patients, 13.4±0.5 years old, 60% female, 60% colonized by Pseudomonas aeruginosa and 57.8% with at least one F508del mutation participated in the study. When assessing the perception of quality of life, the weight domain reached the lowest values, and the digestive domain, the highest. In the pulmonary function test, the forced expiratory volume of the first second was 77.3±3.3% and the 6MWT and MGF presented values within the normal range. There was an association between quality of life and functional capacity, nutritional status and clinical status of CF patients. Conclusions: The study participants had good clinical conditions and satisfactory values of functional capacity and quality of life. The findings reinforce that the assessment of quality of life may be important for clinical practice in the management of treatment.

Cystic fibrosis (CF) is an autosomal recessive genetic disease characterized by the dysfunction of a protein called cystic fibrosis transmembrane conductance regulator (CFRT), affecting multiple systems and thickening airway secretions. Respiratory physiotherapy has as main role to promote the removal of accumulated secretions in the lungs due to the dysfunctions that cystic fibrosis entails, being one of the therapeutic resources indispensable for the maintenance, prevention and promotion of the patient's health. Low adherence to treatment is one of the main causes of worsening of the clinical situation becoming a difficulty for physiotherapists. The aim of this research is to analyze the importance and benefits for the pediatric patient with cystic fibrosis in joining respiratory physiotherapy. An integrative bibliographical review was carried out, which identified 817 publications of the subject, articles were excluded that the public studied or method did not correspond to the one proposed in the research; after the refinement of the search, 21 original articles were included. Respiratory physiotherapy enters as one of the traditional methods of treatment, aiding in the removal of secretions with bronchial hygiene techniques. In the studied population, the low adherence to the therapy is related to the dependency of those responsible and the lack of knowledge of the future implications of the disease. It is up to the professional who works in this area to explain and clarify to parents and patients that respiratory therapy is equally important for drug therapy and that both act together in the treatment of cystic fibrosis. It is concluded that low adherence is directly related to the lack of information about the disease and that parents and professionals have to promote daily the education of this patient in relation to their pathology. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the degree of adherence to physiotherapeutic treatment in adolescents with Cystic Fibrosis (CF) followed up in a multidisciplinary program. Method: Observational cross-sectional study with 14 patients from the multidisciplinary CF outpatient clinic from May 2016 to October 2016. A questionnaire was used to determine the adherence of physiotherapy. Together with the adhesion of the drug alpha dornase, analyzed by the Morisky-Green Test and the severity of the disease was evaluated by applying the Shwachman-Kulczycki Score. Results: Patients' ages ranged from a mean of 15.14 years, of which 8 (57.1%) were male. According to the degree of adhesion, 5 (35,7%) were classified with low adhesion, 4 (28,7%) with moderate adhesion and 5 (35,7%) with high adhesion. The Shwachman-Kulczycki score was classified as excellent in 6 (48.8%), good 4 (28.5%), mild 3 (21.4%), moderate 1 (7.1%), severe (0%). In the present study, no correlation was observed between the adherence of physiotherapy and the severity score, and also with the use of the alphadornase medication. Conclusion: Satisfactory (moderate to high) adherence to respiratory physiotherapy was observed in 64.3% of the CF adolescents investigated. However, a balance between low adherence and high adherence (35.7%) for this treatment was noted. [ABSTRACT FROM AUTHOR]

Copyright of Jornal de Pediatria is the property of Sociedade Brasileira de Pediatria and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Cystic fibrosis is an autosomal recessive genetic disease whose pulmonary involvement is the main cause of morbidity and mortality. Objective: To describe the combination of CFTR protein mutations and the functional status of adolescents with Cystic Fibrosis treated at a referral center. Methods: A case series study was carried out, in which 11 adolescents chronically colonized by Pseudomonas aeruginosa were included in the respiratory physiotherapy outpatient clinic of the Fernandes Figueira Institute (IFF / Fiocruz). Patient identification data, genetic study, anthropometric data, pulmonary function and functional evaluation were collected. Results: Of the patients evaluated, 3 were male and 8 female, with ages varying between 14 and 18 years. The most present individual mutation was DF508. The greatest impairment of lung function (forced expiratory volume in the 1st second of 30% predicted) and shorter distance walked in the 6-minute walk test (64% predicted) was observed in the patient with the combination of DF508 / R1066C mutations. In dynamometry, the worst performance was observed in the patient with the DF508 / R334W mutations (45% predicted). Regarding nutritional status, the lowest percentile of the body mass index for age (1.4) was observed in the patient whose combination of mutations was DF508 / Y913k. Conclusions: We observed that the genetic study in CF is fundamental for the understanding of the clinical and functional picture and for the establishment of individualized therapeutic behaviors. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the effects of a pulmonary rehabilitation program on functional capacity and pulmonary function in patients with cystic fibrosis. Methodology: This is a randomized, controlled clinical trial. The sample consisted of 14 participants, divided into experimental group (GE) and control group (CG), in which the EG was submitted to a protocol of aerobic exercises and supervised respiratory physiotherapy and the GC to unsupervised breathing exercises. Pre- and post-intervention analyzes to verify the effects of aerobic exercise on functional capacity were through the six-minute walk test (CT6) and pulmonary function by spirometry and manuovacuometry, with 20 and 40 sessions respectively. The Results: Regarding the intragroup comparison, we observed statistically significant differences in the EG for distance traveled, FEV1, FVC, PImax and Pemax after the intervention period of the participants with 40 sessions. Conclusion: There was an increase in functional capacity and pulmonary function after the pulmonary rehabilitation protocol. [ABSTRACT FROM AUTHOR]

