Mediastinal lymph node involvement is uncommon in intrathoracic tuberculosis. We report three cases of this disease, each of which had a different clinical course. Chest CT scans showed preferntial involvement of right paratracheal nodes, central areas of relatively low density with peripheral rim enhancement after injection of contrast medium. Specimens obtained by mediastinoscopy and fiberoptic bronchoscopy revealed acid-fast bacilli in all cases.
In view of its relative frequency, tuberculous mediastinal lymphadenitis in adults must be distinguished from other causes of mediastinal masses.

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We studied the effect of T-kinin on airway vascular permeability and its modulation by endogenous peptidases in anesthetized rats in vivo, Vascular permeability was assessed by photometric measurement of extravasated Evans blue dye after formamide extraction. Intravenous injection of T-kinin increased dye extravasation in the trachea and main bronchi in a dose-dependent manner. Plasma extravasation evoked by T-kinin was inhibited by Hoe 140, a B₂ receptor but-not by des Arg⁹-Leu⁸-bradykinin, a B₁ receptor antagonist. Treatment with captopril, an angiotensin-converting enzyme inhibitor, potentiated the T-kinin-induced plasma extravasation, whereas phosphoramidon, a neutral endopeptidase inhibitor, had no effect. These results suggest that T-kinin increases airway vascular permeability via stimulation of B₂ receptors, and that this effect is modulateld by endogenous angiotensin-converting enzyme.

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The role of nitric oxide (NO) in the activity of cyclooxygenase (COX) in cultured canine tracheal epithelium was studied. Tracheal epithelium spontaneously released prostaglandin E₂ (PGE₂), which is a product of COX. The release of PGE₂ was increased by bradykinin and was decreased by two NO synthase inhibitors: N^G-nitro-L-arginine methyl ester and N^G-monomethyl-L-arginine. That decrease was reversed in the presence of L-arginine. Chrolpromadin, but not aminoguanidine, inhibited PGE₂ production, which suggests that constitutive NO synthase is involved. Two stable NO donors, sodium nitroprusside and S-nitroso-N-acetyl DL-penicillamine, also increased the production of PGE₂. These effects were abolished by coincubation with hemoglobin, which binds and inactivates NO, but not by methylene blue, an inhibitor of soluble guanylate cyclase. NADPH diaphorase histochemistry of cultured tracheal cells revealed activity in the periphery of the cytoplasm. These results suggest that, in cultured canine tracheal epithelium, NO directly interacts with COX to regulate PGE₂ production.

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KL-6 is a mucinous glycoprotein expressed on Type 2 pneumonocytes, and serum levels of KL-6 are reported to be abnormally high in patients with interstitial pneumonia. A new assay kit for serum KL-6 (ED046) was used in the evaluation of patients with pneumonitis. To clarify whether KL-6 is a useful marker of pneumonitis activity, 649 subjects were studied, including 185 healthy controls, 187 patients with 3 types of interstitial lung diseases, and 277 patients with 4 types of non-interstitial lung diseases. The serum KL-6 level was significantly higher in the patients with pneumonitis (1285±1196U/ml)than in the patients without pneumonitis (307±232U/ml). The KL-6 level was also significantly higher in patients with clinically active pneumonitis (1708±1338U/ml)than in those with inactive pneumonitis (820±796U/ml) (p<0.0001). Serum KL-6levels correlated significantly with serum c-reactive protein, lacticdehydrogenase, and PaO₂ values. These results suggest that the ED046 assay is useful for measuring KL-6 as a marker of pneumonitis activity.

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We developed a program of respiratory muscle stretch gymnastics (RMSG), and measured lung function, exercise capacity, dyspnea, and quality of life before and after 4 weeks of training with this program. Thirteen patients with chronic obstructive pulmonary disease (mean FEV₁, 1.24 liters) began the program. They participated in three sessions of RMSG each day. Twelve patients completed all 4 weeks.
RMSG significantly (p<0.01) decreased functional residual capacity (from 4.19±1.27 to 3.88±1.03 liters), total lung capacity (from 5.98±1.35 to 5.66±1.20 liters), residual volume (from 3.29±1.16 to 2.89±0.89 liters), and residual volume as a percent of total lung capacity (from 53.9±11.2% to 50.6±9.74%). The distance walked in 6 min increased by an average of 43±30 meters (+15%, p<0.01). Dyspnea after a 6-minute walk (measured with a 150-mm visual analog scale) decreased significantly (from 65.1±40.8 to 36.1±36.8, p<0.05). Quality of life was measured with the Chronic Respiratory Disease Questionnaire of Guyatt, et al., and was found to have improved significantly. Respiratory muscle stretch gymnastics may be useful in pulmonary rehabilitation.

