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1/15. Solitary unilocular cyst of the lung with features of persistent interstitial pulmonary emphysema: report of four cases.

    Neonatal interstitial pulmonary emphysema (IPE) is a well-characterized lesion usually presenting in preterm newborns as a complication of respiratory distress syndrome and/or assisted ventilation. Occasionally, IPE may occur spontaneously in infants with no underlying pulmonary disease. Persistence of IPE (PIPE) may be diffuse or localized. Localized PIPE usually presents as multiple cysts 0.3 to 3 cm in one or more lobes of the lung. In this report, we describe four cases of unilocular large cysts (up to 5 cm in diameter) partially lined by uni- and multinucleated histiocytes in a foreign body type reaction and showing gas dissection of the surrounding parenchyma (present in case 1). These histological features favored the diagnosis of PIPE and the cases were interpreted as such, since no other clear-cut diagnosis could be defined. However, because the lesion was limited to one lobe, and the children were full term, asymptomatic at birth and without history of respiratory distress or assisted ventilation, differential diagnosis with other pulmonary cystic lesions of infancy is mandated.
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keywords = emphysema
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2/15. Anaesthetic management of a 6-week-old child with unilateral pulmonary interstitial emphysema.

    We report the case of a 6-week-old female infant suffering from respiratory distress related to cystic changes affecting the entire left lung. Anaesthesia was induced with sevoflurane in oxygen and spontaneous ventilation was maintained until intubation of the right main bronchus was secured. A left pneumonectomy was performed and the postoperative course was uneventful. The pathological diagnosis was pulmonary interstitial emphysema. This has not been previously reported in an otherwise normal child delivered uneventfully at term. The anaesthetic management of a child with a cystic lung lesion is discussed.
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keywords = emphysema
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3/15. The potencial role of rare earths in the pathogenesis of interstitial lung disease: a case report of movie projectionist as investigated by neutron activation analysis.

    A 60-year-old male subject who worked as a movie projectionist and who was exposed for 12 years to rare earths (RE) containing dusts from cored arc light carbon electrodes was investigated. Chest X-ray films and pulmonary function tests showed an interstitial lung disease, emphysema and a severe obstructive impairment with marked decrease of carbon monoxide diffusion capacity. The histological examination of a transbronchial biopsy confirmed the diffuse interstitial lung fibrosis. neutron activation analysis (NAA) of the biopsy showed concentrations of cerium (Ce), lanthanum (La), neodimium (Nd), samarium (Sm), terbium (Tb) and ytterbium (Yb) which were high compared to the corresponding elements in the transbronchial biopsies of 5 unexposed subjects as a control group. thorium (Th) (which is generally present as an impurity of the RE compounds) was also determined in order to estimate the radiation dose in the lung of the worker. On the basis of the clinical observations, of the analytical results by neutron activation analysis of RE and of the presence of Th in the transbronchial biopsy, as well as of the differential diagnosis, which tended to exclude other occupational or non-occupational lung diseases, a relation between the observed interstitial lung fibrosis and occupational exposure to RE is highly probable.
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keywords = emphysema
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4/15. Pulmonary storage with emphysema as a sign of Niemann-Pick type C2 disease (second complementation group). Report of a case.

    A case is described of Niemann-Pick type C2 disease presenting an infantile pneumopathic phenotype known to occur in this recently established, second, minor complementation group of Niemann-Pick type C (NPC) disease. However, the pulmonary involvement was unique, being dominated, in addition to the usual storage macrophage infiltration of the alveolar and septal compartments, by irregular emphysema attributed to storage cell migration into the bronchiolar lumen. The latter modified considerably the X-ray findings and hindered the initial clinical diagnosis. Otherwise, the storage phenotype, including the range of stored lipids, storage distribution, and cell and organ pathology, was found to be identical to that in the whole Niemann-Pick type C disease group dominated by NPC1. The biochemical findings (cholesterol esterification level) corresponded to the classical biochemical phenotype. Emphysema should thus be considered as a variant of the pulmonary NPC2 storage process, governed most probably by an epigenetic mechanism responsible for storage macrophage migration into the bronchiolar compartment.
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keywords = emphysema
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5/15. Persistent pneumomediastinum and dermatomyositis: a case report and review of the literature.

