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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lung transplantation has become a successful method in the therapy for end-stage pulmonary disease. While single-lung transplantation provides benefit to patients with pulmonary fibrosis, bilateral lung transplants are required for septic or emphysematous lung disease. We describe the technique employed in 6 patients to transplant en bloc both lungs with the recipient heart left in place. The lungs are connected by a left atrial cuff, main pulmonary artery, and trachea. The completed implantation has a tracheal anastomosis securely wrapped in omentum, a left atrial anastomosis posterior to the heart, and a pulmonary artery anastomosis anteriorly. Airway ischemia resulted in the death of 1 patient. This procedure allows complete excision of all diseased pulmonary tissue, retention of the recipient's own heart, and separate excision of the donor heart for use in another recipient, thereby markedly increasing the supply of donor lungs for transplantation.
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PMID:Technique of successful clinical double-lung transplantation. 328 41

Sickle cell chronic lung disease (SCLD) is a prime contributor to mortality in young adult patients with sickle cell disease, especially those with sickle cell anemia (SS). Both perfusion and diffusion defects have been demonstrated, with generalized pulmonary fibrosis and disabling restrictive lung failure. We report 28 cases (25 SS, 1 S beta(0) thalassemia, 1 S beta(+) thalassemia and 1 SO-Arab) which began during the second decade of life and which ended in death by the fourth decade, after an ordered progression to pulmonary failure and cor pulmonale. Myocardial hypoxia with multifocal fibrosis and segmental infarction occurred in more than one-third of the cases and sudden death was a frequent final event. We define 4 stages of SCLD, based on pulmonary function tests, chest roentgenograms, blood gases, and noninvasive cardiac studies; each stage is 2 or 3 years in length, until death ensues in Stage 4. Case-control analysis showed that the significant risk factors associated with SCLD are 1) the total number of acute chest syndrome events in an individual before the onset of SCLD, (p = 0.0001), 2) sickle cell crisis marked by chest pain (p = 0.03) and 3) aseptic necrosis (p = 0.005). Temporal clustering of acute chest syndrome episodes frequently heralds the onset of SCLD. The pulmonary arterial bed, which has low oxygen tension and low pressure in a slow-flow system, is ideally suited to facilitate the polymerization of sickle hemoglobin, causing endothelial damage and culminating in an obstructive arteriolar vasculopathy. Identification of the significant risk factors predictive of SCLD can lead to early diagnosis of the disease; this is the only hope for effective intervention therapy.
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PMID:Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. 333 82

Interleukin 1 secretion from human alveolar macrophages was studied in patients with interstitial pulmonary fibrosis, sarcoidosis, and the acquired immunodeficiency syndrome with pneumonitis and compared to secretion from alveolar macrophages of normal volunteers. Macrophages lavaged from the lungs were stimulated with 10 micrograms/ml of lipopolysaccharide and cultured for 24 hr. In some cases macrophages were also stimulated with 1 microgram/ml lipopolysaccharide. After dialysis of the culture supernatants, interleukin 1 secretion was quantified by the thymocyte proliferation assay and probit analysis and expressed in terms of secretion from 1 million macrophages. Results showed that, on average, macrophages derived from patients secreted more interleukin 1 after stimulation with lipopolysaccharide compared to normal subjects. Mean secretion was significantly greater from macrophages of patients with acquired immunodeficiency syndrome and interstitial pulmonary fibrosis when stimulated with 10 micrograms/ml lipopolysaccharide. Of the 24 individuals studied, spontaneous interleukin 1 secretion was detected from unstimulated macrophages in only 1 patient and 1 normal volunteer. We conclude that alveolar macrophages lavaged from the lungs of patients with inflammatory lung disease have an increased capacity to secrete interleukin 1 on in vitro stimulation with lipopolysaccharide. Possible mechanisms for this increase are discussed.
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PMID:Interleukin 1 secretion from human alveolar macrophages in lung disease. 348 3

