Bronchiectasis has come to be considered as a type of sinobronchial syndrome in Japan, but there exist some cases without chronic sinusitis. We studied the clinical features of 14 cases of bronchiectasis with definitely normal paranasal sinuse roentgenogram, diagnosed during the past ten years.
There were eleven middle-aged women and three men. Ten patients (71%) complained of hemotysis, one (7%) of dry cough, one (7%) of productive cough, and the two (14%) had no complaint. In seven ptients (50%) CT and bronchography showed localized cylindrical bronchiectasis in the right middle lobe and/or left upper lobe lingular division. They were considered to be middle lobe lingular syndrome. Three patients (22%) with localized varicose or cystic bronchiectasis had a history of pneumonia or pertussis in their infancy, so their bronchiectasis were considered secondary to infantile bronchopulmonary disease. Two patients (14%) had diffuse cystic bronchiectasis and were almost asymptomatic. They might be cases congenital bronchiectasis or Williams-Campbell syndrome. Pulmonary function tests were normal in most of the cases and sputum culture revealed no cases of persistent bacterial infection.
These clinical features are quite different from those of bronchiectasis reported as sinobronchial syndrome, in which chronic productive cough, poor pulmonary function, persistent bacterial infection, etc. are significant. So we conclude that there are two distinct groups in bronchiectasis.

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In order to evaluate the role of membranous (MB) and respirtory bronchioles (RB) in patients with normal and mild airflow limitation, we quantitatively assessed the pathological change of MB, RB and alveoli in the pathological specimens of 42 patients who required surgery for removal of a solitary pulmonary nodule. We also measured the destructive index (DI), to evaluate the destruction of alveolar walls. High resolution CT scan and pulmonary function tests were performed within 1 month of resection.
Results of the pulmonary function tests correlated with pathological changes of RB better than with MB. Significant correlation between the emphysema score of resected lung and the pathological change of MB and RB was observed. There was also significant correlations between the CT score or DI and RB changes. However, there was no significant correlation between the CT score or DI and the change of MB. There were significant differences between smokers and nonsmokers in the results of pulmonary function test, pathological changes of RB and severity of emphysema.
We postulate that RB is responsible for subtle functional abnormalities in the early stages of chronic obstructive pulmonary disease.

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Sleep has been shown to be associated with a variety of changes in ventilatory mechanics. Recently, airway occlusion pressure (P_0.1) has come to be widely used as an index of the neural output of the respiratory center, and is considered to be independent of lung mechanics. The purpose of this study was to analyze neural output of the respiratory center during sleep, utilizing an airway occlusion pressure procedure.
Nine normal male subjects, 19 to 32yrs of age, were used. Each subject was fitted with a face mask, and the area between the mask and the face was sealed with medical silicone rubber. They breathed through the mask, which was attached to a “J” valve that separated the inspiratory from the expiratory line. A radio-controlled camera shutter was joined to the “J” valve on the inspiratory line. The volume of dead space of this system was about 150ml. Inspiratory pressure developing in the oral cavity was measured using a pressure transducer and a photocorder. Flow rate, tidal volume (V_T) and inspiratory time (T_I) were measured using a hot wire flow meter. Airway occlusion pressure (P_0.1) was measured 0.1 second after the onset of the inspiratory effort by occluding the shutter on the inspiratory line during expiration. The onset of inspiration and expiration was detected by changes in airway pressure. Great care was taken to make the subjects as comfortable as possible during the procedure. Continuous recordings were made on a polygraph recorder of EEG, EOG, EMG and cardiorespiratory variables. Sleep stages were determined using the standard criteria.
P_0.1 declined in both non-rapid eye movement (NREM) and rapid eye movement (REM) sleep compared with that of wakefulness. The mean value of P_0.1 hit its highest value at stage 1, and decreased to the lowest value at stage 2 during sleep. P_0.1 value during stage 3-4 mildly increased in comparison with those found during stage 2. There was a tendency to take higher P_0.1 value in phasic REM sleep (PREM) in comparison with that in stage 2 NREM and tonic REM sleep (TREM). Linear correlations were observed between the P_0.1 and the mean inspiratory flow rate (V_T/T_I) in wakefulness, NREM and REM sleep; these correlations were statistically significant. This finding suggested that the mean inspiratory flow rate represents the neural drive output during both waking and sleeping. V_T declined in all sleep stages, compared with that during waking. V_T decreased to the lowest value at stage 2 during sleep. There was a tendency to reach higher V_T values in PREM in comparison with that during TREM. These results indicated that neural drive output from the respiratory centers was lower during sleep than during waking. The increase of P_0.1 found in REM sleep, especially phasic REM sleep, indicated an increase in diaphragmatic activities in this sleep stage.

