Video-assisted evacuation of empyema is the preferred procedure for management of pleural space infections.

BACKGROUND: Empyema remains a cause of morbidity and mortality. Thoracoscopy has proved its versatility in the management of pleural space disorders. The suitability of video-assisted thoracic surgery (VATS) for decortication in the management of the fibrotic stage of empyema is unclear. METHODS: VATS evacuation of empyema and decortication was performed on seventeen patients presenting with pleural space infections. A retrospective review was performed and constitutes the basis of this report. RESULTS: VATS evacuation of empyema and decortication was successfully performed in 13 of 17 patients. Blood loss was 325 +/- 331 cc. Mean hospital stay was 18 +/- 10 days. Postoperative hospitalization was 11 +/- 7 days. Chest tubes remained in place for 7 +/- 3 days. There were no operative mortalities. CONCLUSIONS: Video-assisted evacuation of empyema and decortication is an effective modality in the management of the exudative and fibrinopurulent stages of empyema. An organized empyema should be approached thoracoscopically, but may require open decortication.

Effects of standard and high doses of salmeterol on lung function of hospitalized patients with cystic fibrosis.

In a recent investigation we found that hospitalized patients with cystic fibrosis who received 0.5 cc of 0.5% albuterol nebulizer solution TID significantly increased their pulmonary function across the day, but fell back to baseline overnight. To determine whether this fall could be prevented by the long-acting beta-2 agonist salmeterol at both standard (2 puffs: 42 mcg BID) and high (4 puffs, 84 mcg BID) doses, we evaluated the effects of salmeterol vs. albuterol (2 puffs, 180 mcg QID, and 4 puffs, 360 mcg BID) in a placebo-controlled three-way random crossover, double-blind trial. Eighteen patients in the low-dose group and 10 of the same 18 patients in the high-dose group completed the 3 consecutive days of testing and received either salmeterol, albuterol, or placebo with each of four chest physiotherapy sessions given at 7 AM, 11 AM, 3 PM, and 7 PM. At standard doses (2 puffs), the mean percent changes in FEV1 pre- to post-7 AM therapy for salmeterol (5.5%) and albuterol (9.9%) were significantly greater than with placebo (-1.2%) (P < 0.05 and 0.01, respectively). The mean percent changes in FEV1 from morning baseline with salmeterol were also significantly greater than placebo before 3 PM (12.1% vs. 5.4%, P < 0.01), and neither albuterol nor salmeterol were significantly greater than placebo after 3 PM. At standard doses there was a significant carryover effect with salmeterol to the next morning for the FEV1 (7.3%) when compared to placebo (1.5%) and albuterol (-0.7%) (P < 0.05 and 0.05, respectively). At high doses (4 puffs), the mean percent change in FEV1 with pre- to post-7 AM therapy increased to 22.7% and remained significantly greater than with placebo until pretherapy at 7 PM. The carryover effect the next morning was 14.7%. Salmeterol at 4 puffs compared favorably to albuterol nebulizer therapy given TID in both the incidence of responders for the FEV1 (70% vs. 71%) and the mean changes after therapy at 7 AM (22.7% vs. 14.9%), and provided greater carryover effects to the next morning (14.7% vs. -0.7%), thus preventing the fall in pulmonary function back to baseline overnight. We recommend the use of high-dose salmeterol in hospitalized patients with FVC values of 40% of predicted or greater, starting with 2 and increasing to 4 puffs BID as tolerated.

Anabolic effect of biosynthetic growth hormone in cystic fibrosis patients.

The purpose of this study was to determine whether GH treatment of cystic fibrosis (CF) patients can result in an anabolic effect, i.e., increased weight gain, improved growth rate, nitrogen retention, and improved pulmonary function. Nine prepubertal endocrinologically normal CF patients (3 girls, 6 boys; chronological age (CA) 5.5-9.8 years, and bone age (BA) 4.5-9.0 years), received recombinant human growth hormone (rhGH) 0.3 mg/kg/week subcutaneously for a period of 12 months (N = 8) or 9 months (N = 1). Normal glucose tolerance was determined before treatment. Pulmonary function studies and anthropometric measurements were done every 3 months. Thyroid status, somatomedin C (SmC), BA, and routine chemistries were evaluated every 6 months. The pretreatment growth velocity averaged 5.7 +/- 0.3 (SE) cm/year and significantly increased to 7.8 +/- 0.4 (SE) cm/year during therapy, (P < 0.01). Standard deviation scores (SDS) for height significantly increased during rhGH therapy as compared with pretreatment, (P < 0.05). Weight of the patients during rhGH therapy did not significantly change during or after rhGH therapy. After therapy, all patients showed a significant increase in arm muscle area (AMA) and a significant decrement in arm fat area (AFA) (P < 0.01). Net nitrogen anabolism was negative in all subjects before therapy but became more positive in five patients during rhGH therapy. Three patients achieved positive nitrogen retention. SmC values significantly increased from a mean value of 0.62 +/- 0.1 (SE) U/ml to 1.6 +/- 0.6 (SE) U/ml after therapy. BA advanced 1.0 +/- 0.1 SE per year after treatment. Of the seven patients able to perform adequate pulmonary function testing, improvement occurred in FVC, FEV1.0, and PEFR in 5, 5, and 4 patients, respectively, but these changes did not reach statistical significance. We conclude that biosynthetic rhGH therapy had a significant anabolic effect in CF patients as shown by increased growth velocity, SmC values, increased protein and decreased fet stores, and a positive or less negative net nitrogen retention in five of the patients.

The effects of albuterol on the lung function of hospitalized patients with cystic fibrosis.

Twenty-four hospitalized patients with cystic fibrosis were enrolled into a 2-d, double-blind, placebo-controlled, randomized crossover trial comparing albuterol inhalation aerosol with a saline placebo. Aerosols were administered with the first three of four chest physiotherapy sessions given 4 h apart. Spirometry was measured before and 45 min after 7:00 A.M. and 3:00 P.M. therapy and before therapy at 7:00 P.M. and 7:00 A.M. the next morning. The mean percent change in FVC, FEV1, and FEF25-75% at 7:00 A.M. was 10.7, 14.8, and 19.6% with albuterol versus 2.4, 1.0, and -0.8% with placebo (p = 0.0012, < 0.0001, and = 0.003, respectively). A greater than 8% change in FEV1 separated changes with albuterol versus placebo with 96% specificity and occurred in 75% of all patients with albuterol; 71% at 7:00 A.M. versus 24% at 3:00 P.M. The reduction in response at 3:00 P.M. (p < 0.01) was presumably due to prolonged effects of morning therapy ( > 4 h). Individual changes in spirometry were significantly more positive and homogeneous with albuterol versus placebo at both 7:00 A.M. and 3:00 P.M. The mean percent change for the FVC, FEV1, and FEF25-75 across the day (7:00 A.M. pretherapy to 7:00 P.M. pretherapy) was 8.1, 10.1, and 9.7% with albuterol versus 3.9, 3.5 and 2.6% with placebo (p = 0.029, 0.036, and 0.232, respectively). The more positive and homogeneous changes in spirometry with albuterol, along with greater changes in these measures across the day when compared with placebo, suggest that albuterol improves pulmonary function in a majority of hospitalized patients with cystic fibrosis.

Clinical denouement and mutation analysis of patients with cystic fibrosis undergoing liver transplantation for biliary cirrhosis.

OBJECTIVE: To describe the clinical characteristics of patients with cystic fibrosis considered for liver transplantation and the clinical outcome after transplantation. METHODS: Patient charts were reviewed. Mutation analysis was performed on blood or liver tissue samples with a panel of 17 mutations. RESULTS: Eight patients (five girls) with cystic fibrosis have undergone orthotopic liver transplantation for biliary cirrhosis. Mean age at transplantation was 12.0 years +/- 7.7 years (range, 9 months to 23 years). Preoperatively, seven patients had mild to moderate pulmonary dysfunction and one moderate to severe pulmonary dysfunction. All patients required pancreatic enzyme replacement, and four patients required insulin for diabetes mellitus. The 1-year survival rate was 75%, with no deaths related to septic events. Mean time of follow-up the six operative survivors was 4.1 years +/- 1.9 years. Pulmonary function testing, in those serially tested, showed that forced expiratory volume in 1 second was maintained or improved and that forced vital capacity improved after transplantation. Mutation analysis showed the following genotypes: four patients, delta F508/delta F508; one patient, delta F508/N1303K; and three patients, delta F508/unknown. CONCLUSIONS: Despite the high risk of transplantation, these encouraging results indicate that liver transplantation should be considered for patients with cystic fibrosis and complications of end-stage liver disease. We could not demonstrate an unusual pattern of CF gene mutations in these patients with severe liver disease. It appeared that immunosuppressive agents did not have a deleterious effect on pulmonary function.

Fatal eosinophilic disease following autologous bone marrow transplantation.

A 15-year-old girl developed massive, fatal eosinophilic disease following autologous bone marrow transplantation (BMT) for Hodgkin's disease (HD). Prior to autologous BMT, the erythrocyte sedimentation rate (ESR) was elevated, with active HD, but eosinophilia was absent. Post-autologous BMT, ESR and peripheral eosinophilia were observed to correlate with respiratory symptoms. Initial evaluation revealed no recurrent tumor, infection or other identifiable etiology. A diagnosis of chronic eosinophilic pneumonia was made following lung biopsy. A complete response was initially achieved with steroid therapy; however, when steroid therapy was tapered, the eosinophilia and elevated ESR recurred with worsening respiratory symptoms. Terminally, severe pulmonary disease developed and recurrent HD was found in lung, lymph nodes and bone marrow. During episodes of eosinophilia, the patient's serum stimulated her bone marrow as well as control marrow to produce predominantly eosinophilic colonies. Eosinophilic colony production was not observed with patient's sera obtained prior to or during autologous BMT or with control sera. This patient died of eosinophilic inflammatory disease following autologous BMT. The etiology of this disease was not definitely identified but appeared to be due to an eosinophilic-stimulating factor which developed after autologous BMT.

Unusual form of endobronchial Aspergillosis in a patient with cystic fibrosis.

The isolation of Aspergillus fumigatus from airway secretions from patients with cystic fibrosis (CF) is common and usually denotes asymptomatic colonization or allergic broncho-pulmonary aspergillosis (ABPA). A 12-year-old boy with CF acutely developed moderately severe symptoms of unremitting cough, fever, dyspnea, weight loss, and cyanosis. Chest radiographs demonstrated widespread unilateral infiltrates and volume loss. By bronchoscopy tenacious mucous plugs were seen occluding the left lower lobe bronchus. Cultures from sputum and sequential bronchoalveolar lavage grew Aspergillus fumigatus, but other significant criteria for diagnosing ABPA were lacking. No improvement was seen with a 3 week course of systemic corticosteroid and antibiotic therapy. Treatment with amphotericin B and short-term mechanical ventilation resulted in rapid resolution of all symptoms. This form of endobronchial aspergillosis has not been described previously.

Mutation analysis and haplotype correlation for 139 cystic fibrosis patients from the Nebraska Regional Cystic Fibrosis Center.

Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasian populations with an approximate frequency of one in 2,500 live births and a carrier frequency of one in 25. We studied 400 individuals seen at The Nebraska Regional Cystic Fibrosis Center that included 139 CF patients, 206 parents, and 55 unaffected siblings to determine the frequency of the delta F508, R117H, G542X, S549R/N, G551D, R553X, R560T, and W1282X mutations. In addition, we determined haplotypes on each of these individual's chromosomes using four markers that included XV-2c, KM-19, pMP6d.9, and G2. Results from this study showed that the delta F508 mutation was present in 70% of CF chromosomes. Of the 139 CF patients 74 (53%) were homozygous for the delta F508 deletion, 47 (34%) were heterozygous for the delta F508 deletion and an unknown mutation, and 18 (13%) carried two unknown mutations. Four additional mutations were also found in our population and included G542X (6%), G551D (5%), R553X (4%), and R560T (1%). One patient was documented to be a compound heterozygote for G542X/G551D. A polymorphism, F508C, that has previously been reported in several families was also present in our study. The most common haplotype associated with the delta F508 deletion in our CF patients was the E haplotype (CF Consortium B) while other mutations were associated with a variety of haplotypes.

The spectrum of Pneumocystis carinii infection after liver transplantation in children.

We report the experience with Pneumocystis carinii lung infections in the 109 children undergoing liver transplantation at our hospital between August, 1985 and May, 1989. PCP developed in 9 of the 86 patients (10%) surviving > or = 6 weeks after transplantation and not receiving P carinii chemoprophylaxis. Of the 59 patients undergoing BAL 2 or more weeks after transplantation there were 16 specimens from 14 patients (24%) positive for P carinii. These patients had a spectrum of illness ranging from asymptomatic to severe pneumonia requiring mechanical ventilation. The mean interval from first transplantation to bronchoalveolar lavage positive for P carinii was 24.9 weeks and the mean interval to first PCP was 28.0 weeks. The earliest and latest occurrences of PCP were 7 weeks and 73 weeks, respectively, after transplantation. There were no complications attributed to BAL.

Time course of lipid-laden pulmonary macrophages with acute and recurrent milk aspiration in rabbits.

High levels of lipid-laden macrophages (LLM) in bronchial washings have been associated with food aspiration. We studied the time course of appearance and clearance of LLM in rabbits undergoing either a single milk instillation, five weekly milk instillations or saline (control) instillations into the airways. Cells were obtained by bronchoalveolar lavage of intubated rabbits at uniform time intervals following the single or the last of five milk/saline instillations. LLM semi-quantitative indexes were derived using oil-red-O staining. Significantly elevated indexes were found in both milk groups 6 hr after milk instillation. In the single saline and milk instillation groups the indexes were not different beginning on the 4th day, and indexes from 8 of 9 rabbits had returned to baseline by the 6th day. However, indexes remained significantly elevated up to 17 days in the group receiving weekly milk instillations. Indexes from all rabbits in the repeat milk instillation group remained elevated for 12 days or longer. This group also developed increased numbers of binucleated macrophages. Quantitation of LLM in this model appears to be a sensitive indicator of recurrent lipid aspiration, these cells remaining in the airways for several days after the last aspiration event.

Bronchoscopic evaluation of airway obstruction in campomelic dysplasia.

Campomelic dysplasia is a generalized disorder of cartilaginous growth and development, leading to early death from pulmonary insufficiency. We describe the airway dynamics as observed bronchoscopically in two affected infants. Both infants demonstrated anatomic compromise of the upper airway and diffuse laryngotracheobronchomalacia. Additionally, both had a characteristically small, bell-shaped thoracic cage. The abnormal airway dynamics produced serious inspiratory and expiratory obstruction in these infants and, in combination with the restrictive chest wall defect, led rapidly to the development of respiratory failure. While palliative procedures such as tracheostomy may temporarily improve airway dynamics, future respiratory tract insults may prove fatal.