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

Heart-lung transplantation for cystic fibrosis has become an established form of treatment over the last few years. This paper considers the assessment and preparation of patients, the surgical procedure, immunosuppression, post-operative care and the results of surgery. The best survival rates to 1 year are 70%-80% with possibly a less favourable survival for younger patients. Major problems include shortage of donor organs, post-operative management of patients with multisystem disease and obliterative bronchiolitis. The majority of patients, however, do well with a greatly improved quality of life and the medium-term results are encouraging.
 ...
PMID:Heart-lung transplantation for cystic fibrosis. 134 5

Growing experience in terms of immunosuppression, recipient and donor selection as well as organ preservation has established thoracic organ transplantation as a therapeutic option for many children with end-stage cardiopulmonary diseases. While dilated cardiomyopathy and isolated myocardial failure represent the main indications for cardiac transplantation, replacement of the lungs or heart and lungs is necessitated in cystic fibrosis, primary and secondary pulmonary hypertension as well as some types of complex congenital heart defects involving the pulmonary arteries. We have performed a total of 20 heart, 4 heart-lung, 2 single lung and 1 double lung transplantation in the paediatric group up to 17 years of age. While with respect to the limited experience worldwide, early mortality after lung and heart-lung transplantation is still high (50%), long-term results in isolated cardiac transplantation using triple drug immunosuppression are excellent (79% survival after 6 years) without major impairment of renal function, arterial blood pressure, growth development and physical rehabilitation as well as social reintegration. Freedom from graft atherosclerosis of the allografted heart is documented over a 5 year follow up, while no data are available on the incidence of obliterative bronchiolitis after lung transplantation in the paediatric group. Despite only limited evidence of long-term dysfunction, diagnosis and prevention of chronic rejection should be given utmost attention to allow for a normal life span in this younger age group.
 ...
PMID:Thoracic organ transplantation in the paediatric age group. The Hannover experience. 134 7

Between September, 1984, and March, 1991, 79 patients underwent heart-lung transplantation for end-stage cystic fibrosis at the Harefield Hospital. Short-term outcome has already been reported, and we now present intermediate-term results. The overall actuarial patient survival was 69% at 1 year, 52% at 2 years, and 49% at 3 years. 17 patients had diabetes mellitus with a survival of 62% to 1 year and 51% to 2 years. 23 patients had one or more other possible high-risk factors, and survival of these patients was 64% at 1 year and 57% at 2 years, compared with 71% and 49%, respectively, in the low-risk group (n = 56). Pseudomonas aeruginosa infection was the most common respiratory infection encountered postoperatively. 92% of patients had at least one episode of acute rejection during the first 3 postoperative months. Lung function was greatly improved after transplantation, the mean forced expiratory volume in 1 s and forced vital capacity increasing from 22% and 35% predicted, respectively, preoperatively to 68% and 70% predicted, respectively, by the sixth postoperative month. This improvement was maintained at 1, 2, and 3 years after transplantation. Lymphoproliferative disorders (4 patients) were successfully treated. Obliterative bronchiolitis developed in 17 patients and the cumulative probability of getting this complication at 1, 2, and 3 years postoperatively was 17%, 23%, and 48%, respectively. Overall, 7 patients were retransplanted. There was no coronary artery disease in the 37 patients who underwent coronary angiography at 1 year, 14 at 2 years, and 9 at 3 years after surgery. 58 patients donated their hearts for subsequent "domino" heart transplantation. Our 5 1/2-year experience with heart-lung transplantation is encouraging but the shortage of donor organs and the complication of obliterative bronchiolitis are the two main obstacles to be overcome.
 ...
PMID:Intermediate-term results of heart-lung transplantation for cystic fibrosis. 135 59

Lung transplantation has emerged in the last decade as a valid therapeutic endeavor for patients with end-stage lung disease. Although the presence of highly resistant organisms in the airway and sinuses of patients with cystic fibrosis (CF) increases the perioperative risk of infection following lung transplantation, transplant procedures can be undertaken in properly selected CF patients with acceptable morbidity and mortality at experienced centers. The introduction of a modification in the technique of double-lung transplantation has resulted in improved operative survival of CF patients undergoing lung transplantation. Whereas problems with posttransplant obliterative bronchiolitis, infection, and lymphoproliferative disorders contribute to morbidity, it is clear that lung transplantation can offer CF patients with end-stage lung disease improvement in both the quality and quantity of life. The most serious impediment to more widespread application of this therapy in CF is the inadequate number of donor organs.
 ...
PMID:Lung transplantation in cystic fibrosis. 147 46

In spite of a shortage of available donors, an increasing number of heart-lung transplantations have been performed within the last decade. This procedure, first limited to patients with pulmonary vascular disease, has been successfully extended to patients with end stage lung disease, including cystic fibrosis. More recently, single lung, double-lung and bilateral single lung transplantation have become other therapeutic options. Better selection of patients and donors as well as improvements in surgical techniques and immunosuppression regimens have contributed to the reduction in the high perioperative mortality experienced in the early stages. Moreover, the introduction of daily spirometry and transbronchial lung biopsies have permitted early and reliable diagnosis of opportunistic infection and rejection. The most serious late complication of lung transplantation is obliterative bronchiolitis and further research is urgently required to improve diagnosis and management of this condition.
 ...
PMID:Lung allograft transplantation: indications, preoperative assessment and postoperative management. 148 75

Cystic fibrosis is the most common lethal genetic disease occurring in the white population. It is estimated that 3.5% of the 20,000 individuals with cystic fibrosis in North America will die each year of end-stage lung disease. Lung transplantation (heart-lung or double lung) is becoming more frequent as more patients are referred for this procedure. Since January 1988, we have evaluated 60 patients with cystic fibrosis for lung transplantation and have accepted 30 (50%). Nine patients (30%) died while awaiting a donor. Fifteen patients underwent transplantation (13 heart-lung and two double lung procedures). Actuarial survival at 1, 2, and 3 years is 76%. All survivors are without physical limitations. Pulmonary function, as determined by forced vital capacity, forced expiratory volume in 1 second, and arterial blood gas determinations, is within the normal range. Comparing these data with those of a group of patients without cystic fibrosis who underwent transplantation during the same period did not reveal any significant differences with respect to infection, rejection, and outcome. Preliminary data suggest that obliterative bronchiolitis is less prevalent at 1 year in patients with cystic fibrosis (19%) than in those without cystic fibrosis (41%). Patients with cystic fibrosis present a number of challenges. The problems of pleural adhesions from repeated infections, pleurodesis, and previous thoracic procedures are now readily approached through the bilateral thoracosternotomy (clam shell) incision. Insulin-dependent diabetes mellitus and low-dose corticosteroid therapy are no longer considered absolute contraindications. Both septic lungs must be removed at operation, either with heart-lung transplantation or with double lung transplantation. These data support the therapeutic efficacy of lung transplantation for patients with cystic fibrosis.
 ...
PMID:Cystic fibrosis. Target population for lung transplantation in North America in the 1990s. 156 53

We report two cases of allergic bronchopulmonary fungal disease (ABPFD) caused by Curvularia sp and associated with allergic fungal sinusitis (AFS). Curvularia lunata was cultured in one case and Curvularia senegalensis was cultured in the other. Based on these cases and a review of the literature, we discuss unusual clinical and pathologic features that can occur in ABPFD. Unusual clinical aspects of ABPFD include associated AFS, absence of asthma, progression to Churg-Strauss angiitis and granulomatosis, concomitant hypersensitivity pneumonitis, and underlying cystic fibrosis. Atypical pathologic features that may occur in ABPFD include follicular bronchiolitis, xanthomatous bronchiolitis, limited tissue invasion, fungus balls, and association with unusual fungi. Prominent follicular bronchiolitis and xanthomatous bronchiolitis were misleading histologic features in one of our cases and led to a delay in recognition of the diagnosis. Both patients presented primarily with AFS; ABPFD was detected subsequently. This suggests that a small subset of patients with AFS may be at risk for ABPFD. The goal of this review is to increase awareness of unusual clinical and pathologic manifestations of ABPFD. It is hoped that this will result in accurate diagnosis and proper therapy, especially for patients who present with atypical features. Unusual fungal species should be considered in patients who have clinical findings compatible with ABPFD but who do not demonstrate immunologic reactivity to Aspergillus sp, especially Aspergillus fumigatus. In addition, ABPFD should be considered in patients with AFS who develop new pulmonary lesions.
 ...
PMID:Unusual aspects of allergic bronchopulmonary fungal disease: report of two cases due to Curvularia organisms associated with allergic fungal sinusitis. 174 30

We recently described a technique for bilateral sequential lung transplantation that replaces the en bloc double-lung operation, a procedure that was accompanied by frequent problems with airway healing. Twenty-seven patients have undergone 28 bilateral sequential lung transplantations over the past 14 months. Eighteen patients had transplantation because of end-stage emphysema; 6, cystic fibrosis; and 1 each, obliterative bronchiolitis, usual interstitial pneumonitis with pulmonary fibrosis, and bronchiectasis. Cardiopulmonary bypass was used electively in the first 5 patients until it was recognized that the procedure could be done safely without it, and in only 3 additional recipients has it been employed. Mean ischemic time for the first lung was 276 +/- 43 minutes and for the second lung, 410 +/- 64 minutes. There have been five deaths, three in the postoperative period (11% operative mortality) and two late. The other patients are alive and well and do not require oxygen 2 to 15 months after transplantation. Mean forced expiratory volume in 1 second rose from 16% +/- 8% of predicted to 84% +/- 17% at 12 weeks. Six-minute walk values increased from a mean of 251 +/- 91 m to 666 +/- 42 m at 24 weeks. The excellent exposure afforded to both hemithoraces by the thoracosternotomy incision and the rare need of cardiopulmonary bypass have allowed us to offer the option of transplantation to patients who formerly would have been turned down because of previous pulmonary resection or pleurectomy. On four occasions, ventilator-dependent patients underwent successful transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Bilateral sequential lung transplantation: the procedure of choice for double-lung replacement. 189 30

From March 1988 to March 1990, 11 children with cystic fibrosis (age 5-15 years) underwent combined heart-lung transplantation at our institutes. Maintenance immunosuppression consisted of cyclosporin and azathioprine with corticosteroids and antithymocyte globulin used perioperatively and during rejection episodes. Six patients (55%) survive from 1.5-23 months all of whom have improved life quality. Actuarial survival to 1 year was 55%. At six months after transplant, mean forced expiratory volume at one second was 73.5% of predicted normal, compared with 25% before transplant. There was one perioperative death, three later deaths associated with obliterative bronchiolitis at two, eight, and nine months, and one from mediastinitis at four months. Of the 15 children accepted for transplantation but not receiving grafts, 10 have died (eight within four months of being placed onto the transplant list). Early postoperative problems included acute reversible rejection (n = 10), meconium ileus equivalent (n = 3), and pancreatitis (n = 1). There was a high incidence of later pulmonary rejection with a mean of 5.7 episodes per patient in the first six months. Pulmonary infection occurred relatively infrequently, with Pseudomonas aeruginosa being the most common pathogen. Persistent diabetes mellitus requiring insulin occurred in four and systemic hypertension developed in one.
 ...
PMID:Heart-lung transplantation for cystic fibrosis. 2: Outcome. 192 6

Gastroesophageal reflux (GER) usually presents with digestive symptoms, failure to thrive, and/or respiratory symptoms. During the 8-year period from 1981 to 1989, 1,153 children underwent 20-hour pH monitoring to assess GER. All patients were graded using the scoring system of Euler and Byrne. Patients were classified as severe (score greater than 50), moderate (score 25 to 50), and normal (score less than 25). Five hundred (43.3%) of these patients presented with respiratory symptoms including apnea, cyanosis, or "near miss" sudden infant death syndrome (36%), poorly controlled asthma (28%), recurrent bronchopneumonia (13%), bronchiolitis (9%), and miscellaneous symptoms such as intermittent dyspnea, chronic cough, and stridor (12%). Eight patients (2%) had cystic fibrosis. The ages ranged from 1 month to 20 years (mean, 19.5 months). Twelve patients had technically inadequate studies and were excluded. Severe reflux was present in 156 patients (31%) and moderate reflux in 159 patients (31%). All patients were treated initially by medical therapy for a minimum of 8 weeks. The majority of patients (81%) had resolution of their symptoms with change in position, thickened feedings, and, when indicated, additional therapy with metoclopramide, cisapride, or domperidone. Most of these patients were found to have a specific position, usually prone, which decreased reflux. The remaining 57 patients had documentation of persistent reflux by pH monitoring and underwent an antireflux procedure. Of those patients undergoing surgery 51 had severe reflux and 6 had moderate reflux. Forty-four patients had a posterior 270 degrees wrap (Toupet), 10 had a 360 degrees wrap (Nissen), and 3 had an anterior 180 degrees wrap (Boix-Ochoa).(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Diagnosis and treatment of gastroesophageal reflux in 500 children with respiratory symptoms: the value of pH monitoring. 203 Apr 75


1 2 3 4 5 6 7 8 9 10 Next >>