During the past four decades there has been a growing appreciation of the frequency of pulmonary abnormalities associated with RA. Approximately 30% to 40% of patients with RA demonstrate either radiographic or pulmonary function abnormalities indicative of interstitial fibrosis or restrictive lung disease. The severity of pulmonary fibrosis is not associated with rheumatologic symptoms or the duration of the associated RA, nor is there any clear relation to the extraarticular features of RA or serologic findings. Survival rates in patients with coexisting RA and pulmonary fibrosis are similar to those of patients with idiopathic pulmonary fibrosis. However, the spectrum of disease activity is quite variable. The majority of patients with progressive pulmonary symptomatology, when treated with corticosteroids, will have equivocal results. Some patients appear to respond to immunosuppressive or cytotoxic medications. The role of macrophages may be central to the injury to lung. Recent studies suggest a potential treatment role for cyclosporine, which may be able to interrupt lymphocyte-stimulated macrophage activation, and thus, fibroblast-mediated fibrosis in patients with pulmonary interstitial fibrosis. Bronchoalveolar lavage studies may delineate subgroups of patients who are more likely to respond to immunosuppressive agents, especially when treatment is started early.
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PMID:Pulmonary fibrosis in rheumatoid arthritis: a review of clinical features and therapy. 354 56

Chest X-ray, pulmonary mechanics, clinical lung disease and growth were studied in 48 low birthweight infants surviving after ventilator treatment in the neonatal period. Bronchopulmonary dysplasia (BPD) was present in 14 infants shortly after weaning off ventilator. At 4 to 6 years of age most patients had normal chest radiographs but 13 still showed signs of pulmonary fibrosis and hyperinflation. Most patients had low dynamic compliance and high pulmonary resistance shortly after ventilator treatment. All but 8, however, had normal findings at 1 to 1 1/2 years of age. Pneumonias and bronchitis were common during the first two years but thereafter declined in frequency. Weight and length development were retarded for BPD patients during the first two years and for non-BPD patients for the first year. Both groups had a complete catch-up.
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PMID:Long-term follow-up of ventilator treated low birthweight infants. I. Chest X-ray, pulmonary mechanics, clinical lung disease and growth. 355 85

Lung samples from four infants who died of respiratory complications of prematurity (Infant Respiratory Distress Syndrome, IRDS) were analyzed for their content of various collagen crosslink amino acids by newly developed techniques of high-performance liquid chromatography. Comparable analyses were performed with tissue from stillborn infants with apparently normal lungs (control group) and from adults without apparent lung disease. We observed increased amounts of the difunctional crosslink dihydroxylysinonorleucine (DHLNL) in the IRDS lungs. Gestational age seemed to be the most important determinant of total lung content of the trifunctional crosslink hydroxypyridinium (OHP). Term infants had about one-third of the OHP content in their lung collagen as was found in the adult lungs. These observations suggest that there are important changes in the molecular structure of collagen in human fibrotic lung disease, changes that are paralleled in various animal models of experimental pulmonary fibrosis, and in various human diseases involving abnormalities of skin or bone collagen metabolism.
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PMID:A molecular marker for fibrotic collagen in lungs of infants with respiratory distress syndrome. 356 76

Idiopathic pulmonary fibrosis (IPF) and pulmonary fibrosis associated with a collagen vascular disorder (PF-CVD) are chronic inflammatory lung disorders which may be characterized in various subgroups of patients by increased numbers of macrophages, neutrophils, lymphocytes, and/or eosinophils. Previous studies have suggested that the cell populations recovered with bronchoalveolar lavage (BAL) may be important in predicting disease progression and response to therapy. We evaluated this hypothesis in 27 patients by determining if the cell populations recovered with BAL differed between patients who improved, remained stable, or worsened in their pulmonary functions (as defined by at least a 15 percent change in forced vital capacity) over a six-month observation period. The findings suggested that BAL eosinophilia may be a marker of progressive lung disease in patients with IPF and PF-CVD.
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PMID:Prognostic role of eosinophils in pulmonary fibrosis. 359 49

