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Swyer James syndrome following Mycoplasma pneumoniae pneumonia: report of one case.

A previous healthy 5 year-old girl developed a right hyperlucent lung following Mycoplasma pneumoniae pneumonia 14 months before admission. Serial chest radiographs revealed a persistent right upper lobe atelectasis and gradual development of the right hyperlucent lung associated with frequent bouts of wheezing and exertional dyspnea. Physical examination showed markedly decreased breathing sounds in the right hemithorax with fine inspiratory crackles and expiratory wheezes. A diagnosis of Swyer James syndrome was confirmed by the exclusion of other causes of unilateral hyperlucent lung using computed tomographic scans of chest, fiberoptic bronchoscopy and lung perfusion scintigraphy. She has been followed up at our hospital using anti-asthmatic medication.

Child, Preschool↗

[Clinical case of the month. MacLeod syndrome].

MacLeod syndrome is a rare cause of localized hypertransradiancy of the lung. This syndrome is defined by radiological features: localized hypertransradiancy due to oligemia and presence of air-trapping on expiratory chest radiography. Involvement of one entire lung is called "unilateral hyperlucent lung". Whereas the etiology is different, the physiopathology is probably identical to that of the panacinar emphysema of chronic obstructive pulmonary disease. The syndrome is believed to be related to acute bronchiolitis during infancy. Clinical manifestations and prognosis depend mainly on the presence of other lesions due to the same infectious agent like bronchiectasis. Pulmonary function tests, chest CT-scan, ventilation and perfusion scintigraphy and, if necessary, bronchoscopy help the differential diagnosis and detect associated bronchiectasis.

Aged↗

Acquired segmental emphysema: the enlarging spectrum of Swyer-James/Macleod's syndrome.

Three cases of Macleod's syndrome are described, all of which required surgical resections for distressing symptoms. This provided a rare opportunity to examine the pathologic features of a condition that is usually diagnosed on its radiologic features, and the etiology of which remains unestablished. Three patients (2 women and 1 man), aged 20, 23, and 24 years, were referred from respiratory physicians because of unilateral hyperlucent lungs and associated symptoms. All 3 patients had unilateral hyperlucent lungs, but only 1 patient had demonstrable mediastinal shift on expiratory and inspiratory chest computed tomographic scan. Segmentectomies were performed (n = 4) in all the patients without perioperative morbidity or mortality. Patients have been followed up between 6 and 18 months, and remain asymptomatic with a return to normal lifestyle. Histologic examination of the specimens found inflammation of the bronchus in all 3 patients, but only two specimens had evidence of bronchiolar inflammation. In only 1 patient was there a reduction in bronchiole number. All 3 patients showed presence of emphysema. These cases are notable for the segmental distribution of the disease. Pathologic examination lends support to the theory that previous respiratory tract infection may play a role in the pathogenesis of this condition.

Adult↗

Gross pulmonary thrombosis in a greyhound.

A two-year-old greyhound was presented with progressive dyspnoea. Radiography showed a hypovascular lung pattern with hyperlucent lung fields and echocardiography revealed a large thrombus in the main pulmonary artery. Blood results showed azotaemia and marked hypoalbuminaemia. The dog's clinical condition continued to deteriorate and it was euthanased. Postmortem examination confirmed the presence of the pulmonary thrombus and revealed idiopathic membranous glomerulonephritis.

Animals↗

Intralobar bronchopulmonary sequestration: evidence of air trapping shown by dynamic xenon-133 SPECT.

Dynamic single photon emission computed tomography with xenon-133 gas in a 29-year-old male patient showed xenon-133 retention within an intralobar bronchopulmonary sequestration (BPS) with a focal hyperlucent lung area on CT. Left lower lobectomy showed no fistulous connection between the anomalous and normal bronchial trees, but non-contiguous, incompletely developed visceral pleura between the sequestration and the adjacent normally ventilated lung. These features strongly support the role of intralobar collateral air drift and air trapping in producing secondary changes of a focal hyperlucent lung area in BPS.