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Control mechanism of the respiratory muscle during forced expiration was analyzed. Respiratory flow. EMG of the intercostal muscle (IC), external oblique (EO) and internal oblique (IO) abdominal muscle were analyzed in eleven healthy volunteers. The pattern of EMG activity during forced expiration could be classified according to its shape i. e., taper- or hill-shaped. The taper shape was found in 7 of 9 subjects for IC and in 8 of 11 subjects for EO. The hill shape was found in 4 of 6 subjects for 10. Twelve consecutive trials without intervals did not alter the pattern of flow-volume curve, activity of resiratory muscle and frequency component (i. e., H/L ratio). When the expiratory circuit was obstructed for a short interval during forced expiration, the rise in pleural pressure was slight but that in oral pressure was large, and the activity of respiratory muscle decreased for a short period. The latencies from the onset of expiratory circuit obstruction to the reduction in EMG activity were 61.5-100msec. In IC, 61.5-106.5 msec in EO. and 58.0-92.3msec in 10. These values did not differ significantly any given subject. Airway anesthesia with xylocaine did not alter latency.
In conclusion, the pattern of respirtory muscle activity for forced expiration is predetermined and is specific for each muscle. Negative feedback from receptors in airway or in respiratory muscle was strongly suggested. Repetitive trials up to twenty times did not cause respiratory muscle fatigue.

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We measured NCF activity and examined the characteristics of NCF in bronchoalveolar lavage (BAL) fluid, sputum and culture media of alveolar macropages. Several kinds of NCF were detected in BAL fluid obtained from normal volunteers, and at least a part of these NCF was thought to be derived from alveolar macropages. NCF activity, especially complement-derived NCF, was increased in BAL fluid and in sputum obtai-ned from patients with chronic respiratory tract infection. In patients with idiopathic interstitial pnemonia, alveolar macrophagederived NCF as well as complement derived NCF were increased in BAL fluid. These results indicate that different types of NCF may increase in response to the disease state or pathogenesis, and play important roles in neutrophil accumulation in the respiratory tract.

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LSP contents of sputum samples from patients with chronic airway diseases were measured by an enzymelinked immunosorbant assay (ELISA) kit which was designed by Kuroki et al to examine whether a substance identical to lung surfactant contained in alveolar lining layer, is also contained in respiratory tract fluid or not in the ELISA kit. One antibody to LSP was conjugated to peroxidase and another one to LSP was fixed onto a bead.
A neo-anionic detergent, Triton X-100 and an anionic detergent, sodium dodecyl sulfate (SDS) were added to extraction medium to separate LSP from lung surfactant, and LSP reaction of sputum sample was maximal when the ratio of Triton X-100 to SDS was in range of 1 to 4. Airway mucous glycoprotein (AMG) purified from sputum sample did not show any LSP reaction. In CsCl density gradient ultracentrifugation of whole sputum, the LSP reaction was detectable only in the top fraction with density of about 1.40 and AMG was located in the fraction with a density of about 1.50. These results indicate that the LSP reaction of sputum sample is not due to false reaction caused by nonspecific binding of viscous AMG to the two antibodies to LSP, but to the existence of LSP. Therefore it was concluded that lung surfactant is contained in respiratory tract fluid.
In general, the LSP concentrations in sputum samples were lower in purulent suta than in mucoid or mucopurulent sputa, and lower in patients with diffuse panbronchiolitis and bronchiectasia than in those with pulmonary emphysema and chronic bronchitis. It was shown that LSP was hydrolyzed by neutrophil leucocyte homogenate. These results suggest that LSP content of sputum is influenced by various factors such as infection and disease in the respiratory tract, and thus is useful to estimate pathological states of chronic airway diseases.

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To evaluate the significance of TPA (tissue polypeptide antigen) as a tumor marker for lung cancer, the present studies were designed to measure serum TPA levels as well as TPA contents in tumor tissues in patients with lung cancer.
Average serum TPA levels (203.5±180.5U/l, mean±SD) in 76 patients with lung cancer (30 squamous cell carcinoma, 38 adenocarcinoma, 8 small cell carcinoma) were significantly higher than those in pneumonia (83.3±42.7U/l, n=13), pulmonary tuberculosis (87.6±36.3U/l, n=16), and normal controls (46.8±23.7U/l, n=28). There was no significant difference in TPA levels according to the histological type of lung cancer.
Comparing respective stage cases, statistically significant differences were observed in serm TPA levels between normal controls and stage 1, stage 1 and stage 3, and in stage 3 and stage 4, suggesting a gradual increase in TPA levels accompanying the progress of the lung cancer.
In patients with 12 squamous cell carcinoma and 9 adenocarcinoma, the TPA levels in carcinoma tissues and non-tumor invaded lung tissues were 115.2±187.7 and 148.9±223.3U/mg protein, indicating no significant difference in carcinoma and normal tissues.
There was definite correlation between serum TPA levels and TPA contents in carcinoma tissues. No significant relationship, however, was observed between serum TPA levels and TPA contents in tissue not invaded by tumor. These results suggest that serum TPA levels reflect the tumor burden in patients with lung cancer. In conclusion, the determination of TPA in blood is useful for the diagnosis of lung cancer.

