Pediatric pulmonology
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Pediatric pulmonology · Nov 2011
Clinical outcomes in infants with cystic fibrosis transmembrane conductance regulator (CFTR) related metabolic syndrome.
An unavoidable outcome of cystic fibrosis newborn screening (CF NBS) programs is the detection of infants with an indeterminate diagnosis. The United States CF Foundation recently proposed the term cystic fibrosis transmembrane conductance regulator related metabolic syndrome (CRMS) to describe infants with elevated immunoreactive trypsinogen (IRT) on NBS who do not meet diagnostic criteria for CF. The objective of this study was to describe the clinical outcomes of infants with CRMS identified through an IRT/DNA algorithm. ⋯ Close initial monitoring of these patients is warranted. Pediatr. Pulmonol. 2011; 46:1079-1084. © 2011 Wiley Periodicals, Inc.
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Pediatric pulmonology · Nov 2011
Non-invasive ventilation on a pediatric intensive care unit: feasibility, efficacy, and predictors of success.
There is only sparse data on the use of non-invasive ventilation (NIV) in acute respiratory failure (ARF) in infants and children. For this setting we investigated feasibility and efficacy of NIV and aimed to identify early predictors for treatment failure. ⋯ NIV can be effective in infants and children with ARF. Low pH 1-2 hr after start of NIV is associated with NIV failure. It may therefore be useful in the decision to continue or stop mask ventilation.
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Pediatric pulmonology · Nov 2011
Exhaled breath temperature in healthy children is influenced by room temperature and lung volume.
Exhaled breath temperature (EBT) has been proposed for the non-invasive assessment of airway inflammation. Previous studies have not examined the influence of room temperature or lung size on the EBT. ⋯ Exhaled breath temperature measurements are highly feasible in children with a 95% success rate in this healthy population. Room temperature and SVC significantly influence EBT variables in healthy children. Further studies are required to investigate the ability of EBT to assess airway inflammation in children with respiratory disease. Pediatr. Pulmonol. 2011; 46:1062-1068. © 2011 Wiley Periodicals, Inc.
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Pediatric pulmonology · Nov 2011
Bilateral lung transplantation for pediatric idiopathic pulmonary arterial hypertension: a multi-center experience.
Many children with idiopathic pulmonary arterial hypertension (IPAH) experience disease progression despite advanced medical therapy. In these children, heart-lung or bilateral lung transplantation (BLTx) remain the only therapeutic options when other treatments fail. Data on functional outcome after BLTx in children with IPAH are limited. ⋯ The median survival for children in this cohort was 45 months (2-123). Our data suggest that BLTx is a valuable therapeutic option for children with end-stage IPAH with outcomes comparable to that after heart-lung transplantation in children with pulmonary arterial hypertension or those patients undergoing lung transplantation for other indications. In the majority of children, a good cardiopulmonary function is possible following BLTx, making BLTx a good therapeutic option and maximizing donor organ utilization by allowing more hearts to be available for children needing cardiac transplantation.
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Pediatric pulmonology · Nov 2011
Case ReportsWhat could hemoptysis hide in an otherwise healthy child?
We describe a previously healthy 12-year-old boy who attended our Department after a sudden episode of hemoptysis and recurrent consolidation. CT-scan revealed a mediastinal tumor. ⋯ Hemoptysis is an unusual presenting symptom in an otherwise healthy child with a well capsulated, unruptured mature mediastinal teratoma. In this report, hemoptysis prompted us to undertake the diagnostic work-up that eventually disclosed the tumor.