Adult↗

Echocardiographic findings in one case of Swyer-James syndrome.

The Swyer-James syndrome is an uncommon disease. The main finding is a hyperlucent lung with small hilar shadows on chest x-ray. This is due to a decrease in pulmonary artery size at its origin and in the number and size of pulmonary branches. Pulmonary angiography is the standard method for diagnosis. We report a case of a unilateral hyperlucent lung in which the diagnosis was suggested by an echocardiographic evaluation.

Adult↗

Swyer-James syndrome associated with Noonan syndrome: report of a case.

A 28-year-old man with Noonan syndrome associated with unilateral hyperlucent lung is reported. He had the typical craniofacial appearance and short stature of Noonan syndrome; he had mild mental retardation, atrophic testis, mild funnel chest and kyphosis. cardiovascular abnormalities included asymmetric hypertrophic cardiomyopathy and a significantly different caliber of the left and right pulmonary arteries. The unilateral hyperlucent lung was shown to result from acquired nondestructive emphysema caused by nonvalvular obstruction of the bronchi (Swyer-James syndrome or Macleod's syndrome). To the authors' knowledge, this is the first reported case of Noonan syndrome associated with Swyer-James syndrome.

Adult↗

Swyer-James syndrome complicated by lung abscess.

Swyer-James syndrome, a rare disease with unilateral hyperlucent lung due to bronchiolitis obliterans and pulmonary artery hypoplasia, generally develops after lower respiratory tract infection during early childhood. Invasive procedures, including bronchoscopy and angiography, are often necessary for a definitive diagnosis. We report a 17-year-old man admitted because of cystic bronchiectasis complicated by lung abscess. Chest roentgenography showed the typical findings of Swyer-James syndrome. Noninvasive magnetic resonance angiography was used to confirm hypoplasia of the right pulmonary artery. The patient received antibiotic therapy, underwent a right lower lobectomy for the lung abscess, and recovered.

Adolescent↗

Differential effects of respiratory syncytial virus and adenovirus on mononuclear cell cytokine responses.

Respiratory syncytial virus (RSV) and adenovirus (Advs) serotype 3 (Adv3) and 7h (Adv7h) are associated with mild to severe respiratory infection and are indistinguishable during the acute phases of the illnesses. However, outcome and long-term prognosis are different with both infections. RSV infection is associated with later development of asthma, and Adv, mainly Adv7h, with severe lung damage, bronchiectasis, and hyperlucent lung. We hypothesized that this difference could be partly due to different immune responses induced by these viruses. To test this hypothesis we quantified TCD4+, TCD8+, and BCD19+ expressing the interleukin-2 receptor-alpha chain (CD25) and interferon-gamma (IFN-gamma), interleukin (IL)-10, and IL-4 in the supernatant of peripheral blood mononuclear cells (PBMC) from school children infected in vitro with and without RSV, Adv7h, and Adv3 and after phytohemagglutinin (PHA) stimulation in the presence or absence of these viruses at a multiplicity of infection (MOI) of 1. PBMC from every child produced more IL-10 (p </= 0.05) when infected with RSV than with Advs and noninfected control, and Adv induced more (p </= 0.05) IFN-gamma than did RSV and control. The IL-10/IFN-gamma ratio was significantly higher (p </= 0.05) in RSV-infected and significantly lower (p </= 0.05) in Adv-infected PBMC, than in noninfected cells. PHA-stimulated BCD19+ RSV- infected cells expressed more (p </= 0.05) IL-2R than did Adv-infected cells. These results suggest that Advs induce a Th-1-type immune response that is not seen with RSV. These patterns persist despite intersubject variation in the absolute quantity of cytokine produced.

Adenovirus Infections, Human↗

Plasma interferon-gamma, interleukin-10 and soluble markers of immune activation in infants with primary adenovirus (ADV) and respiratory syncytial virus (RSV) infection.

