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

A light and electron microscopic study was carried out in 21 infants in whom the pathologic diagnosis of bronchopulmonary dysplasia had been made. All the infants except two had the respiratory distress syndrome at birth, and all 21 had been treated with respirator and oxygen therapy for various periods of time. The pathologic alterations observed in all the infants studied were primarily damage of the bronchial and bronchiolar ciliary apparatus and mucous membranes, severe necrotizing bronchiolitis, and marked bronchiolar and alveolar fibrosis. These changes were more pronounced in infants who survived the longest period of time. Such inflammatory and fibrotic changes are known to predispose to destruction of lung tissue, emphysema, and pulmonary hypertension. Six of these 21 infants developed symptoms and signs of cardiac atrial or ventricular stress, including cor pulmonale, prior to their demise. These infants were among those that survived the longest periods of time, had the longest exposure to supplemental oxygen, and showed histopathologically severe pulmonary fibrosis and emphysema.
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PMID:Bronchopulmonary dysplasia: the pulmonary pathologic sequel of necrotizing bronchiolitis and pulmonary fibrosis. 99 46

The influence of dexamethasone on levels of total fibronectin (tFn), cellular fibronectin (cFn), plasma fibronectin (pFn), and albumin in lung secretions was determined in tracheal aspirate samples collected from 45 infants with bronchopulmonary dysplasia during a 6-week course of dexamethasone therapy. Secretory component for IgA (SC) was used as a reference protein. Thirty-seven infants (82%) survived and had their endotracheal tubes successfully removed. Corticosteroid therapy was associated with a significant decrease in the cFn/SC ratio. There was also a significant decrease in albumin/SC and pFn/SC ratios, suggesting decreased capillary permeability with corticosteroid therapy. Four of the remaining infants did not improve while receiving corticosteroids and died of respiratory failure at 3 to 8 weeks of age. In these "no response" infants, tFn/SC, cFn/SC, pFn/SC, and albumin/SC ratios when corticosteroid therapy was initiated were threefold to fourfold greater (p < 0.01) than ratios in survivors. Another group of four infants initially responded to corticosteroids but subsequently died with severe pulmonary cystic degeneration at 4 to 6 months of age; in these infants, tracheal aspirate tFn/SC, cFn/SC, and albumin/SC ratios were significantly lower than in survivors and remained unchanged during corticosteroid therapy. The decrease in the concentrations of plasma fibronectin and albumin in tracheal aspirate samples from the survivors suggests that the rapid clinical improvement seen in infants with bronchopulmonary dysplasia after the initiation of dexamethasone therapy is due in part to improvement in the integrity of the alveolar-capillary barrier. In addition, the decrease in the aspirate levels of cFn suggests the potential for corticosteroids to limit pulmonary fibrosis in the surviving infants. The depressed levels of fibronectin observed in the infants with severe cystic lung disease may represent an impaired healing response to lung injury.
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PMID:Effect of dexamethasone therapy on fibronectin and albumin levels in lung secretions of infants with bronchopulmonary dysplasia. 140 98

Further investigation is required to delineate the biochemical and cellular interactions that influence lung injury and fibrosis. The results from studies in adults, neonates, and animals suggest that fibronectin may play a key role in the development of pulmonary fibrosis following acute lung injury. Fibronectin as well as other pulmonary cytokines are essential participants in efficient and orderly wound repair; however, the excessive production of these mediators may result in an exaggeration of the normal healing process with the eventual outcome of pulmonary fibrosis. The potential role of fibronectin and other cytokines as mediators and markers of BPD may thus allow for earlier detection and identification of those infants with RDS who are at greatest risk to develop BPD, as well as aiding in the development and selection of therapeutic interventions that can more effectively treat and possibly prevent the deleterious consequences of bronchopulmonary dysplasia.
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PMID:Fibronectin: role in respiratory distress syndrome and bronchopulmonary dysplasia. 151 7

The supply of donor organs remains extremely limited and improved methods to maintain the lungs of potential donors to allow for transplantation must be developed. Improved methods for preservation will increase the supply of suitable lungs and considerably simplify the logistics of transplantation, just as has occurred with liver transplantation. In the meantime, we continue to strive to utilize donor organs in the most efficient manner. On four occasions within the past 5 months, we have performed two simultaneous single lung operations and are prepared either to do this as a routine, or to offer the other lung to another center. Sufficient progress has been achieved to date to warrant the continued application of lung transplantation for end-stage pulmonary disease. With increasing experience, one can anticipate refinement of techniques and broader application of these procedures. Single lung transplantation, initially restricted to patients with end-stage pulmonary fibrosis, now has been applied successfully to patients with emphysema, pulmonary hypertension, and other conditions. We anticipate offering lung transplantation in the pediatric and even neonatal population in the very near future. This may prove to be particularly important in the group of children with so-called bronchopulmonary dysplasia, an end-stage interstitial fibrosis occurring in a small percentage of children born with what initially appears to be hyaline membrane disease and who require prolonged mechanical ventilation. Though currently transplantation can offer real benefit only to a limited number of individuals, it serves to create hope for many others. An additional benefit may prove to be the interest and attention that transplantation focuses on patients with end-stage lung disease and on the pathophysiology of chronic respiratory failure. Knowledge gained ultimately may result in the prevention of many of the disorders for which lung transplantation currently offers the only hope.
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PMID:The current status of lung transplantation. 153 99