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Among 102 patients with idiopathic interstital pneumonia (IIP), 22 with lung cancer who were treated from 1981 to 1994 were studied retrospectively. As controls, 248 patients with lung cancer only who were treated from 1991 to 1992 were also studied. Lung cancer developed in 6 of 85 patients after IIP was diagnosed (7.1%). The time from the onset of IIP to the onset of lung cancer was 7.7 years. In 17 patients both IIP and lung cancer were present on admission. In general, patients with IIP and lung cancer were heavy smokers. In patients with IIP and lung cancer, %VC was higher than in those with IIP alone, and 20 cancers (90.0%) were in peripheral lung fields. Seventeen patients received anticancer chemotherapy, irradiation, or both. Five patients (29.4%) died of respiratory failure due to progression of pneumonitis after the therapy. The prognosis for patients with IIP and lung cancer was very poor.

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²⁰¹Tl single photon emission computed tomography (²⁰¹Tl SPECT) was used to evaluate 18 patients with large opacities due to silicosis and 22 others with bronchogenic carcinoma. An early scan and a delayed scan were obtained and the retention index was calculated from the early ratio and the delayed ratio.
In patients with silicosis, the retention index and the two ratios were significantly lower than in the patients with bronchogenic carcinoma (p<0.01). In patients with stable shadows on chest X-ray films due to large opacities of silicosis, the delayed vatio was the same as or lower than the early ratio. However, in patients with silicosis who had high activity in large opacities, the delayed ratio was higher than the early ratio.
These results suggest that ²⁰¹⁾Tl SPECT is useful for evaluating the activity of large opacities in patients with silicosis and for differentiating large opacities caused by silicosis from those caused by bronchogenic carcinoma.

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To assess the effects of 2-chloroadenosine (2CA) on acute lung injury caused by endotoxin (lipopolysaccharide), guinea pigs were given 2CA intravenously. Three groups were used: saline control, endotoxin control and 2CA+ endotoxin. In the endotoxin and 2CA+ endotoxin groups, neutrophils accumulated in bronchoalveolar lavage fluid and in lung tissue. Howevre, neutrophil accumulation did not differ significantly between the endotoxin and the 2CA+ endotoxin groups. The number of macrophages in bronchoalveolar lavage fluid was signficantly higher in the endotoxin group than in the saline control group, but the difference between the saline control and the 2CA+ endotoxin groups was not significant. The lung wet-dry weight ratio and ¹²⁵I-albumin lung tissue-plasma ratio, which were used to measure acute lung injury, were significantly higher in the endotoxin group than in the 2CA+ endotoxin and the saline control groups. However, these ratios did not differ between the 2CA+ endotoxin and the saline control groups. These results suggest that 2CA attenuated endotoxin induced acute lung injury in guinea pigs.

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We examined relationships among the distance walked in 10 minutes (10 MD), pulmonary function, and pulmonary hemodynamics during exercise on a bicycle ergometer, in patients with chronic pulmonary empysema who had dyspnea of gale III to IV on the Hugh-Jones scale. The 10MD did not correlate significantly with desaturation during the 10-minute walk, but it did correlate significantly with the percent of predicted maximum voluntary ventilation, and it correlated negatively with airway resistance. These findings indicate that ventilatory impairment is an important factor limiting 10MD in these patients. Also 10MD correlated significantly and positively with %DL_CO; and it correlated negatively with the index of pulmonary vascular resistance and with the ratio of the change in pulmonary arterial pressure to the change in cadiac index during exercise. (ΔPpa/ΔCI). These findings indicate that impair ment of pulmonary circulation may also limit the 10MD and exercise tolerance in patients with chronic pulmonary emphysema.

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We found a novel trypsin-like enzyme (tryptase) in sputum from patients with chronic airway diseases, and named this enzyme human airway tryptase (HAT). To clarify its physiological significance in the airway, we compared biochemical properties of purified HAT with those of purified lung mast cell tryptase (MCT).
Studies with model peptide substrates showed that both the HAT and MCT preferentially cleaved the COOH-terminal side of arginine residues of certain peptides, but substrate specificities to nine synthetic model substrates of HAT differed from those of MCT.
Effects of protease inhibitors on the two enzymes were examined at a concentation of 10μM. Both the HAT and MCT were strongly inhibited by the trypsin inhibitors leupeptin, antipain, and aprotinin. An alpha-1-protease inhibitor inhibited HAT by 50%, but it did not inhibit MCT. In contrast, a secretory leukocyte protease inhibitor strongly inhibited MCT, but not HAT. Mucoid sputum from patients with chronic bronchitis contained much more HAT than MCT.
These differences in biochemical properties between HAT and MCT indicate that they play different physiological roles in the airways.