Adenovirus (ADV) and respiratory syncytial virus (RSV) are etiological agents of acute respiratory tract infection in infants. Long-term prognosis of ADV infection includes severe lung damage, bronchiectasis and hyperlucent lung, while RSV infection is associated with development of recurrent wheezing and subsequent asthma. These differences may be related to differences in the primary immune responses elicited by these viruses. In this paper, we investigated the type of cytokine responses and the magnitude of immune activation in ADV and RSV infections in infants. We examined plasma concentrations of interferon-gamma (IFN-gamma), interleukin-10 (IL-10), soluble interleukin-2 receptor (sCD25) and soluble tumor necrosis factor receptor II (sTNFR-II) in previously healthy infants during the acute phase of primary ADV infection (n = 21) and RSV infection (n = 68), and in uninfected controls (n = 44). In ADV-infected infants, IFN-gamma plasma levels were significantly higher than those observed in RSV cases and the control group (p < 0.05). RSV cases did not show any differences in IFN-gamma plasma levels compared to the other groups. sCD25 levels were significantly higher in ADV- and RSV-infected infants than in controls (p < 0.0001), and higher in ADV than in RSV cases (p < 0.05). sTNFR-II levels were significantly higher in RSV- and ADV-infected infants than in controls (p < 0.0001, p < 0.05, respectively), and higher in RSV than in ADV infection (p < 0.05). No significant differences were observed in IL-10 plasma concentrations between the three groups. These results indicate that ADV and RSV infections in infants differ significantly with regard to the magnitude of production of interferon-gamma and soluble immune activation markers sCD25 and sTNFR-II. These immunological differences may be involved in the different clinical outcomes associated with these viral infections.

Acute Disease↗

Late cardiovascular and pulmonary complications of therapy in Hodgkin's disease: report of three unusual cases, with a review of relevant literature.

With the advent of modern therapeutic approaches, even patients with advanced Hodgkin's disease have high cure rates today. Therefore, more attention is gradually being focused upon the late complications of chemotherapy and irradiation, appearing long after the patient is in remission and thought to be cured. In this report, we review the incidence and presentation of some of the cardiovascular and pulmonary complications which may appear later in the course of the disease. Cardiovascular mishaps reviewed include pericardial manifestations, conduction abnormalities, cardiomyopathy, and premature coronary artery disease. Pulmonary complications discussed are lung fibrosis, spontaneous pneumothorax, pulmonary veno-occlusive disease, and hyperlucent lung. Three instructive cases from our recent experience, are also presented. One fatal case was due to cardiac failure because of radiation-induced pericarditis and coronary artery disease. Another patient with an almost fatal complication required lung transplantation because of severe bilateral radiation fibrosis of the lung and pulmonary veno-occlusive disease. The third instance was also life-threatening in nature, with radiation-induced arterial changes in the major arteries of the chest and neck, resulting in recurrent cerebral and ophthalmic thromboembolic disease. It is suggested that potentially severe cardiopulmonary complications be considered during the planning of the initial and subsequent management of patients with Hodgkin's disease, particularly in an era employing autologous and allogeneic bone marrow transplantation as part of therapy in some cases.

Adult↗

[Unilateral aplasia and hypoplasia of pulmonary artery in childhood (author's transl)].

In the light of 8 observations, made by the authors, vasoanomalies underlying the syndrome of the unilateral hyperlucent lung are discussed in their correlation to homolateral bronchial lesions. There were 3 cases with unilateral aplasia of the pulmonary artery in which the unilateral hyperlucency was found always on the healthy side. In these cases there was a normal bronchogram on both sides. The vascularization by the bronchial arteries causes characteristic changes both in the X-ray-picture and endoscopy. Hypoplasia of the pulmonary artery occurring simultaneously with hyperlucency of the affected side of the lung was found in five cases, 4 times in connection with homolateral, severe, diffuse deforming bronchitis and bronchiectases and 1 case with congenital bronchus stenosis. There is the opinion that besides a congenital form of the hypoplasia of the pulmonary artery a (virus?) --pneumonia, acquired in early childhood, can lead to a pulmonary hypoplasia and a bronchopulmonary dysplasia. The diagnosis can be made only after several special examinations performed in a multi-step system. The significance of the lung scintigraphy within the scope of this complex diagnostics is especially emphasized. Indication for surgery should be made very cautiously. Antibiotic long-term therapy and dsipensary care are considered to be essential.

