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OBJECTIVE: To evaluate the accuracy of the modified Borg scale to estimate lung impairment, measured via FEV1 in children and adolescents with cystic fibrosis. METHODS: This cross-sectional prospective study was conducted with cystic fibrosis patients, 6-18 y old. With the modified Borg scale we evaluated their subjective perceptions of dyspnea before and after submaximal exercises, and its correlation with lung function (spirometry), 6-min walk test (6MWT), and nutritional status according to body mass index. RESULTS: Forty-one patients (mean +/- SD age range 11.1 +/- 4.1 y), were included in the study. The median (and interquartile range) modified Borg scale score after 6MWT was 2 (1-3). The mean percent-of-predicted FEV1 (FEV1%) was 97 +/- 32%. The Z score of the 6MWT distance (6MWTZ) for 61% of the patients was

Objective: To describe the findings of children with Robin Sequence (RS) who received sensory-motor-oral stimulation combined with early sucking during mandibular distraction osteogenesis (MDO), compared with children who did not receive the intervention., Design: A quasi-experimental study. Setting: A tertiary public hospital. Patients: Children with RS referred to MDO. A historical group from the same population but managed according to the institution's standard protocol (no sucking) served as a control group. Interventions: Sensory-motor-oral stimulation, including sucking, starting 24 h after MDO (intervention group). Main Outcome Measure: Our hypothesis is that sensory-motor-oral stimulation, including sucking during the DOM process, do not negatively affect surgical outcomes., Results: Twenty-nine children were included. Eight (72.7%) of the 11 patients in the intervention group and 13 (72.2%) of the 18 controls had MDO complications, with no significant difference between the groups ( p  = 1.000). The most common surgical outcome was antibiotic therapy for surgical site infection (76.2%). Six months after MDO, 22 (75.9%) children attained full oral feeding or associated with alternative feeding methods., Conclusion: The intervention group did not have higher complication rates, from a surgical point of view, than control group. The protocol adopted by some centers that contraindicates sucking during MDO should be revised to consider the benefits of such stimulation. Keywords: Pierre Robin Syndrome, deglutition, therapeutics, child development., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: The aim of the study is to evaluate major causes of upper airway obstruction in newborns receiving healthcare at our institution, their method of endoscopic assessment and the rate of complications related to these procedures., Materials and Methods: This is a case series study of patients from institutional neonatal intensive care unit (NICU) presenting signs of ventilatory dysfunction for whom an endoscopic airway assessment was warranted. Information of interest was collected from medical records according to a Clinical and Endoscopic Assessment Protocol created for the study. The protocol included clinical manifestations needing ENT evaluation, clinical signs of ventilatory dysfunction, comorbidities (pulmonary, cardiac, neurological, and gastrointestinal), examination method (airway endoscopy under general anesthesia or awake), exam complications, and final diagnosis., Results: One hundred sixty-nine newborn patients who underwent airway endoscopy (awake bedside flexible fiberoptic laryngoscopy (FFL) or direct laryngoscopy and bronchoscopy (DLB) in the surgical ward) were included. Thirty-nine patients (23.07%) underwent bedside FFL. For the remaining 130 who underwent DLB under general anesthesia, the median procedure time was 30 min (20-44). Only 9 (5.32%) patients presented complications: desaturation (4), laryngospasm without desaturation with spontaneous resolution (2), apnea with resolution after stimulation (1), seizures (1), nasal bleeding (1). The most frequent diagnoses found were glossoptosis, posterior laryngeal edema, and laryngomalacia., Conclusion: This retrospective case series describes the prevalence of different pathologies that cause upper airway obstruction in neonates. Airway endoscopy seems an effective and safe diagnostic tool in neonatal airway obstruction. Glossoptosis was the most prevalent cause of obstruction in our center., (© 2023. The Author(s), under exclusive licence to Springer Nature America, Inc.)

