Radiation dose assessment of pediatric computed tomography of the chest: the need to consider patient size.

Objective: To evaluate the radiation dose of chest computed tomography (CT) examinations of pediatric patients and the extent to which volume CT dose index (CTDIvol) underestimates radiation dose in comparison to size specific dose estimates (SSDE).

Methods: Single-center, retrospective study of consecutive unenhanced pediatric (age <18 years) chest CTs between October 2015 and October 2016. Radiation dose as well as demographic and clinical data were recorded from 133 chest CTs.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Cystic Fibrosis: A Simple and Customized Strategy for Genetic Screening Able to Detect Over 90% of Identified Mutated Alleles in Brazilian Newborns.

Introduction: The incorporation of molecular genetic testing into cystic fibrosis (CF) screening programs increases the specificity of the diagnostic strategy and has the potential to decrease the rate of false- positive results. In this sense, our objective was to develop a genotyping assay that could detect 25 pathogenic variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene with high sensitivity and that could be incorporated into the routine of newborn screening, complementing the current existing protocol used in our public health institution.

Methods: A mini-sequencing assay was standardized using single-base extension in a previously genotyped control sample.

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.

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.

Microbiological contamination of nebulizers used by cystic fibrosis patients: an underestimated problem.

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.

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.

The role of neonatal screening in nutritional evolution in the first 12 months after diagnosis of cystic fibrosis.

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.

Height and sexual maturation in girls with juvenile idiopathic arthritis.

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.

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.

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).