Adolescent↗

Radiology of airway foreign bodies in children.

Among 91 children with airway foreign bodies there were 10 with radiopaque foreign bodies. Seventy-four of the cases of non-opaque foreign bodies were unilateral. Seventeen had pulmonary consolidation or loss of volume and 33 had a unilateral hyperlucent lung. In 18 of the latter there was unilateral overinflation, but in 15 the hyperlucency was the result of pulmonary vasoconstriction. Twenty-three patients had normal routine inspiration radiographs; in 14 of these fluoroscopy or expiration films were obtained and air-trapping was demonstrated in 13. In twenty-three of the children the diagnosis was delayed. Fifteen of these had normal chest radiographs or unilateral hyperlucency alone. Since normal inspiration chest radiographs are frequently seen in cases of airway foreign bodies even when the diagnosis is delayed, it is strongly recommended that fluoroscopy or expiration films be obtained in any patient where the diagnosis is in doubt.

Adolescent↗

[Diagnostic considerations in unilateral hyperlucency of the lung (Swyer-James-MacLeod Syndrome)].

Swyer-James-MacLeod Syndrome (SJMS) is considered to be a relatively uncommon and complex disease characterized by roentgenographic hyperlucency of one lung, lobe, or part of a lobe, due the pulmonary vascular structure and alveolar overdistension. It is sometimes associated with bronchiectasis. This syndrome seems to be an acquired disease that develops after viral bronchiolitis and/or viral pneumonia in early childhood. Microscopically, there is evidence of patchy bronchitis and bronchiolitis.SJMS is usually asymptomatic and discovered accidentally by chest radiography in a child with respiratory symptoms and should be differentiated from other causes of unilateral hyperlucency on chest radiography, such as those related to congenital bronchial and/or vascular abnormalities. Treatment includes early control of lung infections, as well as influenza and pneumococcal vaccination. Few reports of this syndrome in children have been published. We describe the case of a 12-year-old boy with unilateral hyperlucency of the lung and respiratory symptoms of acute pneumonia and discuss the main diagnostic features of this syndrome.

Acute Disease↗

Bronchial compression by an enlarged left atrium in infants; a cause of hypovascularity of the left lung.

In three infants seen recently at our institution we noted signs of compression of the left main bronchus associated with enlarged left atria. None of our cases demonstrated the more usual signs of hyperinflation which are a hyperlucent lung field, depressed hemidiaphragm and mediastinal shift away from the affected side. In addition, hypoperfusion of the left lung was noted in each case. We believe that bronchial compression due to an enlarged left atrium, with consequent hypoxic vasoconstriction as a clinically significant entity, which is not well described and may be unappreciated in infants in whom the typical signs of hyperinflation are absent.

Bronchial Diseases↗

Persisting unilateral lung hypoperfusion after resection of a subcarinal bronchogenic cyst.

As a rare malformation with a wide variety of clinical modes of presentation, the bronchogenic cyst remains a diagnostic challenge. We report a case with a subcarinal bronchogenic cyst and stenosis of the left main bronchus presenting as "unilateral hyperlucent lung". Hypoplasia of the ipsilateral pulmonary vascular system persisted after surgical restitution of ventilation and is most likely due to an associated vascular malformation. Alternatively, anatomical fixation of pulmonary reflex vasoconstriction can be discussed.

Adolescent↗