Pediatric pulmonology
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Pediatric pulmonology · Mar 2014
Prevalence of pediatric periodic leg movements of sleep after initiation of PAP therapy.
Positive-airway-pressure (PAP) is a treatment for obstructive-sleep-apnea (OSA). In adults, initiation of PAP-therapy may unmask periodic-limb movements-of-sleep (PLMS). We present a series of children in whom PLMS was aggravated or induced following initiation of PAP therapy. ⋯ PLMS were seen in 5.1% of children after initiation of PAP. OSA may mask PLMS, which appears as breathing improves. Alternatively, PAP may unmask or induce PLMS. PLMS may potentially be a cause of treatment failure in children on recent-onset PAP therapy.
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Pediatric pulmonology · Mar 2014
Case ReportsA case of mucoepidermoid carcinoma of the bronchus presented with hydropneumothorax in a child.
Mucoepidermoid carcinoma is a rare pulmonary neoplasm seen in the pediatric population. We report a low grade mucoepidermoid carcinoma in a 12-year-old boy who presented with spontaneous pneumothorax.
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Pediatric pulmonology · Mar 2014
Case ReportsRespiratory insufficiency in a newborn with congenital hypothyroidism due to a new mutation of TTF-1/NKX2.1 gene.
NK2 homeobox-1 (NKX2.1) gene encoding the thyroid transcription factor-1 (TTF-1) plays a critical role in lung, thyroid, and central nervous system morphogenesis and function; mutations cause a rare form of progressive respiratory failure associated with alterations of surfactant synthesis, composition, and homeostasis. Molecular mechanisms are heterogeneous and poorly explored. A 28 days old male, soon after birth, presented respiratory failure requiring oxygen treatment at FiO2 27%, prolonged for 2 weeks. ⋯ Sequencing analysis of the three exons of the TTF1 revealed a heterozygous mutation c.334G > T that results in the replacement of glycine in position 112 with a stop codon, generating a nonsense protein that lacks the correct transactivation domain in the C-terminal region. Genetic analysis of the family showed that the father, who was asymptomatic, carried the mutation. Screening for TTF-1 deletions or mutations should always be considered in children with congenital hypothyroidism and an unexplained neonatal respiratory distress or neurodevelopmental deficits.
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Pediatric pulmonology · Mar 2014
Predictors of severity and mortality in children hospitalized with respiratory syncytial virus infection in a tropical region.
Respiratory syncytial virus (RSV) is one of the leading causes of acute lower respiratory infection (ALRI) in infants and young children. Although ALRI is a major public health problem in developing countries located in tropical areas, studies about RSV epidemiology in these regions are scarce. ⋯ RSV is an important cause of ALRI in infants and young children living in tropical regions, especially during the rainy season. The identified predictors of severe disease and mortality should be taken into account when planning interventions to reduce the burden of ALRI in young children living in these regions.
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Pediatric pulmonology · Mar 2014
Case ReportsAn infant with pulmonary interstitial glycogenosis: clinical improvement is associated with improvement in the pulmonary diffusion capacity.
Pulmonary interstitial glycogenosis (PIG) is an idiopathic interstitial lung disease of infants. The underlying pulmonary pathophysiology of PIG has not been well characterized. Herein we report a term-gestation infant who presented with persistent tachypnea and hypoxia. ⋯ Follow-up demonstrated progressive clinical improvement as well as an increase in Z-FVC and normalization of DLCO /VA. Our in vivo physiological findings are consistent with previous reports that symptom resolution correlated with histological thinning of the alveolar septae upon repeat lung biopsy. The restrictive lung disease we observed is consistent with expected reduced compliance of an alveolar interstitial lung process like PIG, whereas the absence of a reduction in FEV0.5/FVC confirms the absence of obstructive airway disease.