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1/8. Disodium cromoglycate in the treatment of bronchopulmonary dysplasia.

    bronchopulmonary dysplasia (BPD) has become the most common form of chronic lung disease in the neonate. Recently, we have experienced a severe case of BPD and examined the effect of disodium cromoglycate (DSCG) on BPD. The gestational age and birthweight of the patient were 27 weeks and 1,000 g, respectively. Although RDS subsided after surfactant replacement therapy, the arterial-alveolar oxygen tension ratio (a/APO2) gradually decreased and FiO2 increased with age, respectively, and pure oxygen supplementation was eventually required after 67 days of life. The DSCG treatment was commenced at 80 days of life. After 6 days of the inhalation therapy, a/APO2 gradually increased. After 10 days of the treatment, the baby was extubated. While the baby was intubated, intratracheal lavage fluid samples were obtained. Eosinophilic cationic protein (ECP) and polymorphonuclear (PMN) elastase concentrations were determined. ECP and PMN elastase concentrations of intratracheal lavage fluids gradually decreased with the DSCG treatment. These results may indicate that DSCG has led to an improvement of pulmonary function and facilitated weaning from mechanical ventilation in an infant with BPD.
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2/8. Effects of supplemental oxygen administration in an infant with pulmonary artery hypertension.

    In patients with pulmonary disease, pulmonary artery hypertension often occurs as a result of pulmonary artery vasoconstriction, primarily from hypoxia and alveolar hypotension. In this report we describe the hemodynamic effects of breathing supplemental oxygen in a child with bronchopulmonary dysplasia and pulmonary artery hypertension. These hemodynamic effects include an improvement in oxygenation, an increase in systemic vascular resistance, and a decrease in the pulmonary vascular resistance. As a direct result of these changes in vascular resistances, alterations of heart rate, cardiac index, stroke volume, aortic pressure, oxygen consumption, and pulmonary artery pressure have been shown to occur. Oxygen is widely used to treat many physiologic conditions. However, during the administration of supplemental oxygen, rarely do we recognize the hemodynamic changes associated with its use. These hemodynamic effects must be clearly understood and appreciated before oxygen administration in any clinical situation.
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keywords = alveolar
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3/8. Rhabdomyomatous dysplasia of the lung: a case report with review of the literature.

    The occurrence of rhabdomyomatous dysplasia of the lung in a macerated term baby with multiple congenital anomalies is described. The hypoplastic right lung revealed abundant striated muscle in the alveolar septa and bronchial walls of the middle and lower lobes. A brief review of the literature is followed by a discussion of the pathogenesis of this rare pulmonary malformation.
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4/8. Rhabdomyomatous dysplasia of the lung.

    This article deals with the presence of nontumoral striated muscle fibers in the lungs of 3 neonates. These cells were diffusely distributed in one lung (case 1) or in both (case 2), or focally localized to the lung parenchyma adjacent to the liver in a case with a large right diaphragmatic hernia (case 3). The striated muscle fibers were located in the walls of small bronchi and bronchioli or in the alveolar interstitium. Other major lung malformations found simultaneously were absence of lobation, hypoplastic lungs, and hypoplastic pulmonary vessels. The origin of striated muscle fibers in the neonatal lung has been attributed to anomalous differentiation of mesoblastic cells (as in cases 1 and 2). The presence of striated muscle cells in the lower margin of a hypoplastic lung associated with a right diaphragmatic hernia (case 3) suggests that intrapulmonary inclusion of diaphragmatic muscle fibers might be a source as well. Striated muscle fibers in the lung are commonly associated with major malformations involving heart and lungs, suggesting a much wider morphogenetic error.
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5/8. bronchopulmonary dysplasia in the adult.

    We describe 3 patients with adult respiratory distress syndrome that eventuated in a pathologic picture of honeycomb lung and a radiographic picture of variably cystic lung super-imposed on a background of diffuse alveolar infiltrates. All 3 patients had been treated with unusually high pressures of PEEP as well as high concentrations of oxygen for long periods of time (3 to 7 wk). Microscopically, the cystic structures in our patients appeared to be derived from collapse and fibrosis of the alveolar parenchyma with dilatation of the alveolar ducts. We suggest that this process is morphologically and radiographically similar to bronchopulmonary dysplasia as seen in the newborn.
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keywords = alveolar
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6/8. Pulmonary pathology in surfactant-treated preterm infants with respiratory distress syndrome: an autopsy study.

