Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 14 year-old white girl who developed fatal cor pulmonale from severe pulmonary sarcoidosis had a previous clinical diagnosis of cystic fibrosis. Chest radiographs demonstrated marked pulmonary fibrosis without the changes of severe bronchiectasis that would be anticipated with cystic fibrosis.
 ...
PMID:Childhood sarcoidosis with fatal cor pulmonale. 71 33

We report two siblings with cystic fibrosis and systemic amyloidosis. The major clinical problem in both cases was recurrent respiratory infection with pulmonary fibrosis and bronchiectasis prior to death at ages 20 and 22 years. Findings from postmortem examinations disclosed diffuse amyloidosis. In addition, amyloid infiltration developed in both patients, with enlargement of the thyroid gland, and one required thyroidectomy. An autopsy review of 17 additional cases of cystic fibrosis failed to disclose any other instances of systemic amyloidosis.
 ...
PMID:Systemic amyloidosis in cystic fibrosis. 88 4

Significant progress has been made since the first successful human heart-lung transplantation (HLT) for pulmonary vascular disease performed in 1981. The refinement of surgical techniques, use of cyclosporin as the main immunosuppressant, technique of distant organ procurement to expand the donor organ pool, and improved diagnosis and management of pulmonary infection and rejection have all contributed to this accomplishment. This has inevitably coincided with the extension of this procedure to other groups of patients with end stage heart and lung disease. Initially, HLT was offered to patients with cardiac disease associated with pulmonary hypertension. Because of the success, consideration was given to transplantation for parenchymal pulmonary diseases, initially pulmonary fibrosis and emphysema, and then suppurative lung disease such as in cystic fibrosis (CF). However, the application of HLT to patients with CF lagged behind because of concern related to the risk of sepsis, the systemic nature of the disease, malnourishment, and fear of recurrence of the epithelial CF defect in the transplanted lungs.
 ...
PMID:Lung transplantation for cystic fibrosis. 145 9

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease caused by bronchial colonization with Aspergillus fumigatus that affects approximately 10% of patients with cystic fibrosis (CF). The diagnosis in CF patients is difficult because the cardinal symptoms of ABPA occur frequently in CF, ie, pulmonary infiltrates and wheezing, as well as the frequent colonization with A fumigatus that leads to humoral reactivity. If left untreated, ABPA leads to bronchiectasis and pulmonary fibrosis. The pathogenesis of ABPA seems to be a prolonged asthmatic late-phase reaction orchestrated by CD4+ Th2-like T cells in response to persistent pulmonary A fumigatus allergen exposure. Thus, polyclonal and A fumigatus-specific IgE antibodies (and IgA and IgG) and blood pulmonary eosinophilia are stimulated by Th2-derived cytokines such as IL-4 and IL-5. In addition, IL-4 would also promote pulmonary transendothelial migration of eosinophils, basophils, and lymphocytes via induction of cell adhesion molecules and their ligands. IgE mast cell interactions would also contribute to the bronchial reactivity and inflammation. Recent advances have begun to identify immunodominant A fumigatus allergens. Evaluation of the quantity of IgE antibodies (and IgA and IgG) and T-cell cytokine responses to specific A fumigatus allergens should aid in the diagnosis and immunopathogenesis of ABPA, especially in CF patients.
 ...
PMID:Allergic bronchopulmonary mycosis complicating cystic fibrosis. 147 42

The types of lung transplants are single lung (performed on either side), double lung en bloc (which requires total cardiopulmonary bypass and myocardial protection), and bilateral-sequential lung transplant (which uses separate single lung transplants with separate main stem bronchial anastomoses). Single lung transplants are used for restrictive lung disorders, such as pulmonary fibrosis; double lung procedures are used for patients with obstructive lung disease, such as emphysema, and bilateral-sequential lung transplantation is used in patients diagnosed with generalized bronchiectasis, including cystic fibrosis, or any other chronically infected lung disease. The criteria for differentiating single versus bilateral transplantation is constantly being evaluated and redefined. The future of lung transplantation depends on continued research, improved methods of preservation, improved care to potential donors, and an increase in the availability of donors.
 ...
PMID:Pulmonary transplant. Transplantation for end-stage lung disease: a nurse's perspective. 153 47

