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)

The purpose of this study was to explore the relationship between diagnosis and the cost-effectiveness and cost-utility of lung transplantation. A microsimulation model was used, based on empirical data from the Dutch lung transplantation program, collected between 1991 and 1999. We assessed life-years, quality-adjusted life-years, and costs with and without transplantation for the diagnostic categories alfa-1 antitrypsin deficiency, COPD/emphysema, bronchiectasis, primary and secondary pulmonary hypertension, cystic fibrosis, and pulmonary fibrosis. Alfa-1 antitrypsin deficiency and bronchiectasis had the highest survival gain. Secondary pulmonary hypertension and pulmonary fibrosis had the lowest survival gain and the lowest gain of quality-adjusted life-years. As compared with COPD/emphysema, alfa-1 antitrypsin deficiency, bronchiectasis, and CF had 25%, 40% and 19% more favorable cost-effectiveness ratios, respectively. Cost-utility ratios varied less, with values of -7%, -14% and -11% for alfa-1 antitrypsin deficiency, bronchiectasis, and primary pulmonary hypertension, respectively, compared with COPD. In conclusion, our model suggests that there is considerable variation in cost-effectiveness and, to a lesser degree, in cost-utility between the different diagnostic categories. These variations are the result of differences in survival and in quality of life with and without lung transplantation.
 ...
PMID:Cost-effectiveness of lung transplantation in relation to type of end-stage pulmonary disease. 1519 75

Gene expression changes in the lungs induced by paraquat (PQ) administration were studied in rats using DNA microarrays that were detectable for 1,090 genes per DNA microarray. The rats were subjected to subacute PQ exposure (7 mg/kg, s.c., daily for eight administrations). Two days after the final administration, the rats were divided into two groups. Group 1 experienced significant body weight loss and displayed signs of subacute PQ toxicity, but Group 2 showed no significant effects due to the PQ treatment. A control group, Group 3, was also included. In the comparison of the gene expression levels in the animals from Group 1 or Group 2 to the control animals treated by vehicle, 48 genes in Group 1 and 29 genes from Group 2 were differentially expressed. The twenty-eight genes were common to these two groups. These differentially expressed genes following paraquat treatment were classified as follows: 5 neurotransmitter receptor genes; 4 transporter genes; 4 voltage-gated ion channel genes; 2 lipid metabolism enzyme genes; 2 G-proteins involved in endocytosis and exocytosis genes; 7 cytokine genes; 4 ADP ribosylation genes involved in cell death and regeneration; CFTR gene, which is the causal gene for cystic fibrosis; neurofibromatosis type 1 gene, which is the causal gene for the neurofibromatosis type 1 that is known to accompany pulmonary fibrosis; and the causal gene for spinocerebellar ataxia. These genes may prove to be the keys for the elucidation of the mechanism of PQ toxicity, e.g. PQ-induced pulmonary fibrosis.
 ...
PMID:Gene expression analysis of the lung following paraquat administration in rats using DNA microarray. 1520 77

To date, lung transplantation is an established therapy for advanced lung diseases such as chronic obstructive lung disease, cystic fibrosis, pulmonary fibrosis, and pulmonary arterial hypertension. This therapeutic option not only leads to increased survival but also to improvements in quality of life. To date, one-year survival rates at the Zurich Lung Transplant Program are 87% and five-year survival rates are 72%. It is of most importance that the referral to the transplanting center is not too late and that the right time point for transplant surgery is not missed. Progress has been made with regard to early detection and therapy of chronic allograft rejection thereby improving long-term survival substantially.
 ...
PMID:[Lung transplantation--indication, evaluation, risks and complications]. 1607 52

Lung transplantation has been developed over the last fifteen years as a therapeutic option for different forms of advanced-stage lung disease. Idiopathic pulmonary fibrosis is a good indication. For these patients, single lung transplantation is usually preferred, bilateral lung transplantation to a lesser extent. Survival is similar for these two types of transplantation. The post-transplantation survival in patients with pulmonary fibrosis is about 65-70% at one year and 40% at five years. This rate is lower than observed for COPD or cystic fibrosis. If there are no complications, the patient can recover nearly normal lifestyle. Among the different complications, reimplantation edema, infection, rejection, and bronchial complications predominate. Chronic rejection, also called obliterative bronchiolitis syndrome, is a later complication which can be observed in about half of the patients. Improvement in graft survival depends greatly in improvement in prevention and management of complications. Despite such complications, graft survival in fibrosis patients is greater than spontaneous survival on the waiting list; idiopathic fibrosis is associated with the highest mortality on the waiting list. Patients should be referred early for the pre-transplantation work-up because individual prognosis is very difficult to predict.
 ...
PMID:[Lung transplantation in patients with pulmonary fibrosis]. 1614 97