Cystic Fibrosis (CF) is a genetic, congenital, autosomal recessive disease known as mucoviscidosis that affects many organs and systems of the body. Occurs due to a mutation of a protein known as Cystic Fibrosis Transmembrane Conductance Regulator or Cystic Fibrosis Conductance Regulator. This disease affects the white race and both sexes, being more prevalent in Caucasians. This study aimed to present the main methods of diagnosis and treatment of this pathology. The methodology used was the literature review, through scientific articles, official government websites, worldwide health organizations, books on the subject. There are several ways to reach the diagnosis of CF: antenatal, in the first months of life, neonatal screening and clinical manifestations. Currently, several types of treatments are used: pre-symptomatic, gene therapy treatment and drug treatment. However, non-pharmacological treatment is very important, respiratory physiotherapy is an example and is essential for the elimination of secretions to be performed correctly. It is concluded that patient follow-up by a multidisciplinary team is very important, being the pharmacist responsible for the Rational Use of Medicines, avoiding possible drug interactions and side effects related to the drugs used. [ABSTRACT FROM AUTHOR]

Copyright of Revista Ciência & Saúde Coletiva is the property of Associacao Brasileira de Pos-Graduacao em Saude Coletiva and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

This article reports a study that evaluated the relationship between self-efficacy and emergency visits in children and adolescents with chronic respiratory conditions. 36 participants aged between 7 and 18 years, mostly male, were included, with Cystic Fibrosis and Asthma being the most common diagnoses. The results showed a very weak negative correlation between self-efficacy and emergency visits in the last 12 months, indicating that there is no significant relationship between these two factors. Therefore, it is concluded that self-efficacy does not influence the number of emergency visits in these conditions. [Extracted from the article]

This article describes a study on the clinical and physiotherapeutic profile of chronic obstructive pulmonary disease in children (COPD) and adolescents secondary to infection. The research was based on the LILACS, PubMed, and PEDro literature, using conceptual and descriptive statistical analysis. The sample consisted of 18 participants, mostly male, with primary diagnoses of pneumonia, cystic fibrosis, bronchial asthma, and bronchiolitis. The results showed that prolonged hospitalization and treatment dependence are important characteristics of these lung diseases. [Extracted from the article]

This review study analyzes the factors that have influenced the change in the epidemiological profile of patients with cystic fibrosis (CF). The life expectancy of these patients has increased due to advances in disease management. The study highlights the importance of CFTR modulator therapies, early diagnosis and treatment, neonatal screening, studies on the Shwachman-Kulczycki Score, and prevention through genetic testing. The results emphasize the importance of further understanding the etiopathogenesis of CF, early diagnosis, and multidisciplinary disease management. [Extracted from the article]

This article discusses the relationship between self-efficacy and emergency visits in children and adolescents with chronic respiratory conditions. Self-efficacy is the individual's confidence in taking care of their own health. The study, conducted virtually, included 36 participants aged 7 to 18 years, mostly male, diagnosed with Cystic Fibrosis and Asthma. However, no correlation was found between self-efficacy and the number of emergency visits in the last 12 months. [Extracted from the article]

This article analyzes the reliability of the Brazilian version of the Pediatric Rating of Chronic Illness Selfefficacy (PRCISE), a self-efficacy instrument for children and adolescents with chronic respiratory diseases. The study was approved by the Research Ethics Committee of UFRN/FACISA and included 36 participants aged between 7 and 18 years, mostly male and diagnosed with Cystic Fibrosis and Asthma. The reliability of the Brazilian version of PRCISE was considered good, according to internal consistency and test-retest. [Extracted from the article]

ABSTRACT Positive Expiratory Pressure (PEP) improves lung function, however, PEP-induced changes are not fully established. The aim of this study was to assess the acute effects of different PEP levels on chest wall volumes and the breathing pattern in children with Cystic Fibrosis (CF). Anthropometric data, lung function values, and respiratory muscle strength were collected. Chest wall volumes were assessed by Optoelectronic plethysmography at rest and during the use of different PEP levels (10 and 20 cm H2O), randomly chosen. Eight subjects with CF (5M, 11.5±3.2 years, 32±9.5 kilograms) and seven control subjects (4M, 10.7±1.5 years, 38.2±7.8 kilograms) were recruited. The CF group showed significantly lower FEF values 25-75% (CF: 1.8±0.8 vs. CG: 2.3±0.6) and FEV1/FVC ratio (CF: 0.8±0.1 vs. CG: 1±0.1) compared with the control group (p

ABSTRACT Objective: To evaluate the association of body mass index (BMI) and albumin with pulmonary function in cystic fibrosis (CF) pediatric subjects. Methods: This is a cross-sectional study with clinically stable CF’s subjects. Clinical (pulmonary function) and nutritional evaluation (body mass index and albumin) were performed. Univariate analysis was performed using simple linear correlations. Regression analysis was performed using an exit level of p