    We describe a 42-year-old man with dermatomyositis and interstitial lung disease who presented with anterior neck pain and dyspnoea. Chest radiographs showed subcutaneous emphysema, pneumomediastinum and diffuse reticulonodular infiltration in both lungs. After the administration of high doses of prednisolone, an improvement of pulmonary function and respiratory symptoms was observed but the pneumomediastinum persists 12 months after diagnosis, and without any complication. We review the cases that have been reported thus far of pneumomediastinum associated with dermatomyositis and discuss the possible mechanisms involved. We conclude that pneumomediastinum is not an uncommon complication of dermatomyositis and that its aetiopathogenesis remains very unclear.
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ranking = 0.2
keywords = emphysema
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6/15. CT imaging of pulmonary lobar interstitial emphysema in a spontaneous breathing preterm infant.

    Pulmonary interstitial emphysema (PIE) is a well-recognized severe complication of neonatal respiratory distress syndrome (RDS). However, its occurrence under spontaneous breathing conditions has been described rarely. We present a case of PIE of the left upper lung lobe in an extremely low birth weight infant. Recurrent episodes of spontaneous pneumothorax led to the diagnosis, which was confirmed by histopathology. Plain chest X-ray did not show typical signs of PIE, whereas extra-alveolar air accumulation could be visualized by helical computed tomography (CT)-scan. We stress the role of predispositional factors increasing the risk of PIE development in spontaneous breathing preterm infants.
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7/15. A case of dermatomyositis complicated with pneumomediastinum successfully treated with cyclosporin A.

    We describe a rare case of a 46-year-old Japanese man with dermatomyositis (DM) and interstitial lung disease who developed spontaneous pneumomediastinum and subcutaneous emphysema. Relatively mild myositis, mild elevation of CK values and the absence of anti-Jo-1 antibody were observed and the case was similar to amyopathic DM. Treatment of this patient with oral prednisolone and cyclosporin A (CsA) was effective for the myositis and interstitial lung disease. The administration of CsA enabled rapid tapering of the dose of prednisolone without aggravating the disease. Pneumomediastinum and subcutaneous emphysema disappeared 5 months later without recurrence. The serum levels of KL-6 were monitored every 2 weeks to help determine whether this may have contributed to the recurrence of interstitial pneumonitis. This is a rare case of pneumomediastinum in a patient with DM.
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ranking = 0.4
keywords = emphysema
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8/15. Alarming hypoxemia during one-lung ventilation in a patient with respiratory bronchiolitis-associated interstitial lung disease.

    PURPOSE: To report a patient with respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) who developed severe hypoxemia during one-lung ventilation (OLV). CLINICAL FEATURES: A 27-yr-old female, ex-smoker presented with productive cough and dyspnea of 18-month duration. The chest x-ray revealed diffuse abnormalities involving both lungs consisting of interstitial emphysema with irregular shadowing. Preoperative PaO(2) was 88 mmHg and pulmonary function tests showed moderate obstructive disease. The patient underwent right open lung biopsy. After induction of anesthesia, a left double lumen tube was inserted and its position verified with auscultation and fibreoptic bronchoscopy. Upon initiation of OLV, the patient developed severe hypoxemia and the PaO(2) dropped from 500 mmHg during two-lung ventilation (TLV) to 50 mmHg. Hypoxemia was readily corrected by resuming TLV. CONCLUSION: The severe hypoxemia during OLV in this patient with RB-ILD may be attributed to impaired hypoxic pulmonary vasoconstriction. Other causes were not excluded. Caution is warranted when initiating OLV in these patients.
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ranking = 0.2
keywords = emphysema
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9/15. titanium particles identified by energy-dispersive X-ray microanalysis within the lungs of a painter at autopsy.

    A 72-year-old male painter, who complained of his "lungs burning" for 2 weeks, died suddenly. autopsy examination revealed severe coronary atherosclerosis with plaque rupture as the cause of death. Examination of the lungs revealed emphysema, interstitial fibrosis, and multinucleated giant cells with intra- and extracellular brown-black, crystalline, polarizable foreign material. Energy-dispersive X-ray microanalysis showed the material to contain titanium, aluminum, silicon, and iron. An increased incidence of respiratory disease has been reported in professional painters. titanium is widely used as a pigment in the manufacturing of commercial paints. Cases of pneumoconiosis and alveolar proteinosis have been described in painters in which analysis of lung tissue revealed increased levels of titanium. This case is presented as an example of a rarely reported phenomenon, which may have clinical implications for evaluation and management of lung disease in painters.
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ranking = 0.2
keywords = emphysema
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10/15. Multiple rheumatoid nodules with rapid thin-walled cavity formation producing pneumothorax.

    We report a rare case of multiple rheumatoid nodules that on chest CT demonstrated peripheral location with thin-walled cavity formation, rapid increase in size within several months, and complicating pneumothorax, pneumomediastinum, and subcutaneous emphysema due to bronchopleural fistula formation.
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ranking = 0.2
keywords = emphysema
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