Dysphagia in Robin Sequence can be present in varying degrees, requiring multidisciplinary management and specific swallowing assessment by a specialist. Most studies published to date have evaluated only respiratory outcomes, and the available evidence on the improvement of swallowing is questionable. To conduct a systematic review and meta-analysis of studies evaluating swallowing in children with Robin Sequence before and after airway clearance procedures. The research question was developed based on the PICO strategy. The literature search was performed in electronic databases and gray literature. Studies were selected by 3 independent reviewers. The risk of bias and level of evidence of the studies were assessed. A proportion meta-analysis was performed to calculate the prevalence of dysphagia after airway clearance procedures. The search identified 4938 studies, 5 of which were included. All studies had limitations in terms of design and sample size. The prevalence of dysphagia after airway clearance was obtained by analyzing treatment subgroups: mandibular distraction osteogenesis, mandibular distraction osteogenesis + tracheostomy tube, and nasopharyngeal tube. Clinical and/or instrumental assessment was assessed by a swallowing specialist. The meta-analysis was precluded by the limitations of the studies, especially regarding sample size, which affected the accuracy of the findings. Dysphagia remained unresolved in 55% of children (95% CI 1-99%). The methodological quality of the studies indicated a high risk of bias and very low level of evidence. It was not possible to confirm that airway clearance techniques used in Robin Sequence improve dysphagia., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

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.

Objectives: To evaluate the bacterial microbiome found in tracheostomy cannulas of a group of children diagnosed with glossoptosis secondary to Robin Sequence (RS), and its clinical implications., Methods: Pediatric patients were enrolled in the study at the time of the cannula change in the hospital. During this procedure, the removed cannula was collected and stored for amplicon sequencing of 16s rRNA. DNA extraction was performed using DNeasy PowerBiofilm Kit (QIAGEN® ‒ Cat nº 24000-50) while sequencing was performed with the S5 (Ion S5™ System, Thermo Fisher Scientific), following Brazilian Microbiome Project (BMP) protocol., Results: All 12 patients included in the study were using tracheostomy uncuffed cannulas of the same brand, had tracheostomy performed for over 1-year and had used the removed cannula for approximately 3-months. Most abundant genera found were Aggregatibacter, Pseudomonas, Haemophilus, Neisseria, Staphylococcus, Fusobacterium, Moraxella, Streptococcus, Alloiococcus, and Capnocytophaga. Individual microbiome of each individual was highly variable, not correlating to any particular clinical characteristic., Conclusion: The microbiome of tracheostomy cannulas is highly variable, even among patients with similar clinical characteristics, making it challenging to determine a standard for normality., (Copyright © 2022 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier España, S.L.U. All rights reserved.)

Aims: To verify the prevalence of Potentially pathogenic bacteria (PPB) and their antimicrobial resistance profile in tracheal aspirates of children with tracheostomy and compare it to clinical data., Methods: A cross-sectional study was conducted in patients aged 0-18 years who all underwent tracheostomy cannula change (TCC) performed by the Otolaryngology Unit at Hospital de Clínicas de Porto Alegre, Brazil, between October, 2017 and December, 2018. Patients were submitted, at the time of TCC, to a tracheal aspirate through the tracheostomy and secretion was sent to microbiological analysis and antimicrobial susceptibility testing. Clinical data were evaluated through available patients' electronic medical records., Results: Forty-four patients had their tracheostomy aspirate cultured and all but one presented PPB growth (97.7%). Median age was 3 years-old. Pseudomonas aeruginosa was the most prevalent bacteria (56.9%) and it was resistant to gentamycin, amikacin and cefepime in 36%, 28% and 12% of the culture tests, respectively. P. aeruginosa resistance to gentamycin and to cefepime suggested an association with the number of antibiotic classes used in the 12 months before enrollment (both p=0.04) and with 2 or more hospital admissions in the same period (p=0.03 and p=0.02, respectively). Staphylococcus aureus was isolated in 9.1% and there was no MRSA., Conclusion: It was found a 97.7% prevalence of PPB in the cultured aspirates; the most prevalent bacterium was P. aeruginosa and there was no MRSA identification. Data suggest an association between P. aeruginosa antimicrobial resistance with previous use of antibiotic therapy., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Introduction: Inadequate drooling can cause serious clinical, functional and social problems. Validated questionnaires to evaluate drooling impact on quality of life are lacking in Brazilian Portuguese., Objectives: To translate and validate the drooling impact scale to Brazilian Portuguese., Methods: The drooling impact scale was translated to Brazilian Portuguese and back- translated to English to assess potential conceptual differences. Brazilian Portuguese version of drooling impact scale was applied to a 40 patients' sample of sialorrhea presenting pediatric patients (up to 20 years of age). Chronbach's alpha, exploratory factorial analysis and confirmatory factorial analysis were then proceeded with data collected., Results: The mean drooling impact scale value for the whole population was 51.77 (SD = 16.13). The internal consistency obtained with Cronbach's alpha indicated a value of 0.72 for the entire sample. The Bartlett's test of sphericity was significant (p <  0.0001), confirming correlation among variables tested. Kaiser-Meyer-Olkin measure of sampling adequacy revealed a value of 0.72, indicating that the correlation matrix was reasonably suitable for factor analysis. Regarding exploratory factorial analysis, parallel analysis suggested a two-factor solution that was used for confirmatory factorial analysis. The first factor was responsible for 33.78% of the variance with an Eigenvalue of 3.38. The second factor explained 16.1% of the variance with an Eigenvalue of 1.61. At confirmatory factorial analysis, the two-factor model showed consistently better adjustments parameters than the one-factor model., Conclusion: The drooling impact scale has been successfully translated to Brazilian Portuguese language, showing adequate internal validity. Validation of this instrument allows physicians and other personnel involved in the care of these patients to perform a better management of patients experiencing drooling. With this tool, we are now able to guide routines and provide guidelines both before and after the different kinds of treatments in order to improve the general well-being of the patient and his family., (Copyright © 2020 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.)