Bronchopulmonary dysplasia (BPD) is a common pulmonary complication in preterm infants that leads to fibrosis of the bronchoalveolar walls and often to severe clinical consequences. Type III collagen is deposited early in progressive fibrosis. Because the N-terminal propeptide of type III collagen (PIIINP), a by-product of type III collagen synthesis, reflects the degree of pulmonary fibrosis in adults, we hypothesized that PIIINP in tracheal aspirates and/or serum may be a useful early marker of developing BPD in neonates. We serially measured PIIINP in tracheal fluid and serum samples during the first weeks of life in 41 consecutive respirator-treated preterm infants (mean birth weight 1067 g, mean gestational age 28.3 wk). Eight of the infants died and 22 infants fulfilled the criteria for BPD at age 28 d. The mean level of PIIINP decreased with advancing postnatal age in tracheal fluid but not in serum. The mean tracheal fluid PIIINP during d 1 and 2 of life, respectively, was 175 and 200 ng/mg protein in infants who were still in a respirator at age 28 d (n = 13), 122 and 97 ng/mg protein in those who were weaned earlier (n = 20), and 50 and 30 ng/mg protein in those who died before age 28 d (n = 8). These differences are not statistically significant, and the variability of the values was large. The PIIINP concentrations in tracheal aspirates of infants subsequently developing BPD did not differ from those without BPD. Neither did the levels correlate with the degree of BPD or radiologically defined fibrosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:N-terminal propeptide of type III collagen in tracheal fluid and serum in preterm infants at risk for bronchopulmonary dysplasia. 157 Feb

The first Danish experience of treatment of the respiratory distress syndrome (RDS) in preterm infants with exogenous surfactant is described. Fifteen infants with birthweights of 645-1,865 g and gestational ages of 25-32 weeks, all receiving artificial ventilation with at least 60% oxygen for severe RDS, were treated with purified porcine surfactant (Curosurf) within the first 28 hours of life. Pulmonary function improved immediately in all of the infants. Four infants (27%) died, four (27%) developed bronchopulmonary dysplasia (pulmonary fibrosis) and two (13%) had late neurological sequelae. These preliminary results are considered to be promising and they are in complete agreement with the results of randomised, controlled investigations from abroad. Systematic registration is, however, still necessary.
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PMID:[Treatment of severe respiratory distress syndrome with surfactant]. 163 99

The sequential stages of bronchopulmonary dysplasia occurring in 18 infants after intensive respiratory therapy supplemented by oxygen in high concentrations were studied by correlative light, scanning, and transmission electron microscopy. Infant survival ranged from 3 to 225 days. The earliest stage was an exudative reaction with a predominance of hyaline membranes. This merged with a subacute reparative response that was replaced by a chronic fibroproliferative stage in infants of longest survival; this stage was complicated by pulmonary fibrosis and emphysema. Correlative scanning and transmission electron microscopy demonstrated that type 2 pneumocytes contributed significantly to the reparative fibroproliferative response by organization of hyaline membranes and reepithelialization of damaged septal walls.
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PMID:Bronchopulmonary dysplasia: a correlative study by light, scanning, and transmission electron microscopy. 235 88

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

Plasma for fibronectin determinations was obtained from 39 neonates with uncomplicated respiratory distress syndrome (RDS) and from 15 infants with RDS who developed bronchopulmonary dysplasia (BPD). Tracheal lavage fibronectin and albumin concentrations were measured in 15 infants with RDS and 15 with BPD. Control plasma fibronectin values were obtained from 20 healthy preterm infants on days 1, 2, 3, 14, and 30 of life. Control tracheal lavage fibronectin and albumin concentrations were measured in 17 neonates of various gestational ages who required tracheal intubation for nonpulmonary indications. Mean plasma fibronectin concentrations from patients with RDS was 121 +/- 11 micrograms/ml on days 1, 2, and 3, versus control level of 163 +/- 12 micrograms/ml (P less than 0.01). Mean tracheal lavage fibronectin/albumin ratio was 3.8 +/- 0.6 ng per microgram of albumin on days 1 to 5 for infants with RDS, versus control level of 5.6 +/- 3.6 (P = NS). Tracheal lavage fibronectin/albumin ratio from patients with BPD was elevated at 16.3 +/- 5.0 ng fibronectin per microgram of albumin on days 14 to 21, and 23.6 +/- 7.4 on day 30 (P less than 0.05 versus control and and versus RDS days 1 to 10). Low plasma fibronectin concentrations early in RDS may contribute to the development of pulmonary capillary leak. High tracheal lavage fibronectin levels may foster the development of pulmonary fibrosis in patients with BPD.
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PMID:Tracheal lavage and plasma fibronectin: relationship to respiratory distress syndrome and development of bronchopulmonary dysplasia. 395 35

Continuous exposure of newborn C57BL mice to 80% oxygen at normal atmospheric pressure for as many as 6 weeks results in significant pulmonary injury. This injury is reflected morphometrically and morphologically primarily in an increase in the pulmonary interstitial compartment and in pulmonary fibrosis. The fibrotic response is both peribronchiolar and parenchymal. Lowering the oxygen concentration of continuous exposure from 100 to 80% appears to reduce the cellular response of the alveolar lining cells and the bronchiolar mucosa in the newborn lung more than the fibrotic response. This suggests that the most persistent response in the growing lung to supplemental oxygen concentrations at or below 80% will be peribronchiolar and parenchymal fibrosis. These findings would account for the clinically observed reduction of stage II bronchopulmonary dysplasia, yet persistence of chronic bronchopulmonary dysplasia in human infants treated with supplemental oxygen concentrations below 100%.
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PMID:Effect of prolonged exposure to 80% oxygen on the lung of the newborn mouse. 685 96


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