Serum KL-6 differentiates neuroendocrine cell hyperplasia of infancy from the inborn errors of surfactant metabolism.

BACKGROUND: The study was conducted in order to determine if the glycoprotein KL-6 is a useful biomarker in differentiating neuroendocrine cell hyperplasia of infancy (NEHI), a benign form of children's interstitial lung disease, from the more severe inborn errors of surfactant metabolism (IESM), since their clinical presentation can be similar. METHODS: Serum KL-6 levels were measured in 10 healthy control children, 6 with NEHI and 13 with IESM (4 with surfactant protein C (SP-C) and 9 with ABCA3 mutations). The initial clinical presentation, findings on previous CT scans and interstitial lung disease (ILD) scores at the time of KL-6 testing were compared. Correlations of KL-6 levels with age and with interval from lung biopsy were evaluated. RESULTS: The median (range) KL-6 levels were 265 (1-409), 194 (47-352), 1149 (593-4407) and 3068 (726-9912) U/ml for the control, NEHI, SP-C and ABCA3 groups, respectively. When compared with the control and NEHI groups, median KL-6 levels were significantly higher in the SP-C (p<0.01; p = 0.01, respectively) and ABCA3 groups (p<0.001; p = 0.001, respectively); however, there was no difference between the control and NEHI groups (p = 0.91). An inverse relationship was seen between KL-6 levels and age in the IESM groups, but not in the NEHI or control groups. Children with NEHI had similar presenting clinical features and were equally symptomatic at the time of KL-6 measurement as those with IESM. CONCLUSIONS: Children with NEHI have normal KL-6 levels, in contrast to those with IESM, who have elevated serum KL-6 levels; serum KL-6 may be a useful biomarker in distinguishing between these entities when their clinical presentations overlap.

Seasat scatterometer: results of the gulf of alaska workshop.

The Seasat microwave scatterometer was designed to measure, globally and in nearly all weather, wind speed to an accuracy of +/- 2 meters per second and wind direction to +/- 20 degrees in two swaths 500 kilometers wide on either side of the spacecraft. For two operating modes in rain-free conditions, a limited number of comparisons to high-quality surface truth indicates that these specifications may have been met.

Ciprofloxacin versus tobramycin plus azlocillin in pulmonary exacerbations in adult patients with cystic fibrosis.

Twenty adult patients with cystic fibrosis who were experiencing acute pulmonary exacerbations were enrolled in a randomized, controlled trial comparing oral ciprofloxacin with intravenous tobramycin plus azlocillin. Efficacy of the two treatments was compared based upon changes in clinical status, pulmonary function tests, white blood cell counts, and quantitative bacteriology of sputum. No statistically significant differences were detected in these parameters of response between the two treatment groups (p greater than 0.05). Ciprofloxacin appears to be therapeutically equivalent to intravenous antibiotics in the treatment of adult patients with cystic fibrosis who are experiencing pulmonary exacerbations associated with susceptible bacteria.

Controlled trial of aztreonam vs. tobramycin and azlocillin for acute pulmonary exacerbations of cystic fibrosis.

The efficacy of aztreonam was compared to that of standard therapy consisting of tobramycin and azlocillin in the treatment of acute pulmonary exacerbations of cystic fibrosis in a randomized, open trial. Fifteen patients were randomized to each treatment. Responses were assessed based on changes in pulmonary and clinical scores, white blood cell counts, pulmonary function tests and quantitative bacteriology of sputum which were performed before, every 5 to 7 days during and on the last day of therapy. Patients in both groups responded to therapy and there were no statistically significant differences in changes in the above indicators of response with therapy between the two groups (P greater than 0.05). The incidence of detection of Pseudomonas aeruginosa isolates resistant to all three study antibiotics increased with therapy. Side effects were limited to transient elevations of liver enzymes (both groups) and rash and fever in one patient treated with azlocillin. Aztreonam represents effective therapy for pulmonary exacerbations of cystic fibrosis associated with susceptible pathogens.

Efficacy of aztreonam in pulmonary exacerbations of cystic fibrosis.

A noncomparative pilot study was conducted to assess the potential usefulness of aztreonam in pulmonary exacerbations of cystic fibrosis. Of 27 patients initially enrolled 25 received sufficient courses of aztreonam therapy to be evaluable. All patients received 200 mg/kg/day of aztreonam in 4 equally divided doses administered intravenously. Of 57 isolates of Pseudomonas aeruginosa from pretherapy sputum cultures, 48 were susceptible to aztreonam in vitro as were 11 of 18 strains isolated at the conclusion of therapy. With treatment colony counts of P. aeruginosa in sputum were reduced by 3 log10 or more in 15 patients. It was totally (but temporarily) eradicated in 11 of these patients. Clinical scores and white blood cell counts improved significantly (P less than 0.05). Side effects of aztreonam were limited to transient elevations of liver enzymes occurring in 16 patients. Aztreonam merits further evaluation in a randomized, comparative trial with standard antibiotic therapy for cystic fibrosis.

The use of aztreonam in pediatric patients: a review.

Aztreonam is the first monobactam available for use in this country. A parenteral antibiotic, its antibacterial spectrum is limited to gram-negative aerobic pathogens. The drug's pharmacologic, pharmacokinetic, and toxicologic properties are reviewed. Based on early experience in pediatric patients, certain recommendations can be made for the use of aztreonam.

Cost savings and economic considerations using home intravenous antibiotic therapy for cystic fibrosis patients.