Background: We present the first case to our knowledge of a spontaneous twin pregnancy in a 16-year-old Caucasian patient with cystic fibrosis and systemic lupus erythematosus. Cystic fibrosis is one of the most common autosomal recessive genetic disorders and primarily affects the respiratory and digestive systems. Systemic lupus erythematosus is a chronic inflammatory disease of unknown cause that affects nearly every organ. Patients with cystic fibrosis or systemic lupus erythematosus are progressively having longer life expectancy and better quality of life, which has led a greater number of female patients reporting the desire to become mothers., Case Presentation: We present a case of a Caucasian 16-year-old pregnant with twins being treated for both cystic fibrosis and systemic lupus erythematosus. She has two CFTR mutations: p.F508del and 1812&#95;1G>A. In the second trimester, she was admitted for possible preterm labor, which was successfully stopped. The patient's nutritional status worsened, and she had a pulmonary exacerbation as well as a flare of systemic lupus erythematosus. At the 28th gestational week, she presented with a massive hemoptysis episode. The cesarean delivery had no complications, and there were no serious immediate postpartum complications., Discussion and Conclusions: While adolescent pregnancies in and of themselves are considered high risk for both the young mothers and their children, they are further complicated when the mother has two chronic diseases and a twin pregnancy. We achieved positive results using a multidisciplinary approach; however, the risks involved were so high that major efforts are to be taken by our medical community to prevent unplanned pregnancies in all patients with cystic fibrosis, especially when a serious comorbidity like the one in this case is present., (© 2022. The Author(s).)

Objective: Patients with cystic fibrosis (CF) have a high incidence of pubertal and growth delay. In girls with CF, pubertal delay has an important psychological impact. Still, only a few studies have explored the occurrence of pubertal delay in girls with CF. The aims of this study were to compare the pubertal development of girls with CF compared with healthy controls regarding Tanner staging and pelvic ultrasound and, in girls with CF, correlate the findings with those of spirometry, body mass index, Shwachman-Kulczycki score (SKS), and genotyping., Methods: This was a cross-sectional, case-control study including 35 girls with CF aged 6-17 years and following up at the Pediatric Pulmonology Outpatient Clinic of a tertiary hospital. These patients were compared with 59 healthy controls who had undergone pelvic ultrasound as part of another study conducted by the same group. Girls with CF were consecutively enrolled in the study during their annual routine check-up visit. Data collected in the CF group included spirometry and anthropometric results, SKS values, bone age, occurrence of current cystic fibrosis-related diabetes (CFRD) and Pseudomonas aeruginosa colonization, history of meconium ileus, genotype, ultrasound parameters, and Tanner stage., Results: Pelvic ultrasound findings and Tanner stage reflected less pubertal development in girls with CF compared with healthy controls. Pubertal stage in patients with CF who presented CFRD (3.17 ± 1.16), had chronic colonization by Pseudomonas aeruginosa (3.10 ± 1.10), or were homozygous for the F508del mutation (1.91 ± 1.30) was more delayed than in controls (3.41 ± 1.41). Tanner stage correlated with age at menarche, bone age, and anthropometric and ultrasound data., Conclusion: Girls with CF presented a delay in pubertal development evaluated by Tanner stage and ultrasound parameters, which was more evident in the presence of comorbidities.