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A 70-year-old woman was admitted to our hospital for evaluation of a nodular shadow and an apporent cavity in the right middle lung field. Transbronchial biopsy and percutaneous meedle biopsy had failed to result in a diagnosis. Serial chest X-ray films revealed slight regression of the nodule without therapy. Pulmonary dirofilariasis was diagnosed after open-lung biopsy and Ouchterlony's double diffusion test. Cavity formation and spontaneous regression are rare in pulmonary dirofilariasis.

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A 57-year-old woman with congenital syphilis had a productive cough, fever, cholecystolithiasis, and paralytic ileus. She had a 30-year history of recurrent bronchitis accompanied by yellowish serous sputum. A chest radiograph showed bilateral infiltrates resulting from aspiration pneumonia, and a reduction in volume of the right middle and right lower lobes. After recovering from paralytic ileus, she still had fever and biliptysis. Bronchoscopy revealed bile filling the right basal bronchi. Emergency laparotomy and throacotomy revealed a gall stone, splenomegaly, marked atrophy of the right lobe of the liver, and adhesion between the right pleura and the diaphragm. A T-tube cholangiogram showed that the right hepatic duct communicated with the right basal bronchus. Despite ligation of the right hepatic duct, biliptysis continued. The patient died due to rapidly progressing hepatorenal failure.
Syphilis gummosa due to congenital syphilis was suspected as a cause of the bronchobiliary fistula, but was not confirmed pathologically. The surgical specimen showed only nonspecific fibrosis with calcification. Bronchobiliary fistula is rare in Japan; we know of only 6 other reported cases.

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A 43-year-old man who had been treated for bronchial asthma presented with an increase in dry coughing and wheezing for one and a half years. In August 1994, the patient noted progressive dyspnea on exertion. A chest radiograph revealed nodular opacity in the right upper lung field. In November 1994, the patient was admitted to Kinki University Hospital with an erythematous rash on the soles of both feet. Examination of a specimen biopsy of the skin lesion revealed granuloma with eosinophil infiltration. Peripheral blood eosinohilia was noted and a bone marrow examination also revealed an increased level of eosinophils. Another chest radiograph revedaled that the nodular opacity had disappeared and a new bilateral pleural effusion was seen. Eosinophils were the predominant cells in the pleural effusion. the patient's condition was further complicated by myocarditis.
Allergic granulomatous angitis (Churg-Strauss syndrome) was diagnosed and steroid therapy was started. After the start of steroid therapy, the skin eruption disappered and the myocarditis became less severe. Symptoms of asthma were also well controlled. The eosinophils had hypersegmented unclei and increased expression of adhesion molecules on their surfaces.

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A 45-year-old man was admitted to the hospital because of an increase in the size of an abnormal shadow on chest X-ray films over the preceding 5 years. A chest X-ray film on admission revealed a round air space in a mass-like shadow in the right upper lung field. Results of hpysical examination, sputum cytologic examination, and Ziehl-Neelsen staining were negative. Histopathological examination of a percutaneous lung biopsy specimen revealed a benign fibrous tumor of the pleura. Thoracotomy revealed a pedunculated tumor arising from the visceral pleura at the apical segment of the right upper lobe. A wedge resection was done. Histological examination revealed that the tumor consisted of regularly-shaped spindle cells with densely collagenous tissue. Normal lung tissue was found to be invaginated into the tumor tissue. These findings are consistent with the radiologic findings.

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A 58-year-old woman was admitted to the hospital because of fever and hypotension. That night, shock developed. On the second hospital day, a chest roentgenogram showed retention of pleural fluid and the group A-beta hemolytic organism Streptococcus pyogenes was detected in the effusion. The toxic shock-like syndrome was diagnosed. The patient recovered with artificial ventilation, administration of antibiotics, and blood purification. In this patient, the type of pyrogenic exotoxin was B+C.

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A 47-year-old woman was admitted to our hospital because of shortness of breath. She had wheezed for a long time before admission. A flow-volume curve showed a pattern consistent with a fixed obstruction of the trachea. CT findings also revealed severe stenosis of the trachea caused by a tumor mass. Tracheal carcinoma was diagnosed after transbronchoscopic biopsy. The patient received chemotherapy and radiation therapy concurrently for about six weeks. Her symptoms resolved, and a second flow-volume curve had a normal pattern. Tracheal carcinoma should be included in the differential diagnosis of airway obstruction. In such cases, the flow-volume curve can be useful as a screening test.

