Pediatric pulmonology
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Pediatric pulmonology · Jun 2009
ReviewCongenital central hypoventilation syndrome from past to future: model for translational and transitional autonomic medicine.
The modern story of CCHS began in 1970 with the first description by Mellins et al., came most visibly to the public eye with the ATS Statement in 1999, and continues with increasingly fast paced advances in genetics. Affected individuals have diffuse autonomic nervous system dysregulation (ANSD). The paired-like homeobox gene PHOX2B is the disease-defining gene for CCHS; a mutation in the PHOX2B gene is requisite to the diagnosis of CCHS. ⋯ A growing number of cases of CCHS are identified after the newborn period, with presentation from infancy into adulthood. An improved understanding of the molecular basis of the PHOX2B mutations and of the PHOX2B genotype/CCHS phenotype relationship will allow physicians to anticipate the clinical phenotype for each affected individual. To best convey the remarkable history of CCHS, and to describe the value of recognizing CCHS as a model for translational and transitional autonomic medicine, we present this review article in the format of a chronological story, from 1970 to the present day.
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Pediatric pulmonology · May 2009
Higher pulmonary dead space may predict prolonged mechanical ventilation after cardiac surgery.
Children undergoing congenital heart surgery are at risk for prolonged mechanical ventilation and length of hospital stay. We investigated the prognostic value of pulmonary dead space fraction as a non-invasive, physiologic marker in this population. In a prospective, cross-sectional study, we measured pulmonary dead space fraction in 52 intubated, pediatric patients within 24 hr postoperative from congenital heart surgery. ⋯ The area under the receiver operator characteristic curve for this model was 0.91. Elevated pulmonary dead space fraction is associated with prolonged mechanical ventilation and hospital stay in pediatric patients who undergo surgery for congenital heart disease and has additive predictive value in identifying those at risk for longer duration of mechanical ventilation. Pulmonary dead space may be a useful prognostic tool for clinicians in patients who undergo congenital heart surgery.
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Pediatric pulmonology · May 2009
Changes in lung function in young cystic fibrosis patients between two courses of intravenous antibiotics against Pseudomonas aeruginosa.
Repeated intravenous antibiotic therapy (IVAT) against chronic pulmonary infection with Pseudomonas aeruginosa is often necessary in cystic fibrosis patients (CF). The aim of this study was to monitor kinetics of degradation of pulmonary and nutritional status after IVAT in CF patients. ⋯ In CF, there is a progressive decrease in lung function without any evident deterioration of nutritional status and PA between two IVAT.
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Pediatric pulmonology · May 2009
Early oxygen uptake recovery following exercise testing in children with chronic chest diseases.
The value of exercise testing as an objective measure of disease severity in patients with chronic chest diseases (CCD) is becoming increasingly recognized. The aim of this study was to investigate changes in oxygen uptake (VO2) during early recovery following maximal cardiopulmonary exercise testing (CPXT) in relation to functional capacity and markers of disease severity. Twenty-seven children with CCD (age 12.7 +/- 3.1 years; 17 female) [19 children with Cystic fibrosis (CF) (age 13.4 +/- 3.1 years; 10 female) and 8 with other stable non-CF chest diseases (NON-CF) (age 11.1 +/- 2.2 years; 7 female)] and 27 healthy controls (age 13.2 +/- 3.3 years; 17 female) underwent CPXT on a cycle ergometer. ⋯ In the children with CCD, FEV(1), FEF(25-75), and BMI were not significantly correlated with VO2(peak) or early VO2 recovery. Lung function does not necessarily reflect aerobic fitness and the ability to recover from exercise in these patients. A significant relationship was found between VO2(peak) and early VO2 recovery (r = -0.39, P = 0.044) in the children with CCD, showing that a greater aerobic fitness corresponded with a faster recovery.
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Endobronchial ultrasound (EBUS) is a recently introduced technique that has significantly advanced bronchoscopic techniques in adult medicine. Use of ultrasound allows far more accurate localization and sampling of both peripheral pulmonary, as well as mediastinal and hilar lesions. This has led to greater diagnostic success, with a reduced rate of complications. ⋯ It may be useful in the diagnosis of lymphoma, or neurogenic tumors, as well as many other diseases resulting in mediastinal or hilar lymphadenopathy. EBUS is a minimally invasive technique that allows tissue sampling of peripheral lung lesions, or mediastinal/hilar masses, with a high diagnostic accuracy, and a significantly lower morbidity and mortality than alternative approaches. The indications for and the use of EBUS in pediatric patients is certain to increase in the future.