Background: Elevated extracellular DNA levels are found in the sputum of patients with cystic fibrosis (CF). However, studies investigating the association of extracellular DNA with CF severity are scarce., Objective: To evaluate the association of extracellular DNA levels with pulmonary function, antibiotic use, and hospitalization in CF patients., Methods: This cross-sectional study included CF patients aged ≥5 years who were clinically stable and produced spontaneously expectorated sputum. Extracellular DNA in sputum was quantified, and extracellular DNA networks were seen with immunofluorescence microscopy. Also, cell death profile was assessed. Data on pulmonary function, airway colonization, antibiotic use, and hospitalization in the previous year were collected. Patients were divided into two groups based on median DNA level., Results: Thirty-three patients were included. Their mean age was 16.3 ± 6.2 years, mean forced expiratory volume in the first second (FEV 1 ) was 67.0 ± 26.7 (% of the predicted), and mean DNA level was 241.9 ± 147.2 μg/mL. There were significant correlations of DNA level with FEV 1 (r = -0.60; p < 0.001) and forced vital capacity (r = -0.59; p < 0.001). Moreover, patients with higher DNA level (>243.0 μg/mL) had lower FEV 1 (52.1 ± 27.8% vs. 81.1 ± 16.2%; p = 0.001) and required more hospitalizations (68.8% vs. 35.3%; p = 0.05). Additional findings were the presence of extracellular DNA networks and low rates of necrosis and apoptosis., Conclusion: Elevated extracellular DNA levels in CF sputum are associated with reduced pulmonary function and increased hospitalizations., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: Antioxidant micronutrients and essential fatty acids supplementation intake appears to have a protective effect in some diseases such as cardiovascular disease, cancer, and asthma., Objective: The aim of this study was to perform a systematic review to evaluate the effects of these nutrients on nutritional and clinical outcomes of patients with cystic fibrosis (CF)., Methods: This is a systematic review of randomized clinical trials (RCTs) in CF. MEDLINE (via PubMed), Embase, and Scopus databases were searched for RCTs published from 1948 through February 2019. Two investigators independently reviewed the titles and abstracts and then extracted the data from the included studies using a standardized predesigned form. Two reviewers independently performed the quality assessment of the RCTs according to the Cochrane risk of bias tools., Results: A total of 4,792 studies were identified, and 23 were eligible (8 antioxidant micronutrient and 15 essential fatty acids). The interventions found were beta-carotene, zinc, magnesium, multivitamin, docosahexaenoic acid (DHA), eicosapentaenoic acid (EPA), linoleic acid and lipid matrix with choline supplementation. A significant improvement was observed in: (a) pulmonary function with magnesium (n=1) and essential fatty acids (n=2) supplementation; (b) less pulmonary exacerbations with beta-carotene (n=1), zinc (n=1), antioxidant-enriched multivitamin (n=1) and essential fatty acids (n=2) supplementation. One study with antioxidant-enriched multivitamin and four studies with EPA/DHA supplementation reported significant reductions in inflammatory markers. Nutritional status was not modified by antioxidants supplementation in any of the studies, while in five studies there was an improvement with fatty acids supplementation. The risk of bias of the majority of the parallel studies was high., Conclusions: The benefits of antioxidants or DHA/EPA supplementation for CF, although observed in some studies, are not consistent enough to recommend routine use of these supplements. The mechanisms of action of these nutrients, dose levels and timing should be further explored in future studies., Competing Interests: Author Contributions M. I. S. dos S. Simon and G. C. Forte wrote the first draft with contributions from F. M. Silva and P. J. C. Marostica. M. Feldmann and R. Dalle Molle reviewed the titles and abstracts, R. Dalle Molle and T. W. Rodrigues extracted data from the included studies, M. I. S. dos S. Simon and R. Dalle Molle performed the quality assessment. All authors reviewed and commented on subsequent versions of the manuscript., (Copyright © 2020 Academy of Nutrition and Dietetics. Published by Elsevier Inc. All rights reserved.)

Objective: To translate and culturally adapt the Mediterranean Diet Quality Index in Children and Adolescent (KIDMED) for the Brazilian population., Methods: The processes of translation and cultural adaptation followed internationally standardized methodological norms. We used the intraclass correlation coefficient and the Bland-Altman dispersion analysis to assess the reproducibility and calculated the internal consistency with Cronbach's alpha coefficient., Results: A total of 102 children and adolescents participated in the cross-cultural adaptation, of whom 58 (56.9%) were females, with a mean age of 9.8±4.9 years. The mean overall scores of adherence to the Mediterranean diet in the test and retest were similar (8.00 and 3.80 versus 8.01 and 3.84) for children and adolescents, respectively. The intraclass correlation coefficient for children and adolescents was 0.893 and 0.998, respectively. The internal consistency was 0.72. The Bland-Altman plot analysis showed good agreement between the final scores of the test and retest questionnaires, with no statistically significant difference., Conclusions: The KIDMED questionnaire was translated into Brazilian Portuguese and culturally adapted, presenting high reproducibility. This questionnaire can, therefore, be included and used in Brazilian studies that aim at evaluating the adherence to the Mediterranean diet among children and adolescents.