We present a descriptive study of patients referred as candidates for lung transplantation in the last 14 years. The 837 requests were evaluated stepwise in three phases: phase I, derivation report; phase II, outpatient evaluation; and phase III, inpatient evaluation. Chronic obstructive pulmonary disease was the most common reason for referral (31%). Cystic fibrosis was the referral disease with the best transplanted/referred relation (57%) and pulmonary fibrosis was the disease that had the highest mortality (39.7% of all deaths). Forty-three percent of all patients reached phase III and 29% were transplanted. Mortality on the waiting list was 3.7%. The most important causes of exclusion were inadequate indications and the presence of severe associated diseases. The mean study was 44 days. Knowledge of the natural history, local factors that influence organ availability, expected time on the waiting list, and disease progression allow optimization of this therapeutic option.
 ...
PMID:Evaluation of patients referred for lung transplantation: fourteen years experience. 1709 86

Lung transplantation is now generally accepted as a useful modality of care for patients with severe life-threatening respiratory diseases that are refractory to conventional therapies. Lung transplantation is indicated for patients with end stage lung diseases, who demonstrate declining function despite maximal therapy. The author reports own experience of selection, timing referrals for lung transplantation, and the management of patients on the waiting list. Eleven patients were selected for lung transplantation. Eight of them suffered from end stage pulmonary fibrosis, two of them cystic fibrosis, and one patient had pulmonary hypertension. Seven patients underwent a successful lung transplantation and four patients died during the investigations, before the operation, due to respiratory failure, and pulmonary heart disease. It is generally recommended to consider transplantation when patients who suffer in the end stage pulmonary disease are symptomatic during daily activities too, due to pulmonary status and the expected survival is to be strongly limited, less than two years.
 ...
PMID:[Patient's selection for lung transplantation, and management during the waiting time]. 1729 53

The reported pluripotential capabilities of many human stem cell types has made them an attractive area of research, given the belief they may hold considerable therapeutic potential for treating a wide range of human diseases and injuries. Although the bulk of stem cell based research has focused on developing procedures for the treatment of pancreatic, neural, cardiovascular and haematopoietic diseases, the potential for deriving respiratory cell types from stem cells for treatment of respiratory specific diseases has also been explored. It is suggested that stem cell derivatives may be used for lung replacement/regeneration therapeutics and high though-put pharmacological screening strategies for a variety of respiratory injuries and diseases including: cystic fibrosis, chronic obstructive pulmonary disease, respiratory distress syndrome, pulmonary fibrosis and pulmonary edema. This review will explore recent progress in characterizing adult respiratory and bone marrow derived stem cells with respiratory potential as well as the endogenous mechanisms directing the homing of these cells to the diseased and injured lung. In addition, the potential for embryonic stem cell based therapies in this domain as well as the histological, anatomical and molecular aspects of respiratory development will be summarized.
 ...
PMID:Deriving respiratory cell types from stem cells. 1822 Sep 3

Due to the shortage in organ donation, living related lung transplantation was developed. Over 400 cases were performed worldwide with good success rates with no major morbidity or mortality to the donors. Patients who need this type of procedure are desperate young pulmonary patients waiting for cadaveric transplantation. The procedure requires 2 donors that will donate one lobe each that will replace the recipient lungs. Indications include cystic fibrosis, pulmonary fibrosis and bronhiectasis. Recently, the first procedure was performed in Israel. More experience is needed worldwide in order to recruit more donors with good results.
 ...
PMID:[Living related donation in lung transplantation]. 1903 8

The 104th International Conference of the American Thoracic Society (ATS) was held in Toronto, Canada, May 16-21, 2008. The meeting brought together more than 15,000 scientists and clinicians worldwide and disclosed recent advances related to prevention, diagnosis, prognostic indicators or biomarkers of disease and treatment of thoracic disorders, including asthma, chronic obstructive pulmonary disease, pulmonary fibrosis, pulmonary arterial hypertension, cystic fibrosis and sleep disorders. New future lines of investigation are now open and invite researchers to attend the next ATS conference in San Diego in 2009. This report highlights oral sessions and posters presented during the meeting.
 ...
PMID:Novelties in the treatment and management of thoracic disorders. 1925 52

Lung transplantation remains the hope for many incurable pulmonary diseases, such as cystic fibrosis, pulmonary fibrosis, and chronic obstructive pulmonary disease. Remarkable progress has been made in improving outcomes, although the incidence of acute rejection remains more than 50% in the 1st year, and the 5-year graft survival is still less than 50% primarily because of the development of chronic rejection and graft dysfunction. Chronic rejection is characterized by the development of obliterative bronchiolitis in allografts and manifests as bronchiolitis obliterans syndrome in humans with no effective treatment. Previous studies support a role for alloreactive T cells in the development of bronchiolitis obliterans syndrome, but the specific mechanisms are unknown. One major stumbling block to research in the field of lung transplantation has been the lack of physiologic models to study the disease in the laboratory. We will review the current understanding of the immunology of the pathogenesis of obliterative bronchiolitis and will discuss exciting new advances from the laboratory as well as the implications for future research in lung transplantation.
 ...
PMID:Bronchiolitis obliterans in lung transplantation: the good, the bad, and the future. 1930 74


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