Our Cystic Fibrosis (CF) Center made an effort to utilize home intravenous antibiotic therapy (HIVAT) as an alternative to continued hospitalization during a 1-year study. After thorough individual clinical and financial evaluation, 27 of 41 CF patients admitted for treatment, including antibiotic therapy, were selected for HIVAT to complete a 14- to 21-day treatment course (mean 15.1 days). The 27 patients (6-28 years old, mean 16 years) incurred a total of $698,587 in hospital charges and physician fees during 96 admissions. The average charge for 974 inpatient days was $717/day ($7,280 per admission). After an average of 10.2 days of inpatient care, the 27 patients underwent 79 courses of HIVAT for an additional 8 days; 21 additional HIVAT courses in six of these patients were initiated on an outpatient basis between frequent readmissions. The 811 days of HIVAT resulted in $85,027 total charges by two home care companies. The charges per day of HIVAT by one company were almost twice that of the other. The average daily cost of HIVAT was $108/day. If the HIVAT patients had remained hospitalized to complete the course of intravenous antibiotic therapy, the projected inpatient costs would have been $589,271. Therefore, the 811 days of HIVAT over a 1-year period resulted in total estimated direct cost savings of $501,770. The average savings per course of HIVAT was $5,017, or $618/day.(ABSTRACT TRUNCATED AT 250 WORDS)

Comparison of low, medium, and high carbohydrate formulas for nighttime enteral feedings in cystic fibrosis patients.

This study examined whether the increase in CO2 production (VCO2) and ventilatory demands by carbohydrate loading with different formulas during nighttime enteral feedings could be detrimental in young adult cystic fibrosis patients with moderate to advanced lung disease. Ten patients age 17 to 24 (mean 21.4 years) received 1000 kcal/M2 of a low (Pulmocare), medium (Ensure Plus), and high (Vivonex HN) carbohydrate formula in random order. Eight patients had severe, and two moderate obstructive pulmonary disease; nine used nighttime oxygen therapy. Basal energy expenditure (BEE) without feedings averaged 120% of that predicted by the Harris-Benedict equation. The metabolic expenditure by indirect calorimetry during nighttime feedings was 25 to 36% greater than the BEE. Oxygen consumption (VO2) increased 21 to 27% during nighttime feedings with no difference between formulas. VCO2 increased 29% for Pulmocare, 46% with Ensure Plus, and 53% with Vivonex HN. The increase in VCO2 with Pulmocare was significantly less than Ensure Plus (p less than 0.05) and Vivonex HN (p less than 0.005). The respiratory quotient (RQ) (VCO2-/VO2) for Pulmocare (0.88) was the same as the BEE, but increased with Ensure Plus (1.00), and Vivonex HN (1.08). The 41% increase in minute ventilation with Vivonex HN was greater than the 25 to 28% increase observed for Pulmocare and Ensure Plus (p less than 0.05). Transcutaneous oxygen saturation fell no more than 2% with all formulas. PCO2 changed +/- 5 torr during enteral feedings with similar changes in any patient with all formulas.(ABSTRACT TRUNCATED AT 250 WORDS)

Efficacy of ciprofloxacin in patients with cystic fibrosis.

Twenty-five courses of oral ciprofloxacin were administered to 16 patients experiencing pulmonary exacerbations of cystic fibrosis in an open, uncontrolled trial of efficacy and tolerance. Patients were treated for 7-35 days and efficacy was evaluated based on changes in clinical score, white blood cell count, quantitative bacteriology of sputum, and pulmonary function tests. Most courses of therapy were associated with a positive response with changes in clinical score and forced expiratory volume in one second being statistically significant (p less than 0.05). No side effects to ciprofloxacin were noted. Emergence of bacterial resistance to ciprofloxacin was rare. Ciprofloxacin appears to be effective in patients with cystic fibrosis who are experiencing pulmonary exacerbations associated with susceptible bacterial pathogens.

Clinical manifestations of exacerbations of cystic fibrosis associated with nonbacterial infections.

The purpose of this study was to determine whether acute pulmonary exacerbations of cystic fibrosis associated with nonbacterial infections are clinically distinguishable from other exacerbations. Eighty exacerbations in 54 patients were studied. Exacerbations associated with influenza (n = 8) were compared with those associated with other nonbacterial infections (n = 15) and those in which no nonbacterial infection was detected (n = 57). Patients with influenza had lower Shwachman scores and were more likely to be seropositive for C-reactive protein than patients in the other two groups. Patients with influenza had a mean decrease in forced expiratory volume per second of 26%, compared with test results obtained before the exacerbation. In contrast, the mean decrease in forced expiratory volume per second was 6% for other nonbacterial infections and 12% for the group without nonbacterial infection (p less than 0.05 for both comparisons). The forced expiratory flow in first 25% of vital capacity decreased 44% in the influenza group compared with 13% and 17% in the other two groups, respectively (p less than 0.01 for both comparisons). The influenza group also had a higher proportion of patients with at least a 20% decrease in forced expiratory volume per second and forced expiratory flow in first 25% of vital capacity than the other two groups had (p less than 0.05 for all comparisons). These data suggest that influenza is associated with severe exacerbations in patients with cystic fibrosis and support recommendations for efforts to prevent influenza in this population.

The use of extracorporeal rewarming in a child submerged for 66 minutes.

A 2 1/2-year-old girl had a good neurologic recovery after submersion in cold water for at least 66 minutes; as far as we know, this is the longest time ever reported. Cardiopulmonary resuscitation was maintained for more than two hours before the initiation of extracorporeal rewarming in this child who had a core temperature of 19 degrees C. To our knowledge, this is the first successful use of extracorporeal rewarming in a child suffering from accidental hypothermia. Extension of this technique to children offers rapid rewarming and cardiovascular support for pediatric victims of severe hypothermia. We emphasize the importance of a coordinated response by the entire emergency medical system integrated with hospital-based personnel. Where it is geographically feasible, regionalization of triage and care for the pediatric victim of severe accidental hypothermia should be considered.