Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0006271 (bronchiolitis)
 5,174 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have characterised nitric oxide (NO) synthase activity in lung samples from patients with inflammatory lung disease compared to that in normal donor lung. NO synthase activity was measured by the ability of tissue homogenates to convert L-arginine to L-citrulline. Higher levels of NO synthase activity were found in samples from patients with inflammatory lung disease (mild asthma, cystic fibrosis, obliterative bronchiolitis after lung transplantation) compared to samples from healthy donors. NO synthase activity in all samples was mainly dependent on the presence of extracellular Ca2+. The increased NO synthase activity in diseased-lung samples suggests a modulatory role for nitric oxide in lung inflammation.
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PMID:Nitric oxide synthase activity is elevated in inflammatory lung disease in humans. 749 18

As lung transplantation has become more successful, the selection criteria have broadened; however, some relative contraindications to lung transplantation are controversial. Some programs consider mechanical ventilation to be a major contraindication to lung transplantation because airway colonization with bacteria may lead to nosocomial infection and respiratory muscle deconditioning may necessitate prolonged postoperative ventilatory support. We report our experience of seven double lung transplant procedures on six patients requiring mechanical ventilation. Five patients with cystic fibrosis required preoperative mechanical ventilation for 7 to 19 days (mean, 10.7 days). One patient with acute lung injury required 115 days of preoperative mechanical ventilatory support. Only the latter patient required prolonged (27 days) postoperative mechanical ventilation because of respiratory muscle weakness; the others were extubated in 1 to 19 days (mean, 7.8 days). No early complications related to bacterial infection were seen. Two patients required temporary hemodialysis for transient kidney failure. Three patients had postoperative neurologic residua; one patient had a transient hemiparesis, and seizures developed in two patients. One patient died 3 months after transplantation from severe central nervous system complications with no evidence of pulmonary problems; and two patients died 17 months after transplantation, one of them receiving a second double lung transplant for obliterative bronchiolitis. Except for the patient who required prolonged preoperative ventilatory support, mechanical ventilation did not appear to play a role in the outcome of these patients. The posttransplantation hospital stay and hospital charges for patients requiring pretransplantation ventilatory support were not significantly different from those for other lung transplant recipients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Lung transplantation for mechanically ventilated patients. 751 85

It has been suggested that the presence of airway pathogens prior to lung transplantation (LT) in patients with cystic fibrosis (CF) may place these patients at a higher risk for infectious complications after LT. There is particular concern regarding patients colonized with multiresistant Pseudomonas, including P. cepacia, and fungi, including Aspergillus. We report our experience with LT for patients with CF and compare the results with those of patients with LT for other indications. Between January 1990 and March 1993, we performed LT for 27 patients with CF and 32 without CF. Nearly all (89%) of the patients with CF were colonized with P. aeruginosa; many were cultured with P. cepacia (19%) and Aspergillus (63%). The non-CF group rarely had organisms identified pre-LT. No patients with CF underwent pre-LT sinus drainage or received pre-LT treatment for Aspergillus. All of the patients received perioperative antibiotics and a standard regimen of immunosuppression and prophylactic antibiotics. The incidence of infectious complications was the same in the two groups; however, there was an association between obliterative bronchiolitis and pulmonary infections. One of the patients with CF with P. cepacia died as a result of this organism. None of the patients with CF required treatment for Aspergillus post-transplant. We conclude that patients with CF, despite the presence of airway pathogens, are at no greater risk of infectious complications after LT than are other patients.
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PMID:Infectious complications of lung transplantation. Impact of cystic fibrosis. 751 51

Patients with cystic fibrosis pose particular challenges for lung transplant surgeons. Earlier reports from North American centers suggested that patients with cystic fibrosis were at greater risk for heart-lung or isolated lung transplantation than other patients with end-stage pulmonary disease. During a 3 1/2 year period, 44 patients with end-stage lung disease resulting from cystic fibrosis underwent double lung transplantation at this institution. During the same interval, 18 patients with cystic fibrosis died while waiting for lung transplantation. The ages of the recipients ranged from 8 to 45 years, and mean forced expiratory volume in 1 second was 21% predicted. Seven patients had Pseudomonas cepacia bacteria before transplantation. Bilateral sequential implantation with omentopexy was used in all patients. There were no operative deaths, although two patients required urgent retransplantation because of graft failure. Cardiopulmonary bypass was necessary in six procedures in five patients and was associated with an increased blood transfusion requirement, longer postoperative ventilation, and longer hospital stay. Actuarial survival was 85% at 1 year and 67% at 2 years. Infection was the most common cause of death within 6 months of transplantation (Pseudomonas cepacia pneumonia was the cause of death in two patients), and bronchiolitis obliterans was the most common cause of death after 6 months. Actuarial freedom from development of clinically significant bronchiolitis obliterans was 59% at 2 years. Results of pulmonary function tests improved substantially in survivors, with forced expiratory volume in 1 second averaging 78% predicted 2 years after transplantation. Double lung transplantation can be accomplished with acceptable morbidity and mortality in patients with cystic fibrosis.
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PMID:Improved results of lung transplantation for patients with cystic fibrosis. 753 96

The discovery of the cystic fibrosis gene has provided new approaches to gene therapy and drug therapy. At the present time, however, we are still faced with the morbidity and mortality due to lung infections caused by Pseudomonas aeruginosa and Pseudomonas cepacia. The present survey of some of the recently published articles shows that applied science related to the clinical care of cystic fibrosis (CF) patients is also progressing. Cross-infection in CF centers and during social contacts between CF patients out of the hospital environment has been described in some countries. Prophylactically, cohort isolation procedures are effective. The individual clinical course of the chronic pulmonary infection is adversely influenced during prediabetic mellitus periods and by development of high titers of specific IgG2 and IgG3 antibodies against P. aeruginosa. A vaccine against P. aeruginosa is currently being tested in CF patients. The induced antibodies have much higher affinity and opsonophagocytic activity than infection-induced antibodies. Ciprofloxacin, which is widely used in CF, easily gives rise to resistance in P. aeruginosa and Staphylococcus aureus. Restricting the use of this drug in CF is therefore necessary. Aerosolized recombinant DNase is a promising and efficient new drug for management of airflow limitation due to purulent airway secretion in CF. For end-stage CF, double-lung and heart-lung transplantation show encouraging intermediate-term results, the two main obstacles being donor organ shortage and obliterative bronchiolitis.
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PMID:Cystic fibrosis and endobronchial pseudomonas infection. 769 Jun 37

Between November 1992 and May 1994 we performed 10 single and 5 double lung transplants in patients with end-stage lung diseases due to lymphangioleiomyomatosis (4), cystic fibrosis (3), pulmonary hypertension (3), pulmonary fibrosis (3) and chronic obstructive lung disease (2). In the 13 patients (87%) surviving for median 245 (19-567) days, FEV1 improved from median 640 ml to 1410 ml and the 12-minute walk distance from median 315 to 1100 meters. 10 patients (77%) enjoy a good or even excellent quality of life. 2 patients died 11 and 62 days postoperatively, due to multi-organ failure and invasive pulmonary aspergillosis respectively. The main postoperative problems are fungal and cytomegalovirus infections and chronic rejection in the form of bronchiolitis obliterans. In Switzerland as elsewhere, lung transplantation has become an established modality for the management of end-stage diseases of the lung and pulmonary circulation.
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PMID:[Isolated lung transplantation--evaluation of patients and initial results]. 773 52

We assessed the long-term results of our experience with 109 patients with end-stage cardiopulmonary disease who underwent primary combined heart-lung transplantation at Stanford University Medical Center between March 1981 and January 1994. Average recipient age was 31 +/- 10 years (mean +/- standard deviation) median, 31 years; range, 1 month to 52 years. Recipient diagnoses included primary pulmonary hypertension (31%), Eisenmenger's syndrome (39%), complex congenital heart disease (8%), cystic fibrosis (14%), bronchiectasis (2%), and emphysema (3%). Immunosuppression was with cyclosporine and a tapering regimen of corticosteroids. In 1986 azathioprine was added, and since 1987 induction therapy with OKT3 has been employed. Actuarial survival rates at 1, 5, and 10 years were 68% +/- 4.6%, 43% +/- 5.4%, and 23% +/- 8.1%, respectively (mean +/- 1 standard error of the mean). Fourteen deaths occurred in the hospital for an operative mortality rate of 12.8% +/- 3.3%, and 61 deaths occurred overall. Causes of death included hemorrhage (five patients), infection (21), rejection (one), nonspecific pulmonary failure (four), graft coronary artery disease (six), and obliterative bronchiolitis (eight). Infection, rejection, and obliterative bronchiolitis were the major complications. Only 20% +/- 3.9% of patients were free from any infection 3 months after transplantation. Heart and lung rejection commonly occurred asynchronously; actuarial estimates of freedom from isolated lung rejection at 1 and 5 years were 47% +/- 5.2% and 40% +/- 5.6%, respectively. For simultaneous heart and lung rejection these estimates were 87% +/- 3.5% and 86% +/- 3.8%, and for isolated heart rejection 63% +/- 5.1% and 51% +/- 6.4%, respectively. Although graft coronary artery disease developed less frequently than in patients after isolated heart transplantation (90% +/- 4.6% of patients were free of graft coronary artery disease at 5 years), obliterative bronchiolitis remains a major long-term complication and cause of morbidity and mortality. Actuarial estimates of freedom from obliterative bronchiolitis at 1, 5, and 10 years were 71% +/- 5.1%, 51% +/- 6.1%, and 42% +/- 7.8%, respectively. These results show satisfactory early and medium-term outcome after combined heart-lung transplantation but also underscore that much progress is needed in controlling infection, rejection, and obliterative bronchiolitis, all of which remain as major impediments to long-term survival.
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PMID:Long-term results of combined heart-lung transplantation: the Stanford experience. 786 27

Lung transplantation is a successful alternative treatment for a variety of end-stage lung diseases. The first 20 lung transplants performed in Louisiana between November 1990 and July 1994 are reported from Ochsner Foundation Hospital. Transplant procedures included 1 heart-lung, 11 bilateral sequential lung, and 8 single-lung transplants in 8 males and 11 females (1 retransplantation). The average age was 38 years (range 7-60), and the median waiting time was 34.5 days (range 1-329). Indications for transplant included emphysema, pulmonary fibrosis, pulmonary hypertension, cystic fibrosis, bronchiectasis, and bronchiolitis obliterans. Overall 1-year and 3-year survival were 65.0% and 58.5%, respectively. Infection was the major cause of morbidity and mortality. Rejection episodes were observed but treated successfully in all 20 patients. Lung transplantation has proved to be a successful treatment for a variety of severely limiting and terminal pulmonary conditions for patients in our state.
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PMID:Lung transplantation in Louisiana: report of the first twenty lung transplants performed in the state. 789 Oct 1

Air compressors for inhalation devices were equipped with counters which were activated whenever the inhalation was started. They recorded the time of the activated inhaler. The counters were invisible for the parent and the doctor. 40 outpatients, all children, were randomly given such a device for treatment at home under surveillance of their parents. The duration, dose and frequency of inhalation was explained, and the parents were instructed that if the child should not be well, the inhalation frequency could be doubled. 21 children (age: 3.8 +/- 3.7 years from two months to 14 years), five girls, 16 boys, were on the inhalation devices equipped with the counter; ten had asthma, four acute bronchitis, four mild bronchiolitis, one had pertussis, one cystic fibrosis and one pneumonia. The prescribed inhalation treatment extended over 21 +/- 14 days (3 to 50), the inhalation frequency per day was one to three times. The measured inhalation unit (recorded inhalation time times frequency) amounted to 4.87 +/- 5.01 vs. 4.31 +/- 3.58 (n.s.) for the expected unit (expected inhalation units according to the prescription). The maximum and the minimum differences for the measured vs. the expected unit were significantly different (p < 0.001). Ten children inhaled 80 to 120% of the prescribed inhalation units, six children inhaled more than 140%, and five children less than 70% of the prescribed units. This resulted in an overall inhalation compliance of 47.6%. This is the first double-blind study carried out so far studying inhalation compliance in children with respiratory diseases.
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PMID:[Compliance with inhalation therapy in children with respiratory diseases]. 790 18

Donor scarcity and the limitations of organ preservation dictate that a wide size range be used to maximize effective donor utilization in pulmonary transplantation. Problems of size disparity are magnified in the pediatric population, where relevant dimensions vary considerably by age. There have been 10 pediatric lung recipients (7 bilateral, 2 single, 1 heart-lung) at our institution since 1991. The age range was 3 to 26 years (mean, 13.7), and the weight range was 15 to 57 kg (mean, 33.6). Diagnoses included cystic fibrosis (5), pulmonary fibrosis (2), pulmonary hypertension (2), and arteriovenous malformation (1). The donor-to-recipient weight ratio ranged from 0.45 to 1.9, and the donor-to-recipient thoracic height ratio ranged from 0.6 to 1.6. Lobar resection and delayed chest closure was required in one case and was successful. Cardiopulmonary bypass was used electively in seven cases. No reexplorations for bleeding were necessary. Two of the three hospital deaths resulted from right ventricular failure, judged to be secondary to excessive pulmonary artery pressures intraoperatively associated with small donor allografts. Elective bypass had not been used. Inhaled nitric oxide (6 patients) and/or extracorporeal membrane oxygenation (2 patients) were used for temporary postoperative support. The hospital survival rate was 70% (7/10). One late death occurred 14 months postoperatively and was caused by obliterative bronchiolitis. The authors conclude that size disparity is a significant problem in pediatric lung transplantation. However, with elective use of cardiopulmonary bypass and aggressive postoperative support, a broad size range can be used.
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PMID:Size disparity in pediatric lung transplantation. 796 25


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