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A woman born in 1920 had had multifocal, yellowish nodules on her skin since childhood and had been given a diagnosis of pseudoxanthoma elasticum. When she was 59-year-old, a diffuse granular shadow was seen on a chest radiograph. When she was 74-year-old, she presented with dyspnea on exertion. A thoracoscopic lung biopsy was done. Histologic examination of the lung tissue revealed small calcified nodules scattered in the alveolar septa.
Pseudoxanthoma elasticum is a systtemic, hereditary disorder that usually affects elastic fibers of the skin, eye, and cardiovascular system. It's incidence is quite low: 1 out of 160, 000-1, 000, 000 population. Very few cases of pseudoxanthoma elasticum with pulmonary involvement have been reported, and we know of no previous case in Japan in which pulmonary microcalcification was confirmed histologically.

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A 29-year-old woman in the 6th month of pregnancy was admitted to our hospital because of repeated hemoptysis after an acute respiratory infectin. Fiberoptic bronchoscopy revealed a polypoid lesion at the orifice of the right B³ bronchus and bleeding around the lesion. Bronchial arteriography showed no abnormalities. Transcatheter embolization was unsucessful. Therefore, a right upper lobectomy was done. Microscopical examination showed a mass of coagulated blood on the mucosal surface of the wall of the B³ bronchus, and a bronchial artery running toward the mucosal surface was seen underneath. The mass was thought to be the polypoid lesion seen during bronchoscopy. Serial sections showed a bronchial aneurysm 1mm in diameter that had ruptured into the bronchial lumen. Several bronchial arteries were located in the propria mucosa in other bronchial segments, as observed in the B³ bronchus, but no other focus of bleeding was identified. Some cases of hemoptysis of unknown cause may result from similar histological alterations.

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A 29-year-old woman developed fever and sore throat 14 days before admission. Six days later she noted the onset of a rash and ten days later she noted a dry cough. The chest X-ray film showed no abnormal shadow, but a chest CT scan showed a diffuse ground-glass shadow and a ⁶⁷Ga-scintigram showed abnormal lung uptake. The level of anti-cytomegalovirus antibody of the IgM class was high; cytomegalovirus mononucleosis was diagnosed and cytomegalovirus pneumonia was suspected. Cytomegalovirus DNA was detected in bronchoalveolar lavage fluid by the polymerase chain reaction method. We known of no previously reported case of cytomegalovirus mononucleosis in which cytomegalovirus DNA was found in bronchoalveolar lavage fluid by the polymerase chain reaction method.

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An 18-year-old woman was admitted to our hospital for treatment of the fifth episode of spontaneous pneumothorax. She had a history of repeated pneumonia in childhood and mycoplasma pneumonia at 12 years of age. A chest X-ray film revealed aleft-sided pneumothorax, atelectasis of the left upper lobe, and hyperlucency of the left lung. A bronchogram showed poor filling of the peripheral bronchi by contrast medium and mild cylindrical bronchiestasis in the proximal bronchi. Pulmonary arteriography showed small left pulmonary arteries. From these findings, Swyer-James syndrome was diagnosed. This case was complicated by bronchial asthma, with eosinophilia, a high level of IgE, and airway hyperresponsiveness. Atelectasis, multiple bullae, and bronchial asthma had been caused by mycoplasma pneumonia in childhood. Reccurent pneumothorax had been caused by emphysematous changes in the bronchioli and by underdeveloped pulmonary arteries. Surgery to treat the recurrent spontaneous pneumothorax was considered, but was not done because of the risk of relapse and the ventrilaiton-perfusion imbalance due to the Swer-James syndrome.

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A 35-year-old woman was admitted to our hospital because of a fever and a productive cough. She had undergone renal transplantation and had taken immunosuppressive drugs, a steroid, inhaled amphotericin-B, and pentamidine. She was treated with ganciclovir, becouse infection with cytomegalovirus was suspected but her symptoms did not resolve. A chest X-ray film and a computed tomogram showed an infiltrative shadow in the right lower lung field. Specimens obtained by transbronchial lung biopsy showed lipid-laden macrophages and oil droplets in alveolar spaces. Organisms of the genus nocardia were isolated from bronchial lavage fluid. The final diagnosis was lipoid pneumonia combined with pulmonary nocardiosis. After treatment with Imipenem·cilastatin sodiom, Exacin and Sulfamethoxazole·trimethoprim, her symptoms and the infiltrative shadows on the chest X-ray film resolved. We believe that this patient had an exogenous lipoid pneumonia caused by inhalation of deoxycholic acid in amphotericin-B solubilized liquid, in addition to pulmonary nocardiosis.

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