The effect of dose and time and of the RBE on the pulmonary reaction to radiation has been studied by means of trials with young pigs. 70 animals received a fractionated (five and 15 fractions) telecobalt irradiation of the right lung over total treatment periods T of five, 23, and 35 days. The influence of dose and time on the pulmonary reaction to radiation is defined at the ED50 level (effective dose, after which 50% of animals will suffer from radiopathy) by a proportionality of D approximately N0.32 X T0.05. An alpha/beta value of 3.7 Gy is calculated according to the LQ model. An RBE value of 4.0 to 4.1 was determined in 38 animals treated over five and 35 days with the 6.2 MeV neutrons of the cyclotron Rossendorf. The experimentations on animals allow to classify the lung reaction as late effect. The clinical results found in literature show that this classification is also valid for human lung reaction to fractionated radiotherapy. So it is not possible to achieve a considerable increase of the pulmonary tolerance dose by increasing the total treatment period. The best sparing of the respiratory organ is obtained by an application of small single doses which allows to profit from the large repair capacity of pulmonary tissue. These results, as well as our considerations regarding the latent time between therapy and lung reaction, and some trials on laboratory rodents allow to speak of a radiogenic pneumopathy with the proprieties of a late reaction characterized by a pneumonitis appearing previously and changing into pulmonary fibrosis.
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PMID:[Repair mechanisms of the lung--experimental and clinical results]. 360 64

Pulmonary valve echography was technically possible in 44 of 120 patients with chronic lung disease (CLD). Mean pulmonary arterial pressure (Ppa) was significantly correlated with right ventricular isovolumetric relaxation ratio (RVIRT/RVET) as derived from the movement of cusps in valves of the right heart (r = 0.842; p less than 0.001). Prolonged RVIRT was found in 52% of patients with Ppa greater than 20 mmHg and in all ten patients with Ppa greater than 35 mmHg. Other echographic signs, including abnormal right ventricular systolic time intervals (STI), 'a' wave amplitude, right to left ventricular ratio (RV/LV) and interventricular septum diastolic bulging (IVS-B), were less sensitive, but found more often; when higher Ppa was recorded at catheterization in a given patient (r = 0.869; p less than 0.001). Approximate echographic estimation of Ppa in patients with chronic lung disease is possible if right ventricular systolic and diastolic time intervals can be measured and if abnormalities in right ventricular dimensions, interventricular septum motion and pulmonary valve echogram are considered. In contrast to pulmonary fibrosis, technical problems in adequate visualization of right heart valves in chronic obstructive lung disease limit the use of M-mode echo assessment of Ppa to a clinically unacceptable 22% of subjects.
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PMID:Value of M-mode echocardiography in assessing pulmonary arterial pressure in patients with chronic lung disease. 366 14

A wide variety of types of pulmonary diseases and respiratory symptoms have been associated with gastroesophageal reflux (GER). Asthma, chronic bronchitis, bronchiectasis, and pulmonary fibrosis have all been linked to GER, but causal mechanisms have been difficult to establish. To characterize pulmonary function abnormalities in older children and young adults (age 7-23 years) with GER, lung function was evaluated in 22 patients being treated for reflux. The patients were divided into two groups: nine subjects (Group 1) had no history of pulmonary symptoms. Thirteen subjects (Group 2) had known pulmonary disease; all had diagnosed asthma, and five had a history of recurrent pneumonia. Lung volumes and spirometry were measured. Airway reactivity was assessed by measuring change in flows following isocapneic hyperventilation of subfreezing air. The presence of "small airway" disease was assessed by air-helium flow volume curves and the single breath oxygen test. Lung size, as indicated by measurement of total lung capacity, was normal in all patients. Flow rates, density dependence of maximal expiratory flow, single breath oxygen test, and tests of airway reactivity were abnormal only in Group 2 patients and were normal in the Group 1 patients. That not all children with GER have abnormal pulmonary function suggests that, if there is a causal relationship between GER and lung disease, it is multi-factorial in nature. Children with GER who do have lung disease have evidence of airway obstruction, maldistribution of ventilation, and increased airway reactivity, but do not have restricted lung volumes.
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PMID:Pulmonary function in older children and young adults with gastroesophageal reflux. 376 70


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