Objective: To evaluate height, sexual maturation, and the difference between final and expected height in girls with juvenile idiopathic arthritis and no glucocorticoid treatment for at least six months, as compared to a group of healthy girls., Methods: This cross-sectional study involved 44 girls with juvenile idiopathic arthritis, diagnosed according to the International League of Associations for Rheumatology criteria, and 59 healthy controls aged between 8 and 18 (incomplete) years with no comorbid chronic diseases. Demographic data were collected from all participants, and disease and treatment variables were compiled for the patient group. Anthropometric measurements were converted into Z-scores based on World Health Organization standards. Sexual maturation was classified according to Tanner stages., Results: Body mass index and height Z-scores were lower in girls with juvenile idiopathic arthritis as compared to control participants. These values differed significantly in Tanner stage II. Three (6.8%) girls with juvenile idiopathic arthritis had height-for-age Z-scores <-2 (short stature). Girls with polyarticular juvenile idiopathic arthritis and higher cumulative glucocorticoid doses were significantly more likely to present with short stature. The percentage of prepubertal girls in the juvenile idiopathic arthritis group was significantly higher than that observed in the control group, (p=0.012). Age of menarche, adult height, and the difference between actual and expected height did not differ between groups., Conclusion: These findings suggest that even six months after the suspension of glucocorticoid treatment, children with polyarticular/systemic juvenile idiopathic arthritis subtypes are still susceptible to low height and delayed puberty., (Copyright © 2018. Published by Elsevier Editora Ltda.)

Objective: To assess the accuracy of stridor in comparison to endoscopic examination for diagnosis of pediatric post-intubation subglottic stenosis., Method: Children who required endotracheal intubation for >24h were included in this prospective cohort study. Children were monitored daily and underwent flexible fiberoptic laryngoscopy after extubation. Those with moderate-to-severe abnormalities underwent another examination 7-10 days later. If lesions persisted or symptoms developed, laryngoscopy under general anesthesia was performed. Patients were assessed daily for stridor after extubation., Results: A total of 187 children were included. The incidence of post-extubation stridor was 44.38%. Stridor had a sensitivity of 77.78% (95% confidence interval [95% CI]: 51.9-92.6) and specificity of 59.18% (95% CI: 51.3-66.6) in detecting subglottic stenosis. The positive predictive value was 16.87% (95% CI: 9.8-27.1), and the negative predictive value was 96.15% (95% CI: 89.9-98.8). Stridor persisting longer than 72h or starting more than 72h post-extubation had a sensitivity of 66.67% (95% CI: 41.2-85.6), specificity of 89.1% (95% CI: 83.1-93.2), positive predictive value of 40.0% (95% CI: 23.2-59.3), and negative predictive value of 96.07% (95% CI: 91.3-98.4). The area under the receiver operating characteristic (ROC) curve was 0.78 (95% CI: 0.65-0.91)., Conclusions: Absence of stridor was appropriate to rule out post-intubation subglottic stenosis. The specificity of this criterion improved when stridor persisted longer than 72h or started more than 72h post-extubation. Thus, endoscopy under general anesthesia can be used to confirm subglottic stenosis only in patients who develop or persist with stridor for more than 72h following extubation., (Copyright © 2018. Published by Elsevier Editora Ltda.)