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Five patients with chronic pulmonary diseases (3 pulmonary emphysema, 1 chronic respiratory failure caused by old tuberculosis, 1 diffuse panbronchiolitis) showed a marked increase of plasma angiotensin-I and PRA level, which was accompanied by an increase of angiotensin-II level, however, whose systemic blood pressure was not elevated. The intravenous infusion of angiotensin-II-analogue (1-Sarcosine, 8-Isoleucine-Angiotensin-II) elicited an antagonistic blood pressure response resembling Bartter's syndrome.

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The objective of this study is to demonstrate the significance of superior vena cava (SVC) reconstruction in the surgical treatment of superior and anterior mediastinal invasive malignant tumors, by using a total of 48 cases consisting of 36 invasive thymomas, 3 thymic cancers, 3 carcinoids, 2 germ cell tumors, 2 malignant lymphomas, 1 intrathoracic thyroid cancer and 1 malignant melanoma.
In 22 of 48 cases there was only mediastinal pleural invasion or tumor capsule invasion, on the other hand, 26 cases had high ratios of invasion to adjacent organs, including lung, pericardium, great veins and the phrenic nerve. Sixteen of 26 cases had invasion to 1-2 adjacent organs, but 10 had 3-6 organ invasion.
Among 12 cases invading SVC or brachiocepalic veins, 9 cases during the last 7 years underwent SVC resection and reconstruction with EPTFE grafts. The patency was good in the grafts with external ring support and no SVC symptoms were observed in all cases postoperatively. The longest patent and functional graft is 40 months postoperative. No statistically significant difference were demonstrated in the survivals between cases with SVC reconstruction and those with resections of other adjacent organs, furthermore, remarkable difference of survival was demonstrated in SVC reconstructed cases with complete resection and incomplete resection. The survivals in cases with combined resection of 1-2 adjacent organs was statistically significantly better than those with combined resection of 3-6 adjacent organs (p<0.05, Cox-Mantel test).
In conclusion, SVC reconstruction with EPTFE grafts is considered to be a significant surgical procedure for the recovery of symptoms, improved resectability and survival prolongation in the surgical treatment of superior and anterior mediastinal invasive malignant tumors.

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A 22-year-old man was admitted because of hemosputum and progressive dyspnea with 3 attacks of chest pain and dyspnea over the previous 4 months. Chest roentgenography showed pulmonary infarction of the left lower lobe, and the diagnosis was confirmed by pulmonary perfusion and inhalation scintigraphy and pulmonary arteriography.
Thrombolytic therapy was performed, but no significant effect could be obtained and anticoagulant therapy was performed continuously. No deep-vein thrombosis could be seen. He was considered to have autoimmune hemolytic anemia with lupus anticoagulant on the basis of auto-antibody data.
Lupus anticoagulant is an antibody to phospholipid, and it is suggested that a decrease in the production of prostanoid in the endothelium causes thrombosis.
In this case, as the patient showed a low level of 6-keto-PGF_1α in the blood, it is suggested that one of the etiological factors of pulmonary thromboembolism is a disorder of prostacyclin production in the endothelium, causing thrombosis by lupus anticoagulant.

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A 66-year-old woman was admitted with dyspnea. A tracheal tumor was found by bronchofiberscopy, and histological examination revealed large cell carcinoma. The tumor obstructed nearly 90% of her tracheal lumen, so we performed intratumoral ethanol injection. The tumor became almost completely necrotic, and obstruction of her airway markedly improved. No serious complication was found. Intratumoral ethanol injection was very effective and safe in this case. This is the fifth report of undifferentiated carcinoma of the trachea in Japan.

