Changes in left ventricular diastolic filling patterns by Doppler echocardiography in cystic fibrosis.

The onset of cor pulmonale is a common terminal finding in patients with cystic fibrosis. Since Doppler echocardiography can detect changes in diastolic filling patterns prior to the onset of either systolic dysfunction or clinical symptoms, we utilized this technique to determine whether detectable changes in left ventricular diastolic filling patterns exist in patients with cystic fibrosis. Among 25 patients, the proportion of left ventricular filling attributable to atrial contraction was significantly increased when compared with age-matched control individuals. When filling patterns were compared with severity of pulmonary disease, worsening pulmonary disease was directly correlated to shifts in left ventricular filling patterns. We conclude that changes in left ventricular patterns of relaxation are detectable early in the course of cystic fibrosis and that such changes are probably progressive. Early detection could lead to therapeutic trials designed to improve left ventricular filling and delay the onset of overt cor pulmonale.

Sequential ciprofloxacin therapy in pediatric cystic fibrosis: comparative study vs. ceftazidime/tobramycin in the treatment of acute pulmonary exacerbations. The Cystic Fibrosis Study Group.

BACKGROUND: Cystic fibrosis patients have chronic bacterial infections of the respiratory tract, most commonly Pseudomonas aeruginosa. Although controversial, administration of antibiotic therapy during acute pulmonary exacerbations is standard practice. Fluoroquinolones are currently not indicated for use in young children because of the observation of arthropathy and damage to growing cartilage in beagle puppies. Because of its activity against P. aeruginosa and excellent oral bioavailability, ciprofloxacin offers a unique therapeutic alternative for this patient population. OBJECTIVE: This prospective, randomized, double blind study compared the efficacy and safety of sequential intravenous/oral ciprofloxacin vs. ceftazidime/tobramycin in hospitalized pediatric cystic fibrosis patients with an acute pulmonary exacerbation associated with P. aeruginosa infection. METHODS: One hundred thirty patients (ages 5 to 17 years) were randomized to receive either i.v. ciprofloxacin 10 mg/kg every 8 h for 7 days followed by oral ciprofloxacin 20 mg/kg every 12 h for a minimum of 3 days or i.v. ceftazidime 50 mg/kg every 8 h plus i.v. tobramycin 3 mg/kg every 8 h for a minimum of 10 days. Clinical, bacteriologic and safety responses were assessed throughout the study. RESULTS: All 84 patients (median age, 11 years; range, 5 to 17 years) valid for efficacy in both treatment groups demonstrated clinical improvement. Five patients experienced clinical relapses (3 ciprofloxacin, 2 ceftazidime/tobramycin) by the 2- to 4-week follow-up. Intent-to-treat analysis demonstrated similar clinical findings between the two treatment groups at both the end of therapy and follow-up. Clinical improvement correlated with improvement in pulmonary function studies and the acute clinical scoring system but not with bacteriologic eradication of Pseudomonas. DNA profiles demonstrated that irrespective of colony morphology, usually one clonal strain was associated with each patient's pulmonary exacerbation. Treatment-associated musculoskeletal events occurred with equal frequency (22% vs. 21%) in both study drug groups (n = 129), and arthralgias were within the range of rates for cystic fibrosis arthropathy. None of these events required study drug discontinuation. CONCLUSION: Sequential i.v./oral ciprofloxacin monotherapy offers a safe and efficacious alternative to standard parenteral therapy for acute pulmonary exacerbations in pediatric cystic fibrosis patients.

Comparison of the lung sound frequency spectra of infants and adults.

Auscultation of the infant chest reveals lung sounds that seem different from those of adults. To characterize this subjective difference, we compared the phonopneumographic median frequencies of lung sounds of seven full-term and six premature infants with those of seven adults free of cardiopulmonary disease. The median frequencies over the upper lobes for the adults, term infants, and premature infants were 282 +/- 63(SD) Hz, 383 +/- 80 Hz, and 483 +/- 86 Hz, respectively. At this location the differences among the three groups were significant (p less than 0.01). Over the lower lobes, the median frequencies for the adults, term infants, and premature infants were 243 +/- 56 Hz, 386 +/- 76 Hz, and 390 +/- 63 Hz, respectively. Here the difference between the adults and both groups of infants was significant (p less than 0.01), but that between the term and premature infants was not. We conclude that the normal lung sounds of newborn infants contain higher-frequency components than those of adults. We postulate that this difference is the result of less filtering of the lung sound in infants. This difference should be considered when lung sounds of infants are described or assessed for the presence of abnormalities.

The clinical and imaging spectrum of findings in patients with congenital lobar emphysema.

Congenital lobar emphysema (CLE) is an important cause of infantile respiratory distress that may require surgical intervention. We retrospectively reviewed the clinical and imaging findings in eight infants with CLE. In our series, CLE was more common in females, predominantly involved the right lung, often presented with an opaque lobe from retained fetal lung fluid, and frequently involved the lower lobes, multiple segments or lobes, and both lungs. Most patients with CLE were diagnosed and managed on the basis of clinical and plain radiographic findings alone. Computed tomography, and occasionally ventilation/perfusion scintigraphy, were helpful in confirming the diagnosis and in guiding management decisions in several cases; bronchoscopy showed that stenosis of the right mainstem bronchus was the cause of CLE in one case. Three patients experienced progressive worsening of respiratory distress and required surgical resection of the affected lobe for cure; the remaining five patients were managed medically with eventual remission of symptoms.

Does airway closure affect lung sound generation?

The purpose of this experiment was to test the hypothesis that airway closure impedes the production of lung sounds at low lung volume. We recorded breath sounds in three healthy men during inspiratory vital capacity manoeuvres in upright, head-down and lateral decubitus postures. We then compared the rate of increase of breath sound intensity (BSI) between the dependent and non-dependent lung zones. Closing volumes, measured separately, were normal in the upright but increased in the head-down postures. The data revealed no consistent lag in the rate of increase of BSI over the dependent lung zones in any of the postures. Our data suggest that airway closure does not influence lung sound generation. If true, this implies that lung sounds are produced proximally to the site of physiologic airway closure.

Congenital pulmonary venolobar syndrome revisited.

The term "congenital pulmonary venolobar syndrome" (CPVS) encompasses a number of congenital abnormalities of the thorax that often occur in combination. Major components of CPVS include hypogenetic lung, partial anomalous pulmonary venous return (this and the former are two of the most constantly occurring components), absence of a pulmonary artery, pulmonary sequestration, systemic arterialization of the lung, absence of the inferior vena cava, and accessory diaphragm. Minor components of CPVS include tracheal trifurcation, eventration and partial absence of the diaphragm, phrenic cyst, horseshoe lung, esophageal and gastric lung, anomalous superior vena cava, and absence of the left pericardium. Most patients with CPVS have no symptoms and require no therapy; however, surgical intervention is often necessary in infants with severe symptoms. The authors review the imaging findings in 29 patients with CPVS and review the literature concerning the diagnosis and management of this complex syndrome in order to improve the understanding of CPVS among radiologists and clinicians.