Objective: Home nebulizers are routinely used in the treatment of patients with cystic fibrosis (CF). This study aims to evaluate the contamination of nebulizers used for CF patients, that are chronically colonized by Pseudomonas aeruginosa, and the association of nebulizer contamination with cleaning, decontamination and drying practices., Methods: A cross-sectional, observational, multicenter study was conducted in seven CF reference centers in Brazil to obtain data from medical records, structured interviews with patients/caregivers were performed, and nebulizer's parts (interface and cup) were collected for microbiological culture., Results: overall, 77 CF patients were included. The frequency of nebulizer contamination was 71.6%. Candida spp. (52.9%), Stenotrophomonas maltophilia (11.9%), non-mucoid P. aeruginosa (4.8%), Staphylococcus aureus (4.8%) and Burkholderia cepacia complex (2.4%) were the most common isolated pathogens. The frequency of nebulizers' hygiene was 97.4%, and 70.3% of patients reported cleaning, disinfection and drying the nebulizers. The use of tap water in cleaning method and outdoor drying of the parts significantly increased (9.10 times) the chance of nebulizers' contamination., Conclusion: Despite the high frequency hygiene of the nebulizers reported, the cleaning and disinfection methods used were often inadequate. A significant proportion of nebulizers was contaminated with potentially pathogenic microorganisms for CF patients. These findings support the need to include patients/caregivers in educational programs and / or new strategies for delivering inhaled antibiotics.

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<0.05., Results: Seventy-eight CF's subjects (mean age 12.8±3.8 years) with mean albumin 4.2±0.4 mg/dL, predicted forced expiratory volume in 1 second (FEV1%) 80.8±22.6 and BMI median percentile 51.2 (1.3-97.7). In the multiple regression models, albumin, age and BMI percentile were associated with pulmonary function. Subjects with lower than 25 BMI percentile had 12.2% lower FEV1%. An albumin increase of 0.1 mg was associated with 2.7% increase in predicted FEV1%, and one year increase in age was associated with reduction in 1.2% of predicted FEV1%., Conclusions: BMI percentile, albumin and age were independently associated with predicted FEV1% in a tertiary referral hospital.

Objective: to assess the progression of pediatric cystic fibrosis (CF) patients' nutritional status during the first 12 months after diagnosis and to establish its association with neonatal screening and clinical variables. Patients were recruited from two reference centers in Southern Brazil., Methods: Retrospective cohort study was carried out with all the patients diagnosed between 2009 and 2014. Anthropometric, clinic and neonatal screening were collected from medical files. Analysis of anthropometric markers over time was performed by generalized estimating equations. A multivariate regression analysis model to predict the Δ percentile body mass index (BMI) (BMI percentile difference between one year after the treatment and BMI percentile at diagnosis) was done., Results: Forty-seven patients were included in the study. Analysis of nutritional data over the period between six months and one year after diagnosis showed significant improvement of BMI, weight/age and weight/height percentiles and Z scores. The neonatal screening was associated with a significant increase of 31.2 points in ΔBMI percentile at the one-year evaluation (p<0.05). On the other hand, a one-point increase of initial BMI percentile was associated with a reduction of 0.6 points in ΔBMI percentile., Conclusion: This study demonstrated the role of neonatal screening in the nutritional status of patients diagnosed with CF in the first year after diagnosis. Early diagnosis can significantly contribute to the achievement of appropriate anthropometric indicators and important nutritional recovery of CF patients.

Objective: To compare muscle thickness and subcutaneous fat in cystic fibrosis (CF) patients and healthy controls using ultrasonography (US), and to correlate US findings with nutritional, clinical and functional variables., Methods: Patients aged 6 to 18 years old with a diagnosis of CF and healthy controls were included. Participants underwent anthropometric measurements, an ultrasonographic evaluation of muscle thickness and subcutaneous fat in the triceps, quadriceps, and gastrocnemius regions, and skinfold thickness measurements. Body fat percentage was estimated using skinfold measurement. Subjects with CF also underwent a pulmonary function assessment using spirometry., Results: We studied 39 CF patients and 45 controls. Alower body mass index was observed in CF patients (p=0.011). Body composition and muscle thickness were similar between the groups. Only calf (p=0.023) circumference and femur diameter (p<0.001) were lower in CF patients. Although there were no significant between-group differences in the comparison of US measurements of subcutaneous fat, CF patients exhibited decreased skinfold thickness in the triceps (p=0.031) and quadriceps (p=0.019). Moreover, there were weak and moderate correlations of US quadricep thickness with forced vital capacity (FVC) and lean mass, respectively. Moderate correlations of the triceps, quadriceps and gastrocnemius between US subcutaneous fat and skinfold measurements were found., Conclusions: Patients with CF presented a reduction in subcutaneous fat content. Muscle thickness correlated with FVC and nutritional parameters. In addition, US findings correlated positively with skinfold measurements.

