Sensory-Motor-Oral Stimulation Combined with Early Sucking During the Mandibular Distraction Osteogenesis Process in Children with Robin Sequence.

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.

Therapeutic Management with Airway Clearance in Children with Robin Sequence and Association with Swallowing Outcomes: A Systematic Review and Meta-analysis.

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.

Chloride Conductance, Nasal Potential Difference and Cystic Fibrosis Pathophysiology.

PURPOSE: Cystic fibrosis (CF) is a multisystem genetic disease caused by dysfunction of the epithelial anionic channel Cystic Fibrosis Transmembrane conductance Regulator (CFTR). Decreased mucociliary clearance because of thickened mucus is part of the pulmonary disease pathophysiology. It is controversial if the thickened airway surface liquid (ASL) is caused by the deficient chloride secretion and excessive sodium (through ENaC) and water hyperabsorption from the periciliar fluid or by the lack of bicarbonate secretion with relative acidification of the ASL. Correlations between the magnitude of in vivo chloride conductance with phenotypic characteristics and CF genotype can help to elucidate these mechanisms and direct to new treatments. METHODS: Nasal potential difference was measured in 28 CF patients (age from 0.3 to 28 year) and correlated with pulmonary function, pancreatic phenotype, pulmonary colonization and genotype severity. RESULTS: The CFTR-chloride conductance was better in older patients (r = 0.40; P = 0.03), in patients with better pulmonary function (r = 0.48; P = 0.01), and was associated with genotype severity. Higher chloride diffusion in the presence of a favorable chemical gradient was associated with Pseudomonas aeruginosa negativity (P < 0.05). More negative NPDmax was associated with pancreatic insufficiency (P < 0.01) as well with genotype severity, but not with the pulmonary function. CONCLUSIONS: The anion permeability through CFTR, mainly chloride, but bicarbonate as well, is the most critical factor in CF airway pathophysiology. Treatments primarily directed to correct CFTR function and/or airway acidity are clearly a priority.

Impact of balloon laryngoplasty on management of acute subglottic stenosis.

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.

Association of endotracheal tube repositioning and acute laryngeal lesions during mechanical ventilation in children.

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.

Clinical outcomes before and after videofluoroscopic swallow study in children 24 months of age or younger.

OBJECTIVE: To evaluate the combined impact of videofluoroscopic swallow study (VFSS) and therapeutic feeding and swallowing interventions on clinical outcomes in children with oropharyngeal dysphagia (OPD). METHODS: This was an uncontrolled longitudinal analytical study in which OPD patients were evaluated before and after VFSS. Children ≤ 24 months of age diagnosed with OPD in a clinical setting and undergoing VFSS for investigation and management of OPD were included in the study. The study participants received therapeutic feeding and swallowing interventions after having undergone VFSS, being followed at an outpatient clinic for pediatric dysphagia in order to monitor feeding and swallowing difficulties. Respiratory and feeding outcomes were compared before and after VFSS. RESULTS: Penetration/aspiration events were observed in 61% of the VFSSs (n = 72), and therapeutic feeding and swallowing interventions were recommended for 97% of the study participants. After the VFSS, there was a reduction in the odds of receiving antibiotic therapy (OR = 0.007) and in the duration of antibiotic therapy (p = 0.014), as well as in the odds of being admitted to hospital (p = 0.024) and in the length of hospital stay (p = 0.025). A combination of oral and enteral feeding became more common than oral or enteral feeding alone (p = 0.002). CONCLUSIONS: A high proportion of participants exhibited penetration/aspiration on VFSS. Therapeutic feeding and swallowing interventions following a VFSS appear to be associated with reduced respiratory morbidity in this population.

High microbiome variability in pediatric tracheostomy cannulas in patients with similar clinical characteristics.

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.

Endoscopic evaluation of neonates with signs of upper airway obstruction in the neonatal unit of a tertiary hospital.

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.