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This article describes a case of Goodpasture's syndrome controlled by double filtration plasmapheresis (DFPP) combined with steroid and immnosuppressant therapy. A 48-year-old male, clerk, complaining of fever, dry cough and macroscopic hematuria, was admitted to our hospital. Microscopic hematuria was first pointed out at age 40 on an annual check up. His laboratory data on admission revealed severe anemia, azothemia, macroscopic hematuria and proteinuria. His chest radiograph and CT revealed diffuse nodular densities in bilateral lung fields. Specimens obtained by transbronchial lung biopsy and open renal biopsy revealed linear deposition of IgG by direct immunofluorescent antibody methods. Circulating antiglomerular basement membrane antibody level determined with radioimmunoassay was 1.8% on admission, but one week later it elevated to 5.6% with progression of dyspnea, hypoxemia, and renal failure. Steroid pulse therapy and a total of 6 double filtration plasmaphereses were performed in the first month. Subsequently hypoxemia and dyspne disappeared, and the chest radiograph of the 40th hospital day showed no abnormal shadows. Two months later recurrence of pulmonary hemorrhage was noticed. Immunosuppressant administration (Cyclophosphamide 100mg/day) and a total of 10 DFPP procedures were performed with success. By DFPP, circulating anti-GBM antibody fell rapidly to within normal ranges, and anti-GBM antibody level elevated in removed plasma. We think DFPP is effective to remove circulating anti-GBM antibody in Goodpasture's syndrome.

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This is a very rare case report of Goodpasture's syndrome with IgA antibasement membrane antibody. A 43-year old male was admitted because of severe dyspnea with slight hemoptysis. Chest X-ray demonstrated extensive bilateral infiltrates with air bronchogram, predominantly in the right lung. Laboratory data on admission showed severe anemia and moderate renal impairment. The pumonary infiltrates resolved spontaneously within 10 days. Goodpasture's syndrome or collagen vascular disease was suspected and he underwent a percutaneous renal and transbronchial lung biopsy.
The renal biopsy showed crescent formation affecting 70-80% of glomeruli. Linear IgA deposits, but not IgG, were demonstrated along the glomerular basement membrane by the direct immunofluorescence procedure. The lung biopsy contained many hemosiderin-laden macrophages in the lumen of the alveoli and showed mild thickening of alveolar walls. However, linear immunoglobulin deposits on the alveolar capillary basement membrane were not demonstrated by direct immunofluorescence. The diagnosis of Goodpasture's syndrome with IgA antibasement membrane antibody was made. His serum was negative for antibasement antibody by indirect immunofluorescence.
He was treated with prednisone, 30mg daily. His pulmonary symptoms and anemia improved markedly, but his renal function did not change. Thirteen months after his first admission, he suffered from severe bacterial pneumonia, which was complicated by disseminated intravascular coagulation. He died of respiratory failure. Autopsy was rejected.

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The patient was a female who came to our hospital with a chief complaint of hemoptysis. Radiography of the chest demonstrated a funicular shadow, and CT of the chest also revealed a similar shadow in contact with the thoracic aorta enhanced by the contrast material. Pulmonary areriography and aortography showed that the left S8, S9 and S10 areas were supplied by abnormal blood vessels bifurcating from the thoracic aorta, rather than the pulmonary arery. This finding was supported by scintigraphy of the pulmonary blood flow and pulmonary RI angiography. Since bronchography revealed no obvious abnormality in the bronchial system, the diagnosis of Pryce's type I intralobar pulmonary sequestration was established, and left lower lobectomy was carried out. While the evidence of abnormal blood vessels is important for the definitive diagnosis of pulmonary sequestration, concurrent image processing of the results of scintigrapy of the pulmonary blood flow and pulmonary RI angiography permits noninvasive demonstration of the fact that the sequestral pulmonary tissue is regulated by the systemic circlatory system, rather than the pulmonary circulatory system and thus is a useful technique.

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A 43-year-old man was admitted to our hospital because of cough and dyspnea. His chest X-ray showed bilateral basal infiltrative shadows. The increased eosinophils in BAL suggested chronic eosinophilic pneumonia (CEP), whereas the TBLB specimens showed findings compatible with desquamative interstitial pneumonia (DIP). The open lung biopsy specimens showed typical findings of DIP with a peripheral zone, suggestive of CEP. These two diseases may be only phasal differences of reaction to the same extrinsic stimulation.

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A 71-year-old man complained of mild dyspnea, and his chest X-ray showed a cavitating lesion accompanied by infiltrative changes in the right middle lobe. Percutaneous aspiration revealed numerous gram-positive and acidfast branching rods. Morphological examination and biochemical tests of the colonies confirmed the diagnosis of pulmonary nocardiosis caused by nocardia asteroides. The patient was treated successfully with sulfamethoxazoletrimethoprim.
It has been reported that approximately half of the patients with pulmonary nocardiosis have immunodeficiency, but our patient had no underlying disease.
This disease has no characteristic clinical features, so diagnosis is difficult. As nocardia may invole the central nervous system leading to a poor prognosis, early diagnosis and prompt treatment are required to improve survival.

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