Purpose: To assess the impact of balloon laryngoplasty on clinical and surgical outcomes in pediatric patients with acute subglottic stenosis., Methods: Two case series were included and compared. The first group included patients treated initially either with tracheostomy (if severe symptoms) or with close follow-up (if mild symptoms). Those children underwent re-evaluation and specific treatment of their stenosis with laser incisions or open surgeries some weeks later. The other group included children treated initially with balloon laryngoplasty, reflecting a shift in surgical practice after 2009. Data as success of the procedure, mean hospital stay, mean pediatric intensive care unit (PICU) stay, post-procedure fever, need of antibiotics, procedure-related complications, and deaths were assessed and compared between both cohorts., Results: The sample comprised 38 pediatric patients aged 0-5 years. Fifteen children were treated before 2009, of who 10 (66.7%) required tracheostomy soon after the diagnosis. Ultimately, 13 (86.6%) underwent laryngotracheal reconstruction. Twenty-three children were treated after 2009 and the success rate in these patients treated primarily with balloon laryngoplasty was 82.6%. Of these, only 3 (13%) required tracheostomy and 1 (4.3%) required further open laryngotracheal reconstruction. Patients treated by balloon laryngoplasty underwent fewer procedures under general anesthesia and had a lower burden of treatment-related morbidity, as denoted by shorter PICU stay, less antibiotic use, earlier postoperative resumption of oral feeding, and a lower incidence of postoperative complications and fever., Conclusion: When used for management of acute laryngeal stenosis, balloon laryngoplasty is associated with a high success rate, presenting lower morbidity than open surgery.

Objectives: To evaluate the association of polysomnographic parameters with clinical symptom severity in Robin sequence (RS) patients., Methods: All patients diagnosed as presenting with RS at Hospital de Clínicas de Porto Alegre from October 2012 to June 2016 were enrolled. They were classified as isolated RS, RS-plus, and syndromic RS. Polysomnography (PSG) was performed, except for those patients in need of respiratory support. Symptom severity was evaluated as defined by the Cole et al., Classification: Ordinal OR (for the chance of increase in one grade on the clinical severity scale) and R 2 (determination coefficient from ordinal logistic regression) were computed from data analysis., Results: A total of 80 participants were enrolled in the study. Fifty-five of these were able to undergo polysomnography. Worsening of the studied PSG parameters was associated with increase in clinical severity grading, as follows: desaturation index (OR 1.27; 95% CI; 1.07-1.51; R 2  = 19.8%; p = 0.006); apnea/hypopnea Index (OR 1.13; 95% CI; 1.01-1.26; R 2  = 12.5%; p = 0.02); sleep mean oxygen saturation (OR 0.16; 95% CI; 0.05-0.52; R 2  = 22.6%; p = 0.002); oxygen saturation nadir (OR 0.73; 95% CI; 0.56-0.96; R 2  = 10.0%; p = 0.02); percentage of time with oxygen saturation <90% (OR 9.49; 95% CI; 1.63-55.31, R 2  = 37.6%; p = 0.012); and percentage of time presenting with obstruction (OR 2.5; 95% CI; 1.31-4.76; R 2  = 25.1%; p = 0.006)., Conclusions: Polysomnography parameters were associated with severity of clinical manifestations in patients with RS. Oxyhemoglobin saturation-based parameters had surprisingly significant R 2 values. Therefore, those parameters, which have traditionally been undervalued in other clinical settings, should also be assessed in the polysomnographic evaluation of RS patients., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objectives/hypothesis: To assess the performance of endoscopic grading systems of glossoptosis in identifying severe clinical manifestations in children with Robin sequence (RS)., Study Design: Nested cohort cross-sectional study., Methods: All RS patients diagnosed at Hospital Clinics of Porto Alegre from October 2012 to June 2016 were enrolled in this cohort. Patients underwent sleep endoscopy and were classified according to Yellon (Y) and de Sousa et al. (S) scales. Symptom severity evaluation was performed as defined by Cole et al. The outcome of interest was Cole's clinical classification grade 3., Results: Eighty patients were eligible for analysis. Sensitivity (Y: 56.2%, S: 28.1%, P < .001) and specificity (Y: 85.4%, S: 93.8%, P = .038) in identifying severe clinical symptoms patients (i.e., Cole grade 3) were statistically different between Y and S classifications. A low but significant overall correlation was observed for both Y (rho = 0.372, P < .001) and S (rho = 0.439, P < .001) classifications when compared with Cole classification. Diagnostic odds ratio (DOR) for Y (DOR: 7.53, 95% confidence interval [CI]: 4.15-10.90) and S (DOR: 5.87, 95% CI: 1.86-9.87) were equivalent (P = .92). Also, receiver operating characteristic curves area under the curve were not significantly different between them. The positive likelihood ratio was 3.86 (95% CI: 1.82-8.16) and 4.50 (95% CI: 1.32-15.36) for Y and S, respectively., Conclusions: Y and S grading systems showed a low sensitivity and moderate to high specificity in detecting patients with severe clinical manifestations. Correlation between Y/S and Cole et al. grading were also considered low. Development of a more discriminative anatomic grading system is still needed for this specific disorder., Level of Evidence: 2b. Laryngoscope, 128:502-508, 2018., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

The objective of this study is to determine the incidence of post-extubation acute laryngeal lesions in a pediatric intensive care unit (PICU) and potential risk factors. Children, aged 28 days to 5 years, admitted to the PICU who required endotracheal intubation for at least 24 h were enrolled. Exclusion criteria were a previous intubation, history of laryngeal disease, current or past tracheostomy, the presence of craniofacial malformations and patients considered on palliative care. All patients underwent flexible fiber-optic laryngoscopy (FFL) not later than 8 h after extubation. A blinded researcher identified and classified laryngeal lesions based on recorded media. 231 children were enrolled between November 2005 and December 2015. At FFL examination, 102 children (44.15%) presented moderate to severe laryngeal lesions. On a multivariable analysis, we found that for each additional day with repositioning of the endotracheal tube, there was an increase of 7.3% (RR 95% CI 1.012-1.137; P = 0.018) on the baseline risk of developing moderate to severe acute laryngeal lesions. Furthermore, for each additional dose of sedation per day of intubation, there was also an increase of 3.5% on the same baseline risk (RR 95% CI 1.001-1.070; P = 0.041). The amount of tube repositioning episodes and the need for extra doses of sedation (as a proxy for possible agitation) were found to be associated with acute laryngeal lesions. Adequate sedation and minimized tube repositioning should be pursued to possibly prevent the development of post-extubation airway compromise.

Objective: To analyze the level of sedation in intubated children as a risk factor for the development of subglottic stenosis., Methods: All patients between 30 days and 5 years of age who required endotracheal intubation in the pediatric intensive care unit between 2013 and 2014 were included in this prospective study. They were monitored daily and COMFORT-B scores were obtained. Flexible fiber-optic laryngoscopy was performed within eight hours of extubation, and repeated seven to ten days later if the first examination showed moderate to severe laryngeal injuries. If these lesions persisted and/or if the child developed symptoms in the follow-up period, microlaryngoscopy under general anesthesia was performed to evaluate for subglottic stenosis., Results: The study included 36 children. Incidence of subglottic stenosis was 11.1%. Children with subglottic stenosis had a higher percentage of COMFORT-B scores between 23 and 30 (undersedated) than those who did not develop subglottic stenosis (15.8% vs. 3.65%, p=0.004)., Conclusion: Children who developed subglottic stenosis were less sedated than children who did not develop subglottic stenosis., (Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Objective: To evaluate the association of 6-minute walk test (6MWT) and other variables (anthropometry, chronic Pseudomonas aeroginosa colonization, pulmonary function, and respiratory muscle strength) with the risk of hospitalization for pulmonary exacerbation in children and adolescents with cystic fibrosis (CF)., Study Design: Cohort study that included patients with CF aged 6-18 years. All participants underwent spirometry, manovacuometry, and 6MWT during the 5-year follow-up. Anthropometric and clinical data were collected and the time to first hospitalization, total days of hospitalization, and antibiotic use during follow-up was recorded., Results: A total of 26 patients with CF, mean age 10.2 ± 2.8 years, were included. The group had mild impairment of lung function with a significant decline in forced expiratory volume in 1 second (P = .019) over the 5 years. Respiratory muscle strength and 6MWT proved to be preserved, although maximum inspiratory pressure increased (P < .001) and maximum expiratory pressure and 6-minute walk distance (6MWD) remained stable. There were inverse associations of 6MWD in meters (r = -0.813, P < .001) and z score (r = -0.417, P = .015) with total days of hospitalization. Moreover, there was a reduction in the risk of a first hospitalization (Cox HR 0.32; P = .037) in patients with a greater 6MWD., Conclusions: We found an association between the 6MWT and the risk of hospitalization in children and adolescents with CF. Furthermore, functional capacity apparently does not follow the expected decline in pulmonary function over time, whereas inspiratory muscle strength increases with disease progression., (Copyright © 2016 Elsevier Inc. All rights reserved.)