Brazilian guidelines for the pharmacological treatment of the pulmonary symptoms of cystic fibrosis. Official document of the Sociedade Brasileira de Pneumologia e Tisiologia (SBPT, Brazilian Thoracic Association).

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.

High-risk twin pregnancy: case report of an adolescent patient with cystic fibrosis and systemic lupus erythematosus.

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_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.

Translation and validation of the drooling impact scale questionnaire into Brazilian Portuguese.

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.

Ultrasound findings of pubertal development in girls with cystic fibrosis and their association with clinical outcomes and Tanner staging.

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.

Extensive CFTR sequencing through NGS in Brazilian individuals with cystic fibrosis: unravelling regional discrepancies in the country.

BACKGROUND: The Brazilian population has a tri-hybrid composition with a high degree of ethnic admixture. We hypothesized that Brazilian individuals with CF from different Brazilian regions have a specific distribution of CFTR variants. METHODS: Individuals with CF with data available in the Patient Registry and without an established genotype were submitted to CFTR sequencing by Next Generation Sequencing (NGS) methodology, and results were anonymously incorporated to the Registry Database. Genotyping results were expressed as 'positive', 'inconclusive' or 'negative'. Logistic regression models were performed to investigate the association between demographic/clinical variables and genotyping results. Mediation analysis was conducted to estimate direct and indirect effects of Brazilian region on a binary positive genotyping response. RESULTS: In October 2017, data from 4,654 individuals with CF were available, and 3,104(66.7%) of them had a genotyping result. A total of 236 variants (114 new variants) were identified, with F508del identified in 46% of the alleles tested. Genotyping revealed 2,002(64.5%) individuals positive, 757(24.4%) inconclusive and 345(11.1%) negative. Distribution of genotype categories was markedly different across Brazilian Regions, with greater proportions of negative individuals in the North (45%) and Northeast (26%) regions. Newborn screening (CF-NBS) and age at diagnosis were identified as mediators of the effect of Brazilian region on a positive genotyping result. CONCLUSIONS: This large initiative of CFTR genotyping showed significant regional discrepancies in Brazil, probably related to socio-economic conditions, lack of adequate CF-NBS and poor access to reliable sweat testing. These results may be useful to indicate Regions where CF care demands more attention.

TRANSLATION AND CULTURAL ADAPTATION OF THE MEDITERRANEAN DIET QUALITY INDEX IN CHILDREN AND ADOLESCENTS.

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.

Extracellular DNA in sputum is associated with pulmonary function and hospitalization in patients with cystic fibrosis.

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 (FEV1) 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 FEV1 (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 FEV1 (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.

Antioxidant Micronutrients and Essential Fatty Acids Supplementation on Cystic Fibrosis Outcomes: A Systematic Review.

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.

Accuracy of stridor-based diagnosis of post-intubation subglottic stenosis in pediatric patients.

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.

Infant sleep position: a randomized clinical trial of an educational intervention in the maternity ward in Porto Alegre, Brazil.

BACKGROUND: Few studies in Brazil have been published about sudden infant death syndrome (SIDS), and none has addressed the mother's orientation about placing the infant to sleep in the supine position. The aim of this study was to evaluate the effect on mothers of an individual educational intervention in the maternity ward about infant sleep position. METHODS: A randomized clinical trial was conducted with a study sample of 228 mother-infant pairs assigned to an intervention or a control group. The intervention consisted of an individual orientation session at the maternity ward, at which folders and an oral explanation were given to mothers at discharge about the importance of the supine position as a preventive measure for SIDS. The outcome was the sleeping position at 3 months of age assessed during a home visit. The variables with p< 0.2 at a bivariate analysis were included in a logistic regression model. RESULTS: Among mothers in the intervention group, 42.9 percent put their infants to sleep in a supine position at the 3-month visit, compared with 24 percent of mothers in the control group (p = 0.009). In a multivariate analysis, the intervention at the hospital was the only variable that influenced maternal practices with respect to infant sleep positioning (OR 2.22; 95% CI 1.17-4.19). CONCLUSIONS: An individual educational session in the maternity ward about infant sleep position significantly increased the prevalence of supine position for sleeping in the infant's third month. Nevertheless, the intervention was not sufficient to guarantee that most mothers would put their infants to sleep in the recommended position.

BODY MASS INDEX AND ALBUMIN LEVELS ARE ASSOCIATED WITH PULMONARY FUNCTION PARAMETERS IN PEDIATRIC SUBJECTS WITH CYSTIC FIBROSIS.

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.

Clinical outcomes before and after videofluoroscopic swallow study in children 24 months of age or younger / Desfechos clínicos antes e depois da videofluoroscopia da deglutição em crianças com 24 meses de idade ou menos

ABSTRACT Objective: To evaluate the combined impact of videofluoroscopic swallow study (VFSS) and therapeutic feeding and swallowing interventions on clinical outcomes in children with oropharyngeal dysphagia (OPD). Methods: This was an uncontrolled longitudinal analytical study in which OPD patients were evaluated before and after VFSS. Children ≤ 24 months of age diagnosed with OPD in a clinical setting and undergoing VFSS for investigation and management of OPD were included in the study. The study participants received therapeutic feeding and swallowing interventions after having undergone VFSS, being followed at an outpatient clinic for pediatric dysphagia in order to monitor feeding and swallowing difficulties. Respiratory and feeding outcomes were compared before and after VFSS. Results: Penetration/aspiration events were observed in 61% of the VFSSs (n = 72), and therapeutic feeding and swallowing interventions were recommended for 97% of the study participants. After the VFSS, there was a reduction in the odds of receiving antibiotic therapy (OR = 0.007) and in the duration of antibiotic therapy (p = 0.014), as well as in the odds of being admitted to hospital (p = 0.024) and in the length of hospital stay (p = 0.025). A combination of oral and enteral feeding became more common than oral or enteral feeding alone (p = 0.002). Conclusions: A high proportion of participants exhibited penetration/aspiration on VFSS. Therapeutic feeding and swallowing interventions following a VFSS appear to be associated with reduced respiratory morbidity in this population.

RESUMO Objetivo: Avaliar o impacto conjunto da videofluoroscopia da deglutição (VFD) e intervenções terapêuticas de alimentação e deglutição nos desfechos clínicos em crianças com disfagia orofaríngea (DOF). Métodos: Trata-se de um estudo analítico longitudinal não controlado em que pacientes com DOF foram avaliados antes e depois da VFD. Foram incluídas no estudo crianças com idade ≤ 24 meses e diagnóstico clínico de DOF, submetidas à VFD para a investigação e manejo da DOF. Os participantes do estudo receberam intervenções terapêuticas de alimentação e deglutição após terem sido submetidos à VFD, sendo então acompanhados em um ambulatório de disfagia pediátrica para o monitoramento das dificuldades de alimentação e deglutição. Os desfechos respiratórios e alimentares foram comparados antes e depois da VFD. Resultados: Eventos de penetração/aspiração foram observados em 61% das VFD (n = 72), e intervenções terapêuticas de alimentação e deglutição foram recomendadas a 97% dos participantes do estudo. Após a VFD, houve uma redução das chances de receber antibioticoterapia (OR = 0,007) e da duração da antibioticoterapia (p = 0,014), bem como das chances de internação hospitalar (p = 0,024) e do tempo de internação (p = 0,025). A alimentação por via oral e enteral em conjunto tornou-se mais comum do que a alimentação exclusivamente por via oral ou enteral (p = 0,002). Conclusões: Houve alta proporção de crianças que apresentaram penetração/aspiração na VFD. As intervenções terapêuticas de alimentação e deglutição após a VFD parecem estar associadas à redução da morbidade respiratória nessa população.