    The present study examines the histological features of the lungs of neonates who died of respiratory distress syndrome or related complications after surfactant therapy. Our aim was to determine whether these lungs showed any unusual histological findings. Complete autopsies were performed 6-12 h after death in 10 surfactant-treated preterm infants and in 30 infants who died before surfactant therapy was available. Representative paraffin sections of all pulmonary lobes, stained with haematoxylin and eosin, were examined microscopically. A few selected slides were also stained with periodic acid-Schiff, Vierhoff-van Gieson, and Mallory trichrome. hyaline membrane disease and bronchopulmonary dysplasia were present in each group, although there was an increased incidence of intra-alveolar haemorrhage in surfactant-treated babies (in 8 of 10 surfactant-treated as compared with 7 of 30 untreated babies). Amongst those treated with surfactant, we observed the persistence of acute alveolar damage with unresolved hyaline membrane disease in 5 infants who died at the ages of 5, 6, 10, 12, and 13 days, respectively, and histological evidence of pneumocyte type 2 hyperplasia and dysplasia in 2 infants who died at 22 and 41 days of age, respectively. These observations reveal that surfactant-treated infants who fail to respond to therapy have continuing alveolar injury and an increased incidence of intra-alveolar haemorrhage. Since oxygen radicals can induce pneumocyte damage and necrosis and since free radicals provoke alveolar haemorrhage in animal models, we propose that the lesions we observed may stem from a lack, in some preterm babies, of specific mechanisms that detoxify oxygen radicals.
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ranking = 5
keywords = alveolar
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7/8. Congenital alveolar capillary dysplasia: rare cause of persistent pulmonary hypertension.

    We report on a rare case of fatal congenital alveolar capillary dysplasia. The newborn boy of a 37 weeks' normal gestation suffered from persistent pulmonary hypertension without any cardiovascular malformation and died at the age of 4 weeks despite intensive treatment. The autopsy tissue was examined histologically, immunohistochemically, and ultrastructurally. Moreover, a three-dimensional tissue reconstruction based on serial sections was performed comparing the affected lung with normal lung tissue. We observed a unique pattern of pulmonary dysplasia: An extreme decrease of capillaries was localized centrally within thickened intra-acinar septa instead of capillaries intensely neighboring pneumocytes; ectatic veins normally running in the interlobular septa were found to accompany intralobular bronchovascular bundles, denying a clear distinction between pulmonary and bronchial veins; small muscular pulmonary arteries extended to the precapillary level and type 2 pneumocytes exceeded by far the type 1 pneumocytes, inverting the normal ratio. In summary, alveolar capillary dysplasia is assumed to be a primary capillary disorder of unknown origin, which possibly involves the regular differentiation of pneumocytes, according to the close alveolocapillary relationship during pulmonary ontogenesis. We consider the venous alterations as being part of the dysplasia, whereas the arterial phenomena might occur secondarily. Recent reports on affected siblings suggest a genetic component of pathogenesis.
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ranking = 6
keywords = alveolar
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8/8. Treatment of respiratory failure with inhaled nitric oxide and high- frequency ventilation in an infant with respiratory syncytial virus pneumonia and bronchopulmonary dysplasia.

    In a 7-month-old infant with bronchopulmonary dysplasia and respiratory syncytial virus (RSV) pneumonia, we have shown an additive effect of high-frequency ventilation (HFV) and inhaled nitric oxide (iNO) in terms of improved oxygenation and the avoidance of extracorporeal membrane oxygenation. Apparently, the combined therapy of HFV and iNO is superior to either therapeutic modality alone in the treatment of hypoxemic respiratory failure due to RSV pneumonia. The mechanism of increased lung expansion and alveolar recruitment appears to be responsible for a favorable clinical outcome. We conclude that the combined therapy of HFV and iNO should be considered in hypoxemic respiratory failure in pediatric patients.
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