Since January 1990, we have performed 29 isolated lung transplantations in 28 patients with end-stage lung disease (12 single, 16 bilateral). Recipient diagnoses were: cystic fibrosis (11), chronic obstructive pulmonary disease (6), pulmonary fibrosis (6), eosinophilic granulomatosis (1), postinfectious lung disease (1), adult respiratory distress syndrome (1), and primary pulmonary hypertension (2). There have been four deaths, two in patients with pulmonary fibrosis and two in patients with primary pulmonary hypertension. Four patients have undergone transplantation while on ventilatory support for respiratory failure (2 with cystic fibrosis, 1 having redo lung transplantation with cystic fibrosis, and 1 with adult respiratory distress syndrome); all of these have survived. Six patients required cardiopulmonary bypass, which was associated with increased transfusion requirement. All patients 2 months after discharge have returned to an active life-style, except for 2 patients who currently await retransplantation. Preoperative pulmonary rehabilitation has resulted in significant improvement in exercise performance in all patients. Immunosuppression consists of cyclosporine, azathioprine, and antilymphoblast globulin (University of Minnesota), withholding systemic steroids in the early postoperative period. We have employed bronchial omentopexy in all but four transplants; there has been one partial bronchial dehiscence, two instances of bronchomalacia requiring internal stenting, and one airway stenosis. Cytomegalovirus disease has been seen frequently (15 cases), but has responded well to treatment with ganciclovir. Other complication shave included one drug-related prolonged postoperative ventilation, thrombosis of a left lung after bilateral lung transplantation requiring retransplantation, five episodes of unilateral phrenic nerve palsy after bilateral lung transplantation (4 resolved), and the requirement of massive transfusion (greater than 10 units) in 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Isolated lung transplantation for end-stage lung disease: a viable therapy. 155 66

Allergic bronchopulmonary aspergillosis (ABPA) is a severe disease inducing bronchopulmonary anatomic lesions which complicate those already present in patients with cystic fibrosis. The frequency of this association is estimated between 0.6 and 10% according to American studies. Diagnosis of ABPA is difficult, as findings such as sibilant rales, pulmonary infiltrates, bronchiectasies, anti-aspergillus precipitins may be present as single features in patients with cystic fibrosis. Thus it is important to ascertain the diagnosis as oral corticosteroid treatment is the only one able to prevent evolution towards bronchiectasies and pulmonary fibrosis.
 ...
PMID:[Mucoviscidosis and allergic bronchopulmonary aspergillosis]. 179 39

At the end of December 1990 11 patients had survived lung transplantation at the 2nd Department of Surgery, University of Vienna. In these patients 7 single lung (SLTX) and four bilateral lung transplantations (DLTX) were performed. The indication for operation in 6 cases was emphysema, in 2 cases mucoviscidosis, in 2 cases pulmonary fibrosis, and in 1 case secondary pulmonary hypertension, following operation of a ductus Botalli. By means of fitness tests, blood-gas analysis, pulmonary function tests and ventilation respectively perfusion scan a marked improvement of the clinical status of the patients following lung transplantation could be shown.
 ...
PMID:[Uni- and bilateral lung transplantation: functional results. Vienna Lung Transplant Group]. 179

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

The modern era of lung transplantation followed the introduction of cyclosporin and the development of en bloc heart-lung transplantation in 1980. This was followed in 1983 by the first long-term successful unilateral lung transplant by the Toronto group. The same group carried out the first successful double lung transplantation in 1986. Heart-lung transplantation remains the most common operation for patients with end-stage lung disease but is being gradually replaced by single lung transplantation for end-stage pulmonary hypertension, pulmonary fibrosis and emphysema. The major indication for double lung transplantation is cystic fibrosis or emphysema in subjects less than 45 years of age. The probability of survival after lung transplantation is approximately 65% after one year and 60 and 50% after two and three years respectively. This compares favorably to the early results obtained with heart transplantation. These survival figures represent a marked improvement in the prognosis of patients with end-stage lung disease who are candidates for lung transplantation since such subjects should have an expected survival of less than two years without this intervention. The major problems encountered with lung transplantation are technical difficulties during the operation, serious infections and acute episodes of rejection, both of which are most common in the first six months following the operation, and bronchiolitis obliterans, which is a late complication most probably related to chronic rejection. Organ availability remains a major limiting factor to the availability of lung transplantation and, as a consequence, numerous patients die while awaiting a donor organ.
 ...
PMID:[Lung transplantation: the current state of knowledge]. 204 38


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