Pediatric pulmonology
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Respiratory muscle function in patients with cystic fibrosis (CF) can be assessed by measurement of maximal inspiratory pressure (Pimax ), maximal expiratory pressure (Pemax ), and pressure-time index of the respiratory muscles (PTImus ). We investigated the differences in maximal respiratory pressures and PTImus between CF patients with no gross hyperinflation and healthy controls and described the effects of pulmonary function and nutrition impairment on respiratory muscle function in this group of CF patients. Forced expiratory volume in 1 sec (FEV1 ), forced vital capacity (FVC) and maximal expiratory flow between 25% and 75% of VC (MEF25-75 ), body mass index (BMI), upper arm muscle area (UAMA), Pimax , Pemax , and PTImus were assessed in 140 CF patients and in a control group of 140 healthy subjects matched for age and gender. ⋯ Median PTImus in CF patients compared to controls was significantly increased [PTImus = 0.110 (0.076-0.160) in CF vs. 0.094 (0.070-0.137) in controls, P = 0.049] and it was significantly higher in CF patients with impaired pulmonary function. In CF patients, PTImus was significantly negatively related to upper arm muscle area (r = 0.184, P = 0.031). These findings suggest that CF patients with no severe lung disease compared to healthy subjects exhibit impaired respiratory muscle function, while CF patients with impaired pulmonary function and nutrition indices exhibit higher PTImus values.
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Pediatric pulmonology · Aug 2013
Comparative StudyDiagnosis of invasive pulmonary aspergillosis in children with bronchoalveolar lavage galactomannan.
Invasive pulmonary aspergillosis (IPA) is a life-threatening complication in immunocompromised patients. Early diagnosis and therapy improves outcome. Assessment of galactomannan (GM) in bronchoalveolar lavage (BAL) fluid is a proposed tool to diagnose IPA. Little is known about the diagnostic value of BAL GM in children. ⋯ BAL GM test had good diagnostic value in children suspected of IPA. However, the decision to continue or start antifungal therapy was mainly determined by the clinical suspicion of IPA based on chest CT-outcome, serum GM index values and failure of antibiotic therapy.
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Pediatric pulmonology · Aug 2013
Case ReportsHemoptysis from bronchial varices associated with pulmonary vein stenosis: role of surgical repair.
We present the case of a 6-year-old child who presented with an episode of life threatening hemoptysis. Investigations revealed multiple areas of endobronchial varices and abnormal pleural vessels as well as severe left pulmonary vein stenosis and an atrial septal defect (ASD). ⋯ The bronchial varices in the right lung remain unchanged. No further hemoptysis has occurred and the child continues to be monitored.
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Pediatric pulmonology · Aug 2013
Comparative StudyNight-to-night consistency of at-home nocturnal pulse oximetry testing for obstructive sleep apnea in children.
At-home nocturnal pulse oximetry has a high positive predictive value (PPV) for polysomnographically-diagnosed obstructive sleep apnea (OSA) but no studies have been published testing the night-to-night consistency of at-home nocturnal pulse oximetry for the evaluation of suspected OSA in children. We therefore determined the night-to-night consistency of nocturnal pulse oximetry as a diagnostic test for OSA in children. ⋯ Night-to-night consistency of nocturnal pulse oximetry as a diagnostic test for OSA showed excellent agreement. Night-to-night consistency of pulse oximetry, as analyzed by the MOS, for diagnosis and severity evaluation further validates this abbreviated testing method for pediatric OSA. Polysomnography (PSG) is required to rule in or rule out OSA in children if a single night oximetry testing is inconclusive.
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Pediatric pulmonology · Aug 2013
Comparative StudyIncreased adherence to CFF practice guidelines for pulmonary medications correlates with improved FEV1.
CFF practice guidelines recommend patients ≥age 6 use dornase alfa and hypertonic saline daily, and those ≥age 6 colonized with Pseudomonas aeruginosa use inhaled tobramycin and oral azithromycin to improve lung function and reduce pulmonary exacerbations. A decline in FEV1 was noted in our 2008 CF Center Report. We hypothesized that increasing adherence to prescribing guidelines for these pulmonary medications would improve mean FEV1. ⋯ Educating clinicians about guidelines, providing feedback on adherence to guidelines, and monitoring prescribing patterns improves prescribing adherence. FEV1 showed improvement after months of sustained adherence, trending towards significance. Longer follow-up is necessary to determine if improved prescribing adherence translates into improved FEV1 or slows rate of decline in FEV1.