Emeritus Faculty, Acad Council, Radiology - Pediatric Radiology
With the advent of therapies aimed at young patients with cystic fibrosis, who have mildly reduced pulmonary function, the need for improved outcome measures that discriminate treatment effects has become important. Pulmonary function measurements or chest high-resolution computed tomography (HRCT) scores have been separately used to assess interventions. We evaluated these modalities separately and together during a treatment study to develop a more sensitive outcome measure. In a 1-year trial, 25 children randomized either to daily Pulmozyme or to normal saline aerosol were evaluated at randomization and at 3 and 12 months. Outcome variables were pulmonary function test (PFT) results, a global HRCT score, and a composite score incorporating PFTs and HRCT scoring. Regression analyses with generalized estimating equations permitted estimation of the difference in treatment effect between groups over time for each outcome. The largest difference in treatment effects observed at 12 months, measured by the percentage change from baseline, were with the composite total and maximal CT/PFT scores (35.4 and 30.4%), compared with mean forced expiratory flow during the middle half of the FVC (FEF25-75%) (13.0%) and total and maximal global HRCT scores (6.2%, 7.2%). The composite total and maximal CT/PFT scores were the most sensitive outcome measures for discriminating a treatment effect in children with cystic fibrosis with normal or mildly reduced pulmonary function during a 1-year trial of Pulmozyme.
View details for DOI 10.1164/rccm.200209-1093OC
View details for Web of Science ID 000185039000015
View details for PubMedID 12746252
Bronchopulmonary dysplasia (BPD) is both a significant clinical problem associated with neonatal intensive care and a sign of the success of that care. It can best be understood in the context of the historical continuum of improving survivability for prematurely born infants. Some of the adolescents and young adults surviving with BPD will have late pulmonary dysfunction. All the molecular-biologic and technologic approaches used in the past 25 years have not clearly reduced the overall incidence of BPD. It would seem time to approach seriously the problem of decreasing the overall incidence of BPD by decreasing the incidence of premature birth in the United States.
View details for Web of Science ID A1992LA27000002
View details for PubMedID 1526068
View details for PubMedID 2003428
The effect of dexamethasone (0.1, 1, and 5 mg/kg/d given subcutaneously from d 14-18) was tested in infant mice continuously exposed from birth to either humidified air or 80% oxygen. Dexamethasone significantly decreased lung wet wt (p less than 0.01), lung water (p less than 0.021), lung dry wt, protein, and DNA (p less than 0.001) in both air- and oxygen-exposed animals. Dexamethasone, however, had no effect on lung compliance measured after animals were killed on d 18. It also had no effect on the increase in the blood-air barrier thickness or decrease in the blood-air exchange surface area seen in the 80% oxygen-exposed mice. Dexamethasone decreased thymus gland wt (p less than 0.001), body wt gain (p less than 0.001), brain wt (p less than 0.001), and lung lymphocytes (p less than 0.05) in both air- and oxygen-exposed animals. The effect of 1 mg/kg and 5 mg/kg of the drug could not be differentiated. During the 4 d of drug administration, one air- and one oxygen-exposed animal died; both received 5 mg/kg/d of dexamethasone; microscopic and culture evidence of infection was not found. If dexamethasone causes similar effects in human infants with bronchopulmonary dysplasia, it should be used with great caution even for short-term clinical management.
View details for Web of Science ID A1989T947000008
View details for PubMedID 2726308
To evaluate a high-resolution computed tomography (HRCT) scoring system, clinical parameters, and pulmonary function measurements in patients with cystic fibrosis (CF) before and after therapy for a pulmonary exacerbation.Patients (n = 17) were evaluated by spirometer-triggered HRCT imaging, clinical parameters, and pulmonary function tests (PFTs) before and after treatment. HRCT scans were reviewed by 3 radiologists using a modified Bhalla scoring system.Bronchiectasis, bronchial wall thickening, and air trapping were identified in all subjects on initial evaluation. The initial total HRCT score correlated significantly with the Brasfield score (r = -.91, P <.001) and several PFT measures. After treatment, there were improvements in the acute change clinical score (ACCS) (P <.001), most pulmonary function measurements, and total HRCT score (P <.05). Bronchiectasis, bronchial wall thickening, and air trapping did not significantly change. Mucus plugging subcomponent HRCT score, slow vital capacity (SVC), forced expiratory volume in 1 second (FEV(1)), and forced vital capacity (FVC) (percent predicted) and reversible and total HRCT scores were most sensitive to change by effect size analysis.Improvements occurred with treatment in total and reversible HRCT scores, PFTs, and ACCS. Total and reversible HRCT scores and percent predicted SVC, FEV1, and FVC were the most sensitive to change. The greatest change was seen in the mucus plugging subcomponent HRCT score.
View details for Web of Science ID 000168175400024
View details for PubMedID 11295720
Bronchopulmonary dysplasia is a chronic lung disease that often develops after mechanical ventilation in prematurely born infants with respiratory failure. It has become the most common form of chronic lung disease in infants in the United States. The long-term outcome for infants with bronchopulmonary dysplasia has not been determined.We studied the pulmonary function of 26 adolescents and young adults, born between 1964 and 1973, who had bronchopulmonary dysplasia in infancy. We compared the results with those in two control groups: 26 age-matched adolescents and young adults of similar birth weight and gestational age who had not undergone mechanical ventilation, and 53 age-matched normal subjects.Sixty-eight percent of the subjects with bronchopulmonary dysplasia in infancy (17 of the 25 tested) had airway obstruction, including decreases in forced expiratory volume in one second, forced expiratory flow between 25 and 75 percent of vital capacity, and maximal expiratory flow velocity at 50 percent of vital capacity, as compared with both control groups (P less than 0.0001 for all comparisons). Twenty-four percent of the subjects with bronchopulmonary dysplasia in infancy had fixed airway obstruction, and 52 percent had reactive airway disease, as indicated by their responses to the administration of methacholine or a bronchodilator. Hyperinflation (an increased ratio of residual volume to total lung capacity) was more frequent in the subjects with a history of bronchopulmonary dysplasia than in either the matched cohort (P less than 0.0006) or the normal controls (P less than 0.0004). Six of the subjects who had bronchopulmonary dysplasia in infancy had severe pulmonary dysfunction or current symptoms of respiratory difficulty.Most adolescents and young adults who had bronchopulmonary dysplasia in infancy have some degree of pulmonary dysfunction, consisting of airway obstruction, airway hyperreactivity, and hyperinflation. The clinical consequences of this dysfunction are not known.
View details for Web of Science ID A1990EP37600003
View details for PubMedID 2247118
A case of congenital pancreatic cysts detected antenatally by ultrasound is presented. This is the second case detected antenatally. Congenital pancreatic cysts should be included in the differential diagnosis of upper abdominal cystic masses in the fetus and newborn infant.
View details for Web of Science ID A1990DP57300021
View details for PubMedID 2202976
Research support, training, and methods in diagnostic radiology have been the focus of analysis for 2 decades and continue to be important in the effective competition for research grants, especially those granted by the National Institutes of Health. The limitation of hypothesis formation and testing to imaging methods alone, however, could be a major impediment to more successful sponsorship of research in this discipline. The author emphasizes that a well-designed research plan in diagnostic radiology should not be restricted solely to imaging methods and should include such factors as the formation of well-defined hypotheses testable by means of inductive reasoning and, most important, flexibility in approaches to research methods. Other factors influencing effective research in diagnostic imaging include the establishment of mentors for continuous training and support, uninterrupted time for research, and a totally supportive environment. Improvements in these areas may enhance the competitiveness of research proposals from departments of diagnostic radiology.
View details for Web of Science ID A1989U594000062
View details for PubMedID 2717771
A 14-year-old boy with a retrohepatic para-aortic pheochromocytoma demonstrates the potential difficulties of localization in an unusual extra-adrenal site of pheochromocytoma. Ultrasonography, computed tomography, metaiodobenzylguanidine scanning, and angiography with venous sampling failed to define the true anatomic location of this tumor. Collateral venous drainage produced confusing venous sampling data and represents a potential pitfall in the interpretation of these studies.
View details for Web of Science ID A1988Q843100025
View details for PubMedID 3244086