Pediatric pulmonology
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Pediatric pulmonology · Oct 2016
Multicenter StudyA multicenter study on the utility and safety of EBUS-TBNA and EUS-B-FNA in children.
Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and endoscopic ultrasound with an echobronchoscope-guided fine needle aspiration (EUS-B-FNA) are useful modalities in the evaluation of mediastinal lymphadenopathy in adults; however, there is sparse data in children. The aim of this multicenter study is to describe the efficacy and safety of EBUS-TBNA and EUS-B-FNA in children with mediastinal lymphadenopathy of undefined etiology. ⋯ EBUS-TBNA and EUS-B-FNA are safe techniques with a good diagnostic yield in the evaluation of children with mediastinal lymphadenopathy. Pediatr Pulmonol. 2016;51:1031-1039. © 2016 Wiley Periodicals, Inc.
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Pediatric pulmonology · Sep 2016
Variable phenotypic presentation of a novel FOXF1 missense mutation in a single family.
Heterozygous mutations in the FOXF1 transcription factor gene are implicated in alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV), a developmental disorder of the lungs classically presenting with pulmonary hypertension and early demise. Evidence has suggested haploinsufficiency and partial paternal imprinting. We present a family with several affected members with an extremely variable phenotype. ⋯ FOXF1 mutations may have an extremely variable phenotype, possibly as a result of somatic mosaicism and complex gene regulation including unorthodox imprinting of the gene locus. The prolonged survival of the proband suggests the need for aggressive treatment. Pediatr Pulmonol. 2016; 51:921-927. © 2016 Wiley Periodicals, Inc.
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Pediatric pulmonology · Sep 2016
Long term continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV) in children: Initiation criteria in real life.
Long term noninvasive continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV) are increasingly used in children but limited information is available on the criteria and conditions leading to the initiation of these treatments. The aim of the study is to describe the objective overnight respiratory parameters and clinical situations that led to the initiation of CPAP/NIV in a pediatric NIV unit. ⋯ In clinical practice, CPAP/NIV was initiated in an acute, subacute and chronic setting with most patients having an association of several abnormal gas exchange or sleep study parameters. Future studies should evaluate the effectiveness and benefits of CPAP/NIV according to the clinical situation and initiation criteria. Pediatr Pulmonol. 2016; 51:968-974. © 2016 Wiley Periodicals, Inc.
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Pediatric pulmonology · Aug 2016
Review Meta AnalysisEfficacy of oral corticosteroids in the treatment of acute wheezing episodes in asthmatic preschoolers: Systematic review with meta-analysis.
Systemic corticosteroids (SCS) are used for treat preschoolers with acute asthma or wheezing exacerbations, with conflicting results. ⋯ Treatment with OCS in the ED or hospital may be beneficial in toddlers and preschoolers with frequent asthma/wheezing exacerbations. However, more studies are needed before OCS can be broadly recommended for this age group. Future trials should be carefully designed to avoid bias and according to our findings regarding administration setting. Pediatr Pulmonol. 2016;51:868-876. © 2016 Wiley Periodicals, Inc.
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Pediatric pulmonology · Aug 2016
Liberation from home mechanical ventilation and decannulation in children.
The prevalence of children requiring outpatient invasive long-term mechanical ventilation is increasing. For some children, liberation from home mechanical ventilation (HMV) and decannulation is the desired outcome. This study describes our experience liberating tracheostomy and HMV (T-HMV) dependent children from respiratory technologies. ⋯ Liberation from respiratory technology is a complex, multi-step process that can be accomplished in medically complex children with varying underlying disease processes at relatively young ages. Five major steps (tracheotomy, initiation of HMV, initiation of TC trials, HMV independence, and decannulation) performed in conjunction with clinic visits, procedures, and home nursing support were integral in the successful decannulation process. Pediatr Pulmonol. 2016;51:838-849. © 2016